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Phase 2a Sheffield Medicine > Musculoskeletal and Rheumatology > Flashcards

Musculoskeletal and Rheumatology Flashcards

1
Q

What is Osteomyelitis?

A
  • Osteomyelitis is an inflammatory condition of bone/ bone marrow caused by an infecting organism, most commonly Staphylococcus aureus.
  • All forms of acute osteomyelitis may evolve and become chronic, sharing a final common pathophysiology, with compromised soft-tissue surrounding dead, infected, and reactive new bone.
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2
Q

What are the risk factors for osteomyelitis?

A
  • Diabetes
  • Old age
  • Peripheral vascular disease
  • Immunocompromised
  • Malnutrition
  • Trauma/ injury

Patients with peripheral vascular disease, neuropathy, diabetes, reduced mobility or nutritional deficiency are at greater risk of developing skin ulcers and having poor healing following surgery.

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3
Q

What are the signs of osteomyelitis?

A
  • Erythema
  • Swelling
  • Evidence of previous surgery or trauma
  • Tenderness
  • Discharging sinus
  • Ulcers / skin breaks
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4
Q

What are the symptoms of osteomyelitis?

A
  • Fever
  • Pain
  • Overlying redness
  • Swelling
  • Malaise
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5
Q

What are the histopathological changes seen in osteomyelitis?

A
  • Acute changes:
    • Inflammatory cells
    • Oedema
    • Vascular congestion
    • Small vessel thrombosis
  • Chronic changes:
    • Necrotic bone ‘sequestra’
    • New bone formation - involucrum
    • Neutrophil exudates
    • Lymphocytes & histiocytes (tissue macrophages)
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6
Q

What are the investigations for osteomyelitis?

A
  • MRI - Gold standard and Imaging modality of choice
  • FBC - elevated WCC
  • CRP and ESR - elevated
  • U&Es
  • LFTs
  • HbA1c - patients who don’t have known diabetes, should be screened
  • Urine MSU
  • Blood cultures
  • Wound swabs
  • Bone biopsy and culture
  • X-ray of suspected area
  • CT
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7
Q

What is the management for osteomyelitis?

A
  • Antibiotics
    • Antibiotic courses tend to be a minimum of 4-6 weeks and are guided by microbiology (usually given after blood culture results obtained)
    • Empiric regimens may be given e.g. ceftriaxone and vancomycin for good coverage against S. aureus and MRSA. This can then be updated when positive cultures are obtained.
  • Surgical debridement
    • Most commonly used in non-haematogenous spread.
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8
Q

What are some indications for surgical intervention for osteomyelitis?

A
  • Failure to respond to antibiotic therapy
  • Formation of discrete abscess
  • Neurological deficit(vertebral osteomyelitis)
  • If surgical metalwork is present, its removal must be considered.
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9
Q

What are some complications of osteomyelitis?

A
  • Septic arthritis - if infection spreads to joints
  • Growth disturbance in children and adolescents
  • Amputations
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10
Q

What is osteoporosis? What is osteopenia?

A
  • Osteoporosis is a complex skeletal disease characterised by low bone density and micro-architectural defects in bone tissue, resulting in increased bone fragility and susceptibility to fracture.
  • Osteopenia refers to a less severe reduction in bone density than osteoporosis.
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11
Q

What are the risk factors for osteoporosis?

A
  • Mnemonic SHATTERED
    • Steroid use (long term corticosteroids)
    • Hyperthyroidism, hyperparathyroidism, hypercalciuria
    • Alcohol and tobacco use
    • Thin - Low BMI (<18.5 kg/m2)
    • Testosterone decrease
    • Early menopause
    • Renal or liver failure
    • Erosive/ inflammatory bone disease e.g. myeloma or RA
    • Dietary (reduced Ca2+, malabsorption, diabetes)

Other
- Older age
- Female (especially post-menopausal, as oestrogen is osteo-protective)
- Caucasian/ Asian
- Family history
- Previous fragility fracture
- Reduced mobility and activity

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12
Q

What are the clinical manifestations of osteoporosis?

A
  • Asymptomatic condition with the exception of fractures
  • Common fragility fractures include vertebral crush fracture and those of the distal wrist (Colles’ fracture) and proximal femur.
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13
Q

What are the investigations for osteoporosis?

A
  • DEXA Scan (Dual-Energy X-ray Absorptiometry) - First line and gold standard.
    • Can be measured anywhere on the skeleton but reading at the hip is KEY.
  • FRAX tool calculation
  • X-ray
  • Bloods
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14
Q

What does a FRAX score determine for osteoporosis?

A
  • FRAX without bone mineral density
    • Low risk – reassure
    • Intermediate risk – offer DEXA scan and recalculate the risk with the results
    • High risk – offer treatment
  • FRAX with bone mineral density
    • Treat
    • Lifestyle advice and reassure
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15
Q

What is the management for osteoporosis?

A
  • First line - Bisphosphonates
    • Alendronate 70mg once weekly (oral)
    • Risedronate35mg once weekly (oral)
    • Zolendronic acid 5 mg once yearly (intravenous)
  • Second line - Denosumab in Women, Teriparatide in Men
  • Strontium ranelate, Raloxifene or Parathryoid hormone replacement therapy or Hormone replacement therapy should also be considered.
  • Lifestyle changes
  • Vitamin D and calcium supplementation
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16
Q

What are the complications of osteoporosis?

A
  • Fractures
  • Side effects of bisphosphonates:
    • Refluxandoesophageal erosions. Oral bisphosphonates are taken on an empty stomach sitting upright for 30 minutes before moving or eating to prevent this.
    • Atypical fractures (e.g. atypical femoral fractures)
    • Osteonecrosis of the jaw
    • Osteonecrosis of the external auditory canal
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17
Q

What is Osteomalacia?

A
  • Osteomalacia is a metabolic bone disease characterised by incomplete mineralisation of the underlying mature organic bone matrix (osteoid) following growth plate closure in adults.
  • This results in softening of the bones.
  • The faulty process of bone mineralisation results in rickets in children and osteomalacia in adults.
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18
Q

What are the risk factors for osteomalacia?

A
  • Limited exposure to sunlight
  • Dark skin
  • Dietary vitamin D deficiency: fish, cheese, eggs
  • Chronic kidney disease: reduced activation of vitamin D (1-alpha-hydroxylation)
  • Liver dysfunction:reduced activation of vitamin D (25-hydroxylation)
  • Malabsorption: such as inflammatory bowel disease
  • Anticonvulsant use: phenytoin, carbamazepine and phenobarbital all increase cytochrome P450 metabolism of vitamin D
  • Tumour induced: tumour production of FGF-23 which causes hypophosphaturia (low phosphate)
  • Vitamin D resistance: some inherited conditions
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19
Q

What are the signs of osteomalacia?

A
  • Skeletal deformities
  • Waddling gait: a late sign
  • Signs of hypocalcaemia: such as Chvostek sign (contraction of facial muscles provoked by lightly tapping over the facial nerve)
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20
Q

What are the symptoms of osteomalacia?

A
  • Generalised bone pain: rib, hip, pelvis, thigh and foot pain are typical
  • Proximal muscle weakness
  • Difficulty walking upstairs
  • Muscle spasms and numbness due to hypocalcaemia
  • Fracture: often secondary to mild trauma, most commonly affecting the long bones
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21
Q

What are the investigations for osteomalacia?

A
  • Serum 25-hydroxyvitamin D - gold standard
  • Iliac bone biopsy with double tetracycline labelling - gold standard
  • Serum calcium and phosphate
  • Parathyroid hormone (PTH) level
  • Serum ALP
  • Renal and liver function tests
  • X-ray - not required for children if diagnosis is clear
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22
Q

What are the differential diagnoses for osteomalacia?

A
  • Osteoporosis
  • Paget’s disease of bone
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23
Q

What is the management for osteomalacia?

A
  • First line - Calcium with Vitamin D (colecalciferol)
  • Second line - Calcium with Vitamin D metabolite (calcitriol)
  • If due to an inherited or acquired disorder of phosphate add sodium phosphate to regimen.
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24
Q

What are the complications of osetomalacia?

A
  • Insufficiency fracture
  • Complications of treatment:hypercalcaemia, hyperphosphataemia
  • Secondary hyperparathyroidism
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25
Q

What is Rickets?

A

Ricketsresults from the same underlying process but occurs inchildren and adolescentsbefore the closure of the epiphyseal growth plates. This causes growth retardation and skeletal deformities.

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26
Q

What are the clinical manifestations of Rickets?

A
  • Knock-knees
  • Genu varum - bow legs
  • Craniotabes - thin, soft skull bones
  • Delayed closure of fontanelles
  • Prominent frontal bone
  • Protruding abdomen in severe disease
  • Fractures
  • Features of hypocalcaemia
  • Rachitic rosary - little bumps across the chest due to widening of junction between ribs and costal cartilage in front of the ribcage.
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27
Q

Define Paget’s disease of bone?

A
  • A chronic bone disorder that is characterised by focal areas of increased bone remodelling, resulting in overgrowth of poorly organised bone.
  • Also known as osteitis deformans.
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28
Q

What are the risk factors for Paget’s disease of bone?

A
  • Family history
  • Age >50 years
  • Infection
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29
Q

What are the clinical manifestation of Paget’s disease of bone?

A

Usually asymptomatic at the early stages.

  • Pain - due to bone impinging on nerves
  • Growth of bones in skull can cause:
    • Leontiasis - lion like face
    • Hearing loss - narrow auditory foramen and impinge on auditor nerve
    • Vision loss - bony overgrowth impinges on optic nerve
  • Kyphosis - curved spine
  • Lower limb muscle weakness - due to compression of spinal cord
  • Pelvic asymmetry
  • Bone enlargement - particularly pelvis, lumbar spine, skull, femur and tibia
  • Bowlegs - if tibia and femur becomes too weak to support weight
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30
Q

What are the investigations for Paget’s disease of bone?

A
  • X-ray
  • ALP levels
  • Calcium and Phosphate levels - will be normal
  • Bone biopsy to rule out other malignancies
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31
Q

What is the management for Paget’s disease of bone?

A
  • Pain relief
    • NSAIDs
  • Anti-resorptive medication - Biphosphonates e.g. alendronic acid
    • Along with calcium and vit D supplementation - FIRST LINE
  • Surgery -
    • Correct bone deformities
    • Decompress impinged nerve
    • Decrease fracture risk
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32
Q

What are the complications of Paget’s disease of bone?

A
  • Paget’s sarcoma (osteosarcoma)
  • Spinal stenosis and spinal cord compression
  • Fractures
  • Vision loss
  • Hearing loss
  • Arthritis
  • High-output cardiac failure and myocardial hypertrophy
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33
Q

What is Osteoarthritis?

A
  • Osteoarthritis is characterised by progressive synovial joint damage resulting in structural changes, pain and reduced function. It is the ‘wear and tear’ of joints.
  • Usually primary, but can be secondary to joint disease or other conditions e.g. haemochromatosis, obesity, occupational.
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34
Q

What are the risk factors for osteoarthritis?

A
  • Age
  • Gender → Female > Male
  • Raised BMI
  • Joint injury or trauma
  • Joint malalignment and congenital joint dysplasia
  • Genetic factors (COL2A1 collagen type 2 gene) and family history
  • Abnormal or excessive stress e.g. from exercise or particular occupations
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35
Q

What are the signs for osteoarthritis?

A
  • Heberden’s nodes: swelling in distal interphalangeal joint (top finger joint)
  • Bouchard’s nodes: swelling in proximal interphalangeal joint (middle finger joint)
  • Fixed flexion deformity of carpometacarpal (base of thumb)
  • Mucoid cysts: painful cyst found on dorsum of finger
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36
Q

What are the symptoms of osteoarthritis?

A
  • Joint pain which is worse with activity
  • Mechanical locking
  • Giving way
  • Joint tenderness
  • Joint effusion (fluid in or around joint)
  • Limited joint movement - stiffness
  • Crepitus - crunching sensation when moving joint
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37
Q

What is the diagnostic criteria for osteoarthritis?

A
  • Investigations not always needed if there is a typical presentation:
    • Over 45 years of age
    • Typical activity related pain
    • No morning stiffness (or morning stiffness <30 minutes)
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38
Q

What are the investigations for osetoarthritis?

A
  • X-ray can be used to check severity and confirm diagnosis - FIRST LINE
  • MRI
  • CT
  • Ultrasound
  • Aspiration of synovial fluid
  • CRP may be elevated
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39
Q

What is the LOSS mnemonic for X-ray diagnosis of osteoarthritis?

A
  • X-ray can be used to check severity and confirm diagnosis (mnemonic LOSS)
    • Loss of joint space
    • Osteophytes (bits of bone sicking out - bony overgrowth)
    • Subarticular sclerosis (end of bone at point of articulation is thickened)
    • Subchondral cysts (cysts appearing around the articulation)
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40
Q

What are the differential diagnoses for osteoarthritis?

A
  • Rheumatoid arthritis
  • Chronic tophaceous gout
  • Psoriatic arthritis
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41
Q

What is the pharmacological management for osteoarthritis?

A
  • 1st line - Topical capsaicin
  • 2nd line - Oral paracetamol + Topical capsaicin
  • 3rd line - Topical NSAIDs + Oral paracetamol + topical capsaicin

Other
- Intra-articular steroid injection

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42
Q

What is the non-pharmacological management for osteoarthritis?

A
  • Patient education
  • Weight loss
  • Low impact exercise
  • Heat and cold packs at site of pain
  • Physiotherapy
  • Occupational therapy
  • Orthotics - helps with foot issues

Other
- Joint replacement (arthroplasty) e.g. hip and knee
- Arthroscopy - only done if there are loose bodies causing knee lock

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43
Q

What is septic arthritis?

A
  • Septic arthritis is defined as the infection of 1 or more joints caused by pathogenic inoculation of microbes. It occurs either by direct inoculation or via haematogenous spread.
  • It is a medical emergency.
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44
Q

What are the risk factors for septic arthritis?

A
  • Underlying joint disease:10-fold increased risk; conditions such as rheumatoid arthritis, osteoarthritis and gout
  • Intravenous drug use:transfer of pathogenic organisms into the bloodstream
  • Immunocompromised:elderly, diabetes, HIV
  • Prosthetic joint
  • Recent joint surgery
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45
Q

What are the clinical manifestations of septic arthritis?

A
  • Key presentation - Septic arthritis mainly affects one joint and so should be suspected in all monoarthritic cases. The knee is most commonly affected, but hip, shoulder, wrist and elbow joints are also affected.
  • Hot, tender, erythematous, swollen joint
    • In the elderly and immunosuppressed and in RA the articular signs may be muted
  • Very limited range of movement
  • Difficulty weight bearing
  • Fever
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46
Q

What are the investigations for septic arthritis?

A
  • Gold standard - Joint aspiration - MSC

First line
- FBC
- CRP and ESR
- Blood cultures

Other
- Joint imaging

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47
Q

What are the differential diagnoses for septic arthritis?

A
  • Gout
  • Pseudogout
  • Reactive arthritis
  • Hemarthrosis
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48
Q

What is the management for septic arthritis?

A
  • Empirical therapy: flucloxacillin is first-line
  • Penicillin allergy: clindamycin
  • Suspected or confirmed MRSA: vancomycin
  • Gonococcal arthritis or gram-negative infection: cefotaxime or ceftriaxone
  • Joint drainage
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49
Q

What are the complications of septic arthritis?

A
  • Osteomyelitis: the spread of infection from the joint to the surrounding bone
  • Permanent joint destruction
  • Sepsis
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50
Q

What is dermatomyositis and polymyositis?

A
  • Polymyositis and dermatomyositis are autoimmune disorders where there is inflammation in the muscles
  • Polymyositis- chronic inflammation of the muscles
  • Dermatomyositis- CT disorder where there is chronic inflammation of the skin and muscles
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51
Q

What are the risk factors for dermatomyositis and polymyositis?

A
  • Viruses
  • Genetic predisposition.
  • Drug induced.
  • Malignancy.
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52
Q

What are the general signs and symptoms of dermatomyositis and polymyositis?

A
  • Muscle pain, fatigue and weakness
  • Occur bilaterally and typically affects proximal muscles
  • Mostly affects shoulders and pelvic girdle
  • Develops over weeks
  • Polymyositis is without skin features, whereas dermatomyositis involves skin too.
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53
Q

What are the clinical manifestations specific to dermatomyositis and not polymyositis?

A
  • Symmetrical progressive muscle weakness and wasting of proximal muscles surrounding the shoulder and pelvic girdle.
  • Patients have issues squatting, going upstairs, rising from chair, raising their hand above head.
  • Involvement of pharyngeal, laryngeal and respiratory muscles can lead to dysphagia, dysphonia (difficulty speaking) and respiratory failure.
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54
Q

What are the clinical manifestations specific to polymyositis and not dermatomyositis?

A
  • Skin changes: Heliotrope discolouration of eyelids, scaly erythematous plaques over knuckles (Gottron’s papuples).
  • Arthralgia, dysphagia resulting from oesophageal muscle involvement and Raynaud’s phenomenon.
  • Associated with an increased incidence of underlying malignancy.
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55
Q

What are the investigations for dermatomyositis and polymyositis?

A
  • Muscle biopsy - gold standard.
  • Serum creatinine kinase will be elevated.
  • Serum aldolase will be elevated.
  • Antibodies:
    • Anti-Jo-1 - polymyositis (but often present in dermatomyositis).
    • Anti-Mi-2 - dermatomyositis.
    • Anti-nuclear - dermatomyositis.
  • Electromyogram (EMG) → Not essential but recommended for patients.
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56
Q

What is the management for dermatomyositis and polymyositis?

A
  • Corticosteroids → First line treatment.
  • When response to steroids is inadequate:
    • Immunosuppressants e.g. Azathioprine
    • IV immunoglobulins
    • Biological therapy e.g. Infliximad or Etanercept.
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57
Q

What is fibromyalgia?

A

Fibromyalgia is a chronic pain syndrome characterised by the presence of widespread body pain.

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58
Q

What are the risk factors for fibromyalgia?

A
  • Gender → Female > Male
  • Middle age
  • Genetic association
  • Environmental e.g. child abuse, low household income, divorced, low educational status, emotional problems, social withdrawal
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59
Q

What are the key presentations for fibromyalgia?

A
  • Allodynia - pain in response to non-painful stimulus
  • Hyperaesthesia - exaggerated perception of pain to mildly painful stimulus
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60
Q

What are the symptoms of fibromyalgia?

A
  • Widespread pain
    • Aggravated by stress, cold and activity
  • Extreme tenderness
  • Sleep disturbance
  • Fatigue - extreme and present after minimal exertion
  • Morning stiffness
  • Paraesthesia - abnormal sensation in skin
  • Headaches
  • Poor concentration
  • Low mood
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61
Q

What are the investigations for fibromyalgia?

A

Investigations are all normal.

  • Diagnosis of fibromyalgiais based on clinical features:
    • Chronic pain that has been present for at least 3 months
    • Widespread pain - involved left and right sides, above and below waist, and the axial skeleton.
    • Palpate tender point sites - severe pain in 3 to 6 different areas of your body, or you have milder pain in 7 or more different areas
    • No other reason for symptoms has been found
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62
Q

What investigations would be performed to rule out other conditions to diagnose fibromyalgia?

A
  • Thyroid function test (to exclude hypothyroidism)
  • ANA’s and DsDNA (to exclude SLE)
  • ESR & CRP (to exclude PMR)
  • Ca2+ and electrolytes (to exclude high calcium)
  • Vit D (to exclude low vitamin D)
  • Examine patient and CRP (to exclude inflammatory arthritis)
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63
Q

What are the differential diagnoses for fibromyalgia?

A
  • Rheumatoid arthritis
  • Chronic fatigue syndrome
  • Hypothyroidism
  • SLE
  • Polymyalgia rheumatica (PMR)
  • High calcium
  • Low vitamin D
  • Inflammatory arthritis
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64
Q

What are the non-pharmaclogical management options for fibromyalgia?

A
  • Regular exercise like cardiovascular fitness training which includes fast walking, biking, swimming, or water aerobics can help by reducing pain and fatigue. Grading of exercise is important to avoid over-exertion and fatigue.
  • Relaxation techniques and good sleep hygiene can also help.
  • Physiotherapy and rehabilitation
  • CBT
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65
Q

What is the pharmacological management for fibromyalgia?

A
  • Amitriptyline - tricyclic antidepressant
  • Serotonin-norepinephrinereuptake inhibitors (SNRIs) e.g. duloxetine
  • Help by elevating serotonin and norepinephrine levels.
    • Anticonvulsants like pregabalin and gabapentinwhich slow nerve impulses can help with sleep problems.
  • Cyclobenzaprine - induce muscle relaxation.
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66
Q

What is polymyalgia rheumatica?

A

An inflammatory rheumatological syndrome that manifests as pain and morning stiffness involving the neck, shoulder girdle, and/or pelvic girdle in people over the age of 50.

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67
Q

What are the risk factors for polymyalgia rheumatica?

A
  • SLE.
  • Polymyositis/dermatomyositis.
  • Gender → Female > Male.
  • More common in Caucasians.
  • Associated with Giant cell arteritis - most often both occur together.
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68
Q

What are the clinical manifestations for polymyalgia rheumatica?

A
  • Symptoms must be present for at least two weeks:
    • Bilateral shoulder pain - may radiate to elbow.
    • Bilateral pelvic girdle pain.
    • Worse with movement.
    • Interferes with sleep (symptoms are worse in morning and night).
    • Stiffness for at least 45 mins in the morning.
  • Other non-core features:
    • Systemic symptoms: fever, weight loss, loss of appetite.
    • Upper arm tenderness.
    • Carpal tunnel syndrome.
    • Pitting oedema.
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69
Q

What are the investigations for polymyalgia rheumatica?

A
  • Diagnosis is made based upon clinical presentation and the response to steroids. Other conditions must be concluded.
    • Inflammatory markers - elevated (normal does not exclude diagnosis)
    • There is ususally little damage to muscles
    • Creatinine kinase - Normal
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70
Q

What investigations should be performed to rule out other conditions for polymyalgia rheumatica?

A
  • FBC
  • U&Es
  • LFTs
  • Calcium - raised in hyperparathyroidism or cancer or low in osteomalacia.
  • Serum protein electrophoresis - myeloma and other protein disorders.
  • Thyroid stimulating hormone - thyroid function.
  • Creatine kinase - myositis.
  • Rheumatoid factor - rheumatoid arthritis.
  • Urine dipstick.
  • Anti-nuclear antibodies(ANA) for systemic lupus erythematosus.
  • Anti-cyclic citrullinated peptide(anti-CCP) for rheumatoid arthritis.
  • Urine Bence Jones proteinfor myeloma.
  • Chest X-rayfor lung and mediastinal abnormalities.
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71
Q

What are the differential diagnoses for polymyalgia rheumatica?

A
  • Osteoarthritis
  • Rheumatoid arthirits
  • Systemic lupus erythematosus
  • Myositis (from conditions like polymyositis or medications like statins)
  • Cervical spondylosis
  • Adhesive capsulitis of both shoulders
  • Hyper or hypothyroidism
  • Osteomalacia
  • Fibromyalgia
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72
Q

What is the management for polymyalgia rheumatica?

A
  • Initially - 15mg prednisolone per day.
  • Assess 1 week after steroids, if poor response in symptoms then it probably is not PMR and look for alternative diagnosis. Stop steroids.
  • Assess 3-4 weeks after starting steroids. You would expect a 70% improvement in symptoms and inflammatory markers to return to normal to make a working diagnosis of PMR.
  • If 3-4 weeks of steroids has given a good response then start a reducing regime with the aim of getting the patient off steroids.
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73
Q

What is the DON’T STOP mnemonic for long term steroid use?

A
  • DON’T –Make them aware that they will becomesteroid dependentafter 3 weeks of treatment and should not stop taking the steroids due to the risk ofadrenal crisisif steroids are abruptly withdrawn
  • S–Sick Day Rules: Discuss increasing the steroid dose if they become unwell (“sick day rules”)
  • T–Treatment Card: Provide asteroid treatment cardto alert others that they are steroid dependent in case they become unresponsive
  • O–Osteoporosis prevention: Considerosteoporosisprophylaxis whilst on steroids withbisphosphonatesandcalcium and vitamin Dsupplements
  • P–Proton pump inhibitor: Considergastric protectionwith aproton pump inhibitor (e.g. omeprazole)
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74
Q

What is Sjorgen syndrome?

A
  • A systemic autoimmune disorder affecting the exocrine glands resulting in dry mucosal surfaces, especially the lacrimal, vaginal and salivary glands.
  • Primary Sjogren’s is where the condition occurs in isoloation.
  • Secondary Sjogren’s **is where it occurs related to SLE or Rheumatoid arthritis.
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75
Q

What are the risk factors for Sjorgen syndrome?

A
  • Gender → Female > Male
  • SLE
  • Rheumatoid Arthritis
  • Systemic Sclerosis
  • HLA class 2 marker
  • Age → Peaks in 20s to 30s and then again after menopause
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76
Q

What are the clinical manifestation of Sjorgen syndrome?

A
  • Fatigue.
  • Dry eyes: Keratoconjunctivitis Sicca.
    • Inflammation of cornea and conjunctiva.
    • Blurring of vision, itchiness, redness and burning.
  • Dry mouth: Xerostomia.
    • Difficulty tasting and swallowing.
    • Cracks and Fissures in the mouth.
  • Vaginal dryness.
  • Vasculitis - most common beyond exocrine glands.
  • Dental caries - severe and may lead to loss of all teeth.
  • Arthralgia
  • Raynaud’s, Myalgia
  • Sensory Polyneuropathy
  • Recurrent Episodes of Parotitis
  • Renal tubular acidosis (usually subclinical)
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77
Q

What are the investigations for Sjorgen syndrome?

A
  • Schirmer’s test
    • Involves inserting a folded piece of filter paper under the lower eyelid with a strip hanging out over the eyelid
    • This is left in for 5 minutes and the distance along the strip hanging out that becomes moist is measured
    • The tears should travel 15mm in a healthy young adult.
    • A result of less than 10mm is significant.
  • Anti-RO and Anti-LA found in 90% of patients with Sjogren’s
    • not specific found in other conditions e.g. Lupus
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78
Q

What are the differential diagnoses for Sjorgen syndrome?

A
  • SLE
  • Rheumatoid arthritis
  • Hep C
  • System sclerosis
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79
Q

What is the management for Sjorgen syndrome?

A
  • Artificial tears
  • Artificial saliva
  • Vaginal lubricants
  • Hydroxychloroquine is used to halt the progression of the disease.

Additional presentations
- Vasculitis → Corticosteroids.
- Renal tubular acidosis → Potassium repletion.
- Neuropathy → IV immunoglobulins.

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80
Q

What are the complications for Sjorgen syndrome?

A
  • Eye infections
  • Dental cavities and candida infections
  • Vaginal problems: candidiasis and sexual dysfunction
  • Very rare causes:
    • Pneumonia and bronchiectasis
    • Non-Hodgkins lymphoma
    • Peripheral neuropathy
    • Vasculitis
    • Renal impairment.
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81
Q

What is antiphospholipid syndrome?

A

Antiphospholipid syndrome is the association of antiphospholipid antibodies (lupus anticoagulant, anticardiolipin antibody, and/or anti-beta2-glycoprotein I) with a variety of clinical features characterised by thromboses (arterial and venous) and pregnancy-related morbidity.

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82
Q

What are the clinical manifestations of antiphospholipid syndrome?

A
  • Thrombosis
  • Miscarriage
  • Livedo-reticularis
  • Thrombocytopenia
  • Ischaemic stroke, TIA, MI - arteries
  • Deep vein thrombosis, Budd-chiari syndrome - veins
  • Valvular heart disease, migraines, epilepsy
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83
Q

What are the investigations for antiphospholipid syndrome?

A

A persistently positive test (positive on at least two occasions more than 12 weeks apart) in one or more of these tests, along with clinical features is needed to diagnose APS
- Anticardiolipin test
- Lupus anticoagulant test
- Anti-B2-glycoprotein I test

  • Antinuclear antibody, double stranded DNA -> +ive in SLE
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84
Q

What is the management for antiphospholipid syndrome?

A
  • Low-molecular weight Heparin e.g. Dalteparin or Enoxaparin - FIRST LINE
  • Long term warfarin to minimise thrombosis - contraindicated for pregnancy
  • Prophylaxis:
    • Aspirin or Clopidogrel for people with aPL, especially those with a high IgG aPL
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85
Q

What is Scleroderma?

A
  • Also known as systemic sclerosis.
  • A multi-system autoimmune disease characterised by functional and structural abnormalities of small blood vessels, fibrosis of skin and internal organs and prosecution of auto-antibodies.
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86
Q

What are the first clinical manifestations of scleroderma?

A
  • First symptoms are usually seen in the fingers:
    • Skin becomes tight.
    • Fingers become stiff and inflexible.
    • Symptoms of Raynaud’s (fingers turn cold and blue in response to minor triggers).
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87
Q

What are the later clinical manifestations of scleroderma?

A
  • Later features:
    • Tightening of skin elsewhere (notably on the face)
    • Tightening around the lips, shrinking the opening to the mouth
    • General fatigue
    • Joint and muscle pain
    • Ulceration secondary to skin tightening so much it tears and cannot heal
    • Vascular disease, including coronary artery disease
    • Pulmonary fibrosis
    • Pulmonary artery hypertension (PAH)
    • Renal failure (“scleroderma renal crisis”)
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88
Q

What are the investigations for scleroderma?

A
  • Serum autoantibodies - positive ANAs in >90% of patients.
  • FBC
  • U&E
  • ESR/CRP
  • Complete pulmonary function tests
  • ECG
  • CXR
  • Barium swallow.
    • Could be done for exclusion of differentials or extent of current damage.
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89
Q

What is the management for scleroderma?

A
  • Goals of treatment are based on distribution of organ involvement.
  • No cure, disease is generally progressive.
  • Immunosuppressants are used to slow disease progression.
  • Symptomatic treatment
    • Raynaud’s phenomenon
      • Hand warms
      • Calcium channel blockers e.g. Nifedipine.
      • Endothelia receptor antagonist.
    • Topical skin emollients.
    • ACE inhibitors → prevention of renal crisis.
    • Nutritional supplements - Malabsorption.
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90
Q

What is CREST syndrome?

A

Limited CutaneousSystemic Scleroderma (CREST Syndrome)
- C - CALCINOSIS - hard, calcified nodules under the skin
- R - RAYNAUD’S PHENOMENON
- E - ESOPHAGEAL DYSMOTILITY
- S - SCLERODACTYLY - thickening of the skin of the hands and feet
- T - TELANGIECTASIA - spider veins

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91
Q

What is intervertebral disk disease?

A
  • Intervertebral disk disease is a common condition characterised by the breakdown (degeneration) of one or more of the discs that separate the bones of the spine (vertebra), causing pain in the back or neck and frequently in the legs and arms.
  • Discs in the lower lumbar spine are most commonly affected.
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92
Q

What is acute disk disese?

A

Progressive intervertebral disc breakdown leading to prolapse of the intervertebral disc resulting in acute back pain (lumbago).

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93
Q

What are the risk factors for acute disc disease?

A
  • Genetic predisposition
  • Advanced age
  • Menopause
  • Repeated spinal trauma
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94
Q

What are the clinical manifestations of acute disc disease?

A
  • Sudden onset of severe back pain → often following a strenuous activity.
  • Decreased range of motion.
  • Tingling, paraesthesia and numbness.
  • Muscle weakness and atrophy.
  • Muscle spasm leads to a sideways tilt when standing.
  • Decreased tendon reflexes.
  • The radiation of the pain and the clinical findings depends on the disc affected → the lower three discs are most commonly affected.
  • Headaches, dizziness, vertigo
95
Q

For an S1 root lesion: where would pain occur? What reflex would be lost? and what other sign would be seen?

A
  • Pain would be located in the buttock down the back of the thigh to the ankle/foot.
  • Ankle jerk reflex would be lost.
  • There would be diminished straight leg raising.
96
Q

For an L5 root lesion: where would pain occur? What reflex would be lost? and what other sign would be seen?

A
  • Pain would be located in the buttock to the lateral aspect of the leg and the top of the foot.
  • No reflex lost.
  • There would be diminished straight leg raising.
97
Q

For an L4 root lesion: where would pain occur? What reflex would be lost? and what other sign would be seen?

A
  • Pain would be located in the lateral aspect of the thigh to the medial side of the calf.
  • Knee jerk reflex is lost.
  • There is a positive femoral stretch test.
98
Q

What are the investigations for acute disc disease?

A
  • X-Rays are often normal - however can detect fracture.
  • MRI
    • Evaluates spinal canal
    • Detects annular tears
    • Increased signal may indicate disc dehydration
    • If surgery is being considered
99
Q

What is the management for acute disc disese?

A
  • Acute stage
    • Bed rest on a firm mattress and analgesia (NSAIDs) and local/epidural corticosteroid injection (severe disease).
  • Surgery only for severe or increasing neurological impairment e.g. foot drop or bladder symptoms.
    • Corpectomy - vertebral portion removed to enlarge intervertebral space.
    • Discectomy - herniated disc portion removed.
    • Nerve root injection.
    • Intervertebral disc arthroplasty - replacement with artificial discs
    • Laminotomy - lamina removal to relieve nerve root pressure.
  • Recovery phase
    • Physiotherapy to help correct posture and restore movement.
100
Q

What are the complications of acute disc disease?

A
  • Spine collapse
  • Disc herniation
  • Compression fracture
  • Bony spur growth
  • Neurological deficit
  • Myelopathy
  • Vertebral artery compression
101
Q

What is chronic disc disease?

A

Associated with degenerative disc changes in the lower lumbar discs and facet joints (joining the vertebral bones together).

102
Q

What are the clinical manifestations of chronic disc disease?

A
  • Pain is usually of the mechanical type i.e. aggravated by movement.
  • Sciatic radiation may occur with pain in the buttocks radiating into the posterior thigh.
  • Usually the pain is long-standing and there is no cure.
103
Q

What is the management for chronic disc disease?

A
  • NSAIDs, physiotherapy and weight reduction may be useful.
  • Surgery can be done when pain arises from a single identifiable level → fusion at this level with decompression of the affected nerve root.
104
Q

What is cervical spondylitis?

A

Osteoarthritis of the spine, which includes the spontaneous degeneration of either disc or facet joints.

105
Q

What are the signs of cervical spondylitis?

A
  • Limited neck movement
  • Neck flexion may produce tingling down the spine (Lhermitte’s sign) - does not differentiate between a cord or a rot issue.
  • Root compression presents with:
    • Pain in arms or fingers at the level of the compression, with numbness and dull reflexes.
    • Lower motor neurone weakness and wasting of the muscles innervated by the affected root.
    • Weak clumsy hands.
    • Gait disturbance .
106
Q

What are the symptoms of cervical spondylitis?

A
  • Neck stiffness
  • Crepitis (crunching) on moving the neck.
  • Stabbing or dull arm pain.
  • Forearm / wrist pain.
107
Q

What are the motor/sensory deficits and pain patterns observed in C5 (C4/C5 disc) nerve root spondylitis?

A
  • Weak deltoid and supraspinatus.
  • Decrease supinator jerks
  • Numb elbow
  • Pain in neck and shoulders that radiates down the front of the arm to the elbow.
108
Q

What are the motor/sensory deficits and pain patterns observed in C6 (C5/C6 disc) nerve root spondylitis?

A
  • Weak biceps and brachioradialis.
  • Decreased bicep jerk
  • Numb thumb and index finger
  • Pain in the shoulder which radiates down to the arm and the elbow.
109
Q

What are the motor/sensory deficits and pain patterns observed in C7 (C6/C7 disc) nerve root spondylitis?

A
  • Weak triceps and finger extension
  • Decreased triceps jerk
  • Numb middle finger
  • Pain in upper arm and dorsal forearm
110
Q

What are the motor/sensory deficits and pain patterns observed in C8 (C7/C8 disc) nerve root spondylitis?

A
  • Weak finger flexors and in the small muscles of the hand
  • Number 5th and ring finger
  • Pain in upper arm and medial forearm
111
Q

What are the investigations for cervical spondylitis?

A

MRI

112
Q

What is the management for cervical spondylitis?

A
  • Analgesia
  • Encourage gentle activity
  • Cervical collars for brief painful episodes
  • If no improvement consider:
    • Epidural
    • Surgical decompression
113
Q

What is mechanical lower back pain?

A

Mechanical means that the source of the pain may be in your spinal joints, discs, vertebrae, or soft tissues.

114
Q

What are the risk factors for mechanical lower back pain?

A
  • Gender → Female > Male
  • Increasing age
  • Pre-existing chronic widespread pain - fibromyalgia
  • Psychosocial factors e.g. high levels of psychosocial distress, poor self-related health, smoking and dissatisfaction with work.
115
Q

What are the clinical manifestations of mechanical lower back pain?

A
  • Variety of presentations depending on pathology:
    • Back is stiff
    • Scoliosis (spine twists and curves to a side) may be present when patient is standing
    • Muscular spasm is visible and palpable and causes local pain and tenderness - lessens when sitting or lying
    • Pain is often unilateral (but can be bilateral) and helped by rest
    • Pain usually worse in the evening
    • Exercise aggravates pain
    • Morning stiffness is absent
    • Sudden onset
    • Episodes are generally short-lived and self-limiting - but high chance of recurrence once individual has experienced one episode
116
Q

What are the investigations for mechanical lower back pain?

A
  • MRI - imagine modality of choice.
  • Bone scans
  • Examine patient to exclude pathologies e.g. nerve root lesions affecting reflexes
  • Spinal X-rays - ONLY done if there are red flags for serious pathology.
  • Arrange specific tests if pathology suspected…
117
Q

What are the red-flags that warrant an X-ray when a patient has mechanical lower back pain?

A
  • Age of onset <20 or >55 yrs
  • Acute onset in elderly people
  • Constant or progressive pain
  • Nocturnal pain
  • Worse pain on being supine
  • Fever, night sweats, weight loss
  • History of malignancy
  • Abdominal mass
  • Thoracic back pain
  • Morning stiffness
  • Bilateral or alternating leg pain (points to cauda equina compression, can lead to bladder and bowel incontinence- a surgical emergency!)
  • Neurological disturbance e.g. sciatica
  • Sphincter disturbance (may signify acute cord compression!)
  • Current or recent infection
  • Immunosuppression e.g. steroids or HIV
  • Leg claudication or exercise-related leg weakness/ numbness (spinal stenosis)
  • Violent trauma e.g. fall from height or road traffic accident (RTA)
118
Q

What are the differential diagnoses for mechanical lower back pain?

A
  • Polymyalgia rheumatica (PMR): in elderly, ESR and CRP will distinguish this from mechanical back pain
  • Sinister causes of back pain e.g. malignancy, infection or inflammatory causes. Must be excluded with spinal x-ray.
119
Q

What is the supportive management for mechanical lower back pain?

A
  • Analgesia e.g. paracetamol, NSAIDs, codeine
    • Consider low dose amitriptyline or duloxetine if these fail.
  • Combined with physiotherapy and exercise programmes, if no improvement seen.
  • Acupuncture may be considered
  • Patient should avoid excessive rest
  • Address psychosocial issues
  • Re-education in lifting and exercises to prevent further attacks of pain
  • Comfortable sleeping position using a mattress of medium (not hard) firmness
120
Q

What other management can be given for mechanical lower back pain?

A
  • Refer to pain clinic or surgical options for any intractable symptoms
  • Urgent referral if any neurosurgical emergencies identified e.g. acute cord compression or acute cauda equina compression
    • Treatment may involve laminectomy, radiotherapy, decompression.
121
Q

What is muscular dystrophy?

A
  • Muscular dystrophy is an umbrella term for genetic conditions that cause gradual weakening and wasting of muscles.
  • The main type of muscular dystrophy is Duchenne’s muscular dystrophy.
122
Q

Name 7 types of muscular dystrophy?

A
  • Duchenne’s muscular dystrophy
  • Becker’s muscular dystrophy
  • Myotonic dystrophy
  • Facioscapulo-humeral muscular dystrophy
  • Oculo-pharyngeal muscular dystrophy
  • Limb-girdle muscular dystrophy
  • Emery-Dreifuss muscular dystrophy
123
Q

What are the clinical manifestations of Duchenne muscular dystrophy?

A
  • Usually presents around 3 – 5 years
  • Weakness in the muscles around pelvis. The weakness tends to be progressive and eventually all muscles will be affected.
  • Children start walking later than average
  • Waddling gait
  • Tend to develop calf pseudohypertrophy: visibly enlarged calves which are large because of fat and fibrotic tissue rather than muscle tissue.
  • Usually wheelchair bound by their teenager years
  • Gowers sign
124
Q

What are the clinical manifestations of Becker’s muscular dystrophy?

A
  • Usually presents around 8 – 12 years
  • Similar presentation to Duchennes
  • Some patient require wheelchairs in their late 20s or 30s. Others able to walk with assistance into later adulthood.
125
Q

What are the clinical manifestations of Myotonic dystrophy?

A
  • Usually presents in adulthood
  • Progressive muscle weakness
  • Prolonged muscle contractions
  • Cataracts
  • Cardiac arrhythmias
126
Q

What are the clinical manifestations of Facioscapulo-humeral muscular dystrophy?

A
  • Usually presents in childhood
  • Weakness around the face, progressing to the shoulders and arms
  • Sleeping with eyes slightly open
  • Weakness in pursing their lips
  • Inability to blow their cheeks out without air leaking from their mouth
  • Foot drop
  • Scapular winging (shoulder blade sticks out)
  • Scoliosis
  • Anterior axillary folds
  • Horizontal clavicles
  • Usually need wheelchair by age 40
127
Q

What are the clinical manifestations of Oculo-pharyngeal muscular dystrophy?

A
  • Usually presents in late adulthood
  • Weakness of the ocular muscles (around the eyes)
    • Bilateral ptosis and restricted eye movement
  • Weakness of the pharynx (around the throat)
    • Swallowing problems
  • Muscles around the limb girdles are also affected to varying degrees
128
Q

What are the clinical manifestations of Limb-girdle muscular dystrophy?

A
  • Usually presents in teenage years
  • Progressive weakness around the limb girdles (hips and shoulders)
129
Q

What are the clinical manifestations of Emery-Dreifuss muscular dystrophy?

A
  • Usually presents in childhood
  • Contractures, most commonly in the elbows and ankles. Contractures are shortening of muscles and tendons that restrict the range of movement in limbs
  • Progressive weakness and wasting of muscles, starting with the upper arms and lower legs
130
Q

What are the investigations for muscular dystrophy?

A

GOLD STANDARD - Genetic testing: diagnosis can be confirmed by looking for mutations in dystrophin
- High creatine kinase level
- Muscle biopsy: staining for dystrophin
- Electromyogram: distinguish between neuropathic and myopathic pathology
- Gait assessment
- Muscle strength test

131
Q

What is the management for muscular dystrophy?

A

No curative treatment for muscular dystrophy. Management is aimed at allowing the person to have the highest quality of life for the longest time possible.

  • Occupational therapy
  • Physiotherapy
  • Medical appliances (such as wheelchairs and braces)
  • Surgical and medical management of complications e.g. spinal scoliosis and heart failure.
  • Oral steroids have been shown to slow the progression of muscle weakness
  • Creatine supplementation can give a slight improvement in muscle strength
  • Genetic counselling for patients who want to have children
132
Q

What are the complications of muscular dystrophy?

A
  • Respiratory failure because of a weak diaphragm
  • Scoliosis
  • Dilated cardiomyopathy and arrhythmias: dystrophin protein is also expressed in heart muscle.
133
Q

What us Marfan syndrome?

A

A genetic disorder that results in defective connective tissue. This can affect the skeleton, heart, blood vessels, eyes and lungs.

134
Q

What are the clinical manifestations of Marfan syndrome?

A
  • Features may not always be present and will vary in severity. Symptoms show with age, but there may sometimes be symptoms present at birth (early onset).
    • Tall stature, long arms and long legs due to excessive long bone growth
    • Arachnodactyly - long fingers and toes
    • Pectus excavatum (chest sinks in) or pectus carinatum (chest points out)
    • Scoliosis
    • Inability to extend elbows to 180 degrees
    • Flexible joints (hypermobility)
    • Downward slant of the eyes
    • Narrow high-arch palate - crowds teeth
    • Stretch marks
135
Q

What are the investigations of Marfan syndrome?

A

Diagnosis is based on clinical features (diagnose if >2 features)

  • Lens dislocation
  • Aortic dissection/ dilation
  • Dural ectasia - widening of the dural sac
  • Skeletal features e.g. long arms, arachnodactyly
  • Pectus deformity
  • Scoliosis
  • Pes planus - flat feet

Other
- MRI for Dural ectasia

136
Q

What are the differential diagnoses for Marfan syndrome?

A
  • Ehlers-Danlos syndrome
  • Erdheim’s deformity - presents with dilation of aortic root
  • Homocystinuria - body cannot process amino acids properly. Presents similar to Marfan syndrome
137
Q

What is the management for Marfan Syndrome?

A
  • General
    • Physiotherapy can be helpful in strengthening joints and reducing symptoms arising from hypermobility.
    • Genetic counselling is important in considering the implications of having children that may be affected by the condition
  • Eye-related
    • Replacement of dislocated lens with artificial lens
  • Cardiovascular-related
    • Lifestyle changes, such as avoiding intense exercise and avoiding caffeine and other stimulants - minimise stress to help cardiac complications
    • Beta blockers can also be used to stop aortic dilation
    • Angiotensin receptor blocker e.g. losartan - decreases tgf-beta signalling and can also decrease aortic dilation
    • Surgical repair of wide aorta.
138
Q

What are the complications of Marfan syndrome?

A
  • Retinal detachment and lens dislocation
  • Joint dislocations and pain due to hypermobility
  • Bulla formation on lungs - leading to pneumothorax
  • Cardiovascular:
    • Aortic dilation causing aortic valve insufficiency
    • Cystic medial necrosis (tunica media of aortic wall degenerates)
    • Increased risk of aortic dissection, aneurysm and rupture
    • Mitral valve prolapse
    • Aortic valve prolapse
139
Q

What is Ehlers-Danlos syndrome?

A
  • Ehlers-Danlos syndrome (EDS) is a group of connective tissue disorders caused by mutations of connective tissue proteins, with collagen being the most commonly affected.
140
Q

What are the risk factors for Ehlers-Danlos syndrome?

A

Family history (most common subtypes are autosomal dominant)

141
Q

What are the clinical manifestations of Ehlers-Danlos syndrome?

A
  • Musculoskeletal
    • Joint hypermobility and pain
    • Recurrent dislocation
    • Scoliosis and spinal pain
  • Skin
    • Hyper-elasticity
    • Easy bruising
    • Atrophic skin
142
Q

What are the investigations for Ehlers-Danlos syndrome?

A
  • Beighton score is used to assess the extend of hypermobility. Used for a clinical diagnosis.
  • Examine joints and skin
  • Genetic testing to identify mutation
  • Echocardiogram
  • Spine X-ray
143
Q

What is the management for Ehlers-Danlos syndrome?

A
  • Supportive care:
    • Physiotherapy
    • Orthopaedic instrument e.g. bracing, wheelchair and casting
    • Lifestyle advice: avoid contact sports and heavy labour to reduce the risk of tissue damage and joint dislocation
    • Analgesia
    • Psychological input: due to chronic pain and the impact on quality of life, patients may develop mental illness
144
Q

What are the complications of Ehlers-Danlos syndrome?

A
  • Cardiovascular
    • Mitral valve prolapse: mid-systolic click and late-systolic murmur
    • Aortic dissection
    • Abdominal aortic aneurysms
    • Organ rupture
  • Other
    • Subarachnoid haemorrhage
    • Angioid retinal streaks
    • Abdominal hernia
    • Gastro-oesophageal reflux disease
    • Degenerative arthritis due to recurrent joint dislocation
    • Depression and anxiety
145
Q

What is Rheumatoid arthritis?

A
  • Rheumatoid arthritis (RA) is a chronic, systemic inflammatory disease.
  • This leads to a deforming, symmetrical inflammatory arthritis of the small joints which progresses to involve larger joints and other organs of the body, e.g. skin and lungs.
146
Q

What are the risk factors for rheumatoid arthritis?

A
  • Gender → Female > Male
  • Age → Peak incidence of onset between 50-60 years old
  • Smoking
  • Family history
  • Infections
  • Hormones: increased risk post-menopause, potentially due to a reduction in oestrogen levels
147
Q

What are the signs of rheumatoid arthritis?

A
  • Symmetrical polyarthritis: (on both sides of body)
    • Swollen, warm and tender small joints of the hands and feet (MCP, PIP, MTP)
    • Progresses to larger joints (shoulder, elbow, knee, ankle)
  • Boutonniere deformity: PIP flexion and DIP hyperextension
  • Swan-neck deformity: PIP hyperextension and DIP flexion
  • Z-thumb deformity: hyperextension of the thumb IP joint with flexion of the MCP joint.
  • Ulnar deviation of the fingers
  • Popliteal cyst: synovial sac bulges posteriorly to the knee
  • Rheumatoid nodules - lumps that can appear under skin and other areas e.g. lungs, hearts, eye
148
Q

What are the symptoms of rheumatoid arthritis?

A
  • Morning stiffness: > 30 mins and improves throughout the day
  • Malaise
  • Myalgia
  • Low-grade fever
148
Q

What are the symptoms of rheumatoid arthritis?

A
  • Morning stiffness: > 30 mins and improves throughout the day
  • Malaise
  • Myalgia
  • Low-grade fever
149
Q

What are the investigations for rheumatoid arthritis?

A
  • ESR and CRP
  • Rheumatoid factor: present in 70% of patients
  • Anti-cyclic citrullinated peptide (anti-CCP):present in 80% of patients
  • Joint X-rays:X-rays of the affected joints can aid diagnosis
  • US and MRI can also be used.
150
Q

How is a diagnosis of rheumatoid arthritis made?

A

A score of 6 or over/ 10 needed for diagnosis of RA.

  • 1 large joint - 0
  • 2-10 large joints - 1
  • 1-3 small joints - 2
  • 5-10 small joints - 3
  • > 10 joints (>1 small) - 5
  • Negative RF and anti-CCP - 0
  • Low RF or anti-CCP - 2
  • High RF or anti-CCP - 3
  • Normal CRP and ESR - 0
  • Abnormal CRP and ESR - 1
  • <6 weeks - 0
  • > 6 weeks - 1
151
Q

What are the differential diagnoses for rheumatoid arthritis?

A
  • Psoriatic arthritis
  • Infectious arthritis
  • Gout
  • SLE
  • Osteoarthritis
152
Q

What is the primary management for rheumatoid arthritis?

A
  • NSAID: low dose NSAID (e.g. ibuprofen) to cover the period between symptom onset and rheumatology referral
  • Refer to specialist care
  • Physiotherapy and occupational therapy
153
Q

What is the secondary management for rheumatoid arthritis?

A
  • FIRST LINE - DMARD e.g. Methotrexate, Leflunomide, Sulfasalazine, Hydroxychloroquine
  • Biologics e.g. Abatacept, Rituximab, Etanercept, Infliximab
  • NSAIDs and Glucocorticoids for management of flares.
154
Q

How is rheumatoid arthritis monitored?

A
  • FBC, LFTs and U&Es measured weekly until therapy stabilised, and 2-3 monthly thereafter
  • CRP is measured monthly as well as disease activity (DAS-28 score)
  • Also do an annual assessment
155
Q

What are the complications of rheumatoid arthritis?

A
  • Rheumatoid nodules
  • Keratoconjunctivitis sicca
  • Episcleritis
  • Scleritis
  • Corneal ulceration
  • Keratitis
  • Pulmonary nodules
  • Pleurisy
  • Pleural effusion
  • Interstitial lung disease
  • Caplan’s syndrome: lung nodules with occupational coal exposure
  • Bronchiolitis obliterans
  • Bronchiectasis
  • Anaemia of chronic disease
  • Atlanto-axial subluxation
  • Tendon rupture
  • Carpal tunnel syndrome
  • Secondary amyloidosis
  • Felty’s syndrome
156
Q

What is Gout?

A

Gout is an inflammatory arthritis caused by deposition of monosodium urate crystals within joints, most commonly the first metatarsophalangeal joint (MTP).

157
Q

What are the risk factors for Gout?

A
  • Incidence increases with age. Peak incidence between 40-60 years old.
  • Gender → Male > Female
  • Post-menopausal women
  • Family history of gout
  • Diet high in purines
  • Alcohol consumption
  • Diuretics
  • Impaired renal function
  • DM - high insulin levels lower urate excretion
  • Ischaemic heart disease and hypertension
158
Q

What are the signs of Gout?

A
  • Joint inflammation: tenderness, erythema and swelling
  • Mainly monoarticular but can be oligoarticular (≤ 4 joints):
    • 1st MTPmost commonly affected in a first presentation (70%); previously known as podagra
    • The ankle, wrist, knee and small joints of the hand are also commonly affected
  • Gouty tophi: nodular masses of urate crystals form under the skin, usually as a late complication
    • Usually affect fingers, ears, and elbows
159
Q

What are the symptoms of Gout?

A
  • Red, tender, hot, and swollen joint.
  • Joint stiffness
  • Rapid onset severe joint pain
160
Q

What are the investigations for Gout?

A
  • Joint aspiration:needle-shaped crystals with negative birefringence under polarised microscopy confirm the diagnosis.
  • Serum urate
  • Joint X-ray - often normal
161
Q

What are the differential diagnoses for Gout?

A
  • Septic arthritis
  • Reactive arthritis
  • Haemarthrosis
  • Calcium pyrophosphate deposition
  • Rheumatoid arthritis
162
Q

What is the acute management for Gout?

A

Acute management
- NSAIDs - FIRST LINE
- Colchicine when above is contraindicated.
- Corticosteroids - SECOND LINE
- Ice and rest

163
Q

What is the general management for Gout?

A
  • Lifestyle advice - food low in purine, lose weight, avoid alcohol
  • Xanthine oxidase inhibitors - reduces uric acid
    • Allopurinol - 1st line
    • Febuxostat - 2nd line
  • Uricosuric agent - e.g. sulfinpyrazone, probenecid, benzbromarone can be used if xanthine oxidase inhibitor not working.
164
Q

What are the complications of Gout?

A

Urate nephrolithiasis: there is an association between gout and urate renal stones due to hyperuricaemia

165
Q

What is Calcium Pyrophosphate Deposition Disease?

A
  • Also known as Pseudogout.
  • Pseudogout is a form of inflammatory arthritis caused by deposition of calcium pyrophosphate crystals in the synovium.
166
Q

What are the risk factors for pseudogout?

A
  • Increasing age: the greatest known risk factor for pseudogout
  • Females over 70 years old
  • Previous joint trauma
  • Hyperparathyroidism
  • Haemochromatosis
  • Acromegaly
  • Wilson’s disease
  • Hypomagnesaemia
  • Hypophosphatemia
167
Q

What are the signs of pseudogout?

A
  • Joint inflammation: pain, erythema and swelling
  • Signs can be monoarticular (1 joint) or polyarticular (several joints)
168
Q

What are the symptoms of pseudogout?

A
  • Rapid onset severe joint pain: knee, shoulder and wrist are most commonly affected
  • Joint stiffness
169
Q

What are the investigations for pseudogout?

A
  • Joint aspiration:weakly-positively birefringent rhomboid-shaped crystals confirms diagnosis.
  • Joint X-ray - chondrocalcinosis is seen in 40% of cases.
170
Q

What are the differential diagnoses for pseudogout?

A
  • Gout
  • Septic arthritis
  • Rheumatoid arthritis
  • Osteoarthritis
171
Q

What is the management for pseudogout?

A

Acute
- Anti-inflammatory:NSAIDs or colchicine, particularly in polyarticular disease
- Corticosteroids

Chronic
- DMARDs: e.g. methotrexate and hydroxychloroquine

172
Q

What are the complications of pseudogout?

A

Damage to the joint and loss of function over time.

173
Q

What is ankylosing spondylitis?

A
  • Ankylosing spondylitis (AS) is a chronic progressive inflammatory arthropathy. Mainly affects the spine and causes progressive stiffness and pain.
  • Ankylosis = abnormal stiffening and immobility of joint due to fusion of bones
174
Q

What are the risk factors for ankylosing spondylitis?

A
  • HLA-B27 positive
  • Family history of ankylosing spondylitis
  • Gender → Male (3x) > Female
175
Q

What are the key presentations for ankylosing spondylitis?

A
  • Affects sacroiliac joint and joints of vertebral column. Asymmetrical joint pain - normally oligoarthritic (1 or 2 joints)
  • Symptoms present over 3 months. Symptoms will fluctuate with flares and periods of improvement.
176
Q

What are the signs of ankylosing spondylitis?

A
  • Enthesitis - inflammation at point of insertion of tendons and ligaments in bones
  • Dactylitis - inflammation of entire digit
  • Bamboo spine on x-ray due to fusion of the joints
177
Q

What are the symptoms of ankylosing spondylitis?

A
  • Pain and stiffness of joints
  • Lower back pain
  • Sacroiliac pain (buttock region)
  • Pain worst at night and in the morning (>30 minutes of stiffness in morning)
  • Pain worst with rest and improves with movement
  • Systemic symptoms e.g. weight loss and fatigue
  • Chest pain - related to costovertebral and costosternal joints
  • Investigations
178
Q

What are the investigations for ankylosing spondylitis?

A
  • FBC
  • CRP and ESR
  • Genetic testing for HLA-B27
  • X-ray of spine and sarcum
    • Bamboo spine
    • Squaring of vertebral bodies
    • Subchondral sclerosis
    • Subchondral erosions
    • Syndesmophytes
    • Ossification
    • Fusion
  • MRI of spine if x-ray is normal
179
Q

What is Schober’s test?

A
  • General examination of spine (especially lumbar spine) to assess mobility.
  • Patient stands straight. Find L5 vertebrae and mark a point 10cm above this and 5cm below. Ask patient to bend forward, and measure distance between two points.
  • If distance between the two points is less than 20cm, this indicates restriction in the lumbar movements.
180
Q

What are the differential diagnoses for ankylosing spondylitis?

A
  • Osteoarthritis
  • Psoriatic arthritis
  • Reactive arthritis
  • Vertebral fracture
181
Q

What is the management for ankylosing spondylitis?

A
  • NSAIDs e.g. ibuprofen or naproxen
  • Steroids - used during flares
  • Anti-TNF e.g. etanercept or monoclonal antibodies against TNF e.g. infliximab, adalimumab
  • Monoclonal antibodies targeting IL-7 - e.g. Secukinumab
  • Physiotherapy
  • Lifestyle changes - smoking
  • Bisphosphonates
  • Surgery for fractures
182
Q

What are the complications for ankylosing spondylitis?

A
  • Vertebral fractures
  • Osteoporosis
  • Anaemia
  • Anterior uveitis (eye inflammation)
  • Aortitis (inflammation of aorta)
  • Heart block - fibrosis of the heart conduction system
  • Restrictive lung disease - due to restrictive movement of chest wall
  • Pulmonary fibrosis - especially upper lobes of lungs
  • Inflammatory bowel disease
183
Q

What is psoriatic arthritis?

A

Psoriatic arthritis is a chronic inflammatory joint disease associated with psoriasis, although it can occur without arthritis.

184
Q

What are the risk factors for psoriatic arthritis?

A
  • Psoriasis
  • Family history of psoriasis or psoriatic arthritis
185
Q

What are the signs for psoriatic arthritis?

A
  • Joint tenderness, warmth and reduced range of motion
    • Typically affects DIP joints, rather than MCP/PIP joints in rheumatoid arthritis
  • Dactylitis: swelling of an entire digit
  • Enthesitis: inflammation of the plantar fascia and Achilles’ tendon (back of foot)
  • Psoriasis: psoriatic lesions, scalp and nail symptoms (pitting of nails, onycholisis - separation of nail from nail bed)
186
Q

What are the symptoms of psoriatic arthritis?

A
  • Joint pain and stiffness:
    • Symptoms worse in the morning and improve on movement is typical of an inflammatory arthropathic
  • Swollen fingers or toes
  • Back pain if axial skeleton involved
187
Q

What is Arthritis mutilans?

A
  • Most severe and least common form of psoriatic arthritis.
  • Deforming and destructive subtype
  • Occurs in the digits
  • Osteolysis around the phalynxes
  • Skin around the bones, folds in on itself as the bones get shorter (telescopic finger)
188
Q

What are the 5 types of psoriatic arthritis?

A
  • Symmetrical polyarthritis.
  • Asymmetric oligoarthritis
  • Distal arthritis
  • Spondlyoarthritis
  • Arthritis mutilans
189
Q

What are the investigations for psoriatic arthritis?

A
  • CRP and ESR - normal
  • RF - negative
  • anti-CCP - negative
  • Joint aspiration - no bacteria or crystals
  • X-ray
    • Erosion
    • Osteomyelitis
    • Pencil-in-cup deformity
    • Dactylitis
    • Periostitis
190
Q

What is the CASPAR criteria?

A

CASPAR criteria: diagnosis of psoriatic arthritis can be made if the patient scores > 2 points on the following:

  • History of psoriasis (2)
  • Psoriatic nail changes (1)
  • Rheumatoid factor negative (1)
  • History of dactylitis (1)
  • Radiological evidence (1)
191
Q

What are the differential diagnoses for psoriatic arthritis?

A
  • Rheumatoid arthritis
  • Gout
  • Reactive arthritis
  • Erosive osteoarthritis
192
Q

What is the management for psoriatic arthritis?

A
  • NSAIDs and physiotherapy: first-line options to reduce inflammation, improve range of motion and strengthen muscles
  • Intra-articular steroids - for intra-articular synovitis
  • DMARDs - methotrexate or sulfasalazine
  • Biologic agents: TNF-α inhibitors, such as etanercept or infliximab
  • Ustekinumab - used to treat psoriasis
193
Q

What are the complications for psoriatic arthritis?

A
  • Cardiovascular: ischemic heart disease and hypertension.
  • Aortitis
  • Amyloidosis
  • Conjunctivitis
  • Anterior uveitis
  • Methotrexate hepatotoxicity
194
Q

What is reactive arthritis?

A
  • Reactive arthritis refers to synovitis occurring due to a recent infective trigger. This is an autoimmune response to infection elsewhere in the body. This usually presents as acute monoarthritis.
  • Also known as Reiter syndrome/.
195
Q

What are the risk factors for reactive arthritis?

A
  • HLA-B27 gene
  • Gender → Male > Female
  • Preceding chlamydial or gastrointestinal infection
196
Q

What are the key presentations of reactive arthritis?

A
  • Acute, asymmetrical monoarthritis, typically in the lower leg.
  • Patients may present with triad of - urethritis, arthritis and conjunctivitis ‘Can’t see, pee or climb a tree’
197
Q

What are the signs of reactive arthrititis?

A
  • Warm and swollen joint
  • Iritis - swelling and irritation of eye
  • Keratoderma blenorrhagica - painless, red, raised plaques and pustules
  • Circinate balanitis - dermatitis of the head of the penis
  • Enthesitis - inflammation of the site where tendons and ligaments insert into the bone
  • Nail dystrophy
198
Q

What are the symptoms of reactive arthritis?

A
  • Warm, swollen, painful joint
  • Mouth ulcers
199
Q

What are the investigations for reactive arthritis?

A
  • ESR and CRP - elevated
  • Infectious serology
  • If diarrhoea - culture stool
  • Aspirate joint - MCS and crystal examination - to rule out septic arthritis and crytalopathies
  • Sexual health review
  • X-ray - may show enthesitis with periosteal reaction
200
Q

What are the differential diagnoses for reactive arthritis?

A
  • Septic arthritis
  • Gout
  • Pseudogout
  • Ankylosing spondylitis
  • Psoriatic arthritis
201
Q

What is the management for reactive arthritis?

A
  • Presume septic arthritis until excluded (antibiotics)
  • NSAIDs
  • Steroid injections
  • Systemic steroids

Chronic
- DMARDs e.g. methotrexate or sulfasalazine
- Anti-TNF

202
Q

What are the complications of reactive arthritis?

A
  • Bilateral conjunctivitis (non-infective)
  • Anterior uveitis
  • Circinate balanitis (dermatitis of the head of the penis)
  • Keratoderma blennorrhagia
203
Q

What is enteric arthritis?

A
  • A form of chronic inflammatory arthritis associated with occurrence of inflammatory bowel disease (Crohn’s and UC).
  • One in 5 people with IBD will develop enteropathic arthritis.
204
Q

What are the clinical manifestations of enteric arthritis?

A
  • Symptoms of inflammatory bowel disease
    • Constipation
    • Diarrhoea
    • Abdominal pain, cramps and distension
    • Nausea and vomiting
  • Arthritic symptoms of the joints
    • Joint pain, welling and stiffness
    • Uveitis
    • Enthetitis
205
Q

What are the investigations for enteric arthritis?

A
  • Inflammatory markers will be elevated.
  • Stool culture/endoscopy - used to provide a diagnosis of inflammatory bowel disease.
  • Arthrocentesis of synovial fluid in the joint.
  • X-Ray of the affected joints.
206
Q

What is the management for enteric arthritis?

A
  • Corticosteroids.
  • Disease-modifying anti-rheumatic drugs (DMARDs).
  • Anti-TNF-alpha.
207
Q

What are the 4 spondylarthropathies?

A
  • Ankylosing spondylitis
  • Psoriatic arthritis
  • Reactive arthritis
  • Enteric arthritis
208
Q

What are the 4 benign primary bone tumours?

A
  • Osteochondroma
  • Giant cell tumour
  • Osteoblastoma
  • Osteoid osteomas
209
Q

What are the 3 malignant primary bone tumours?

A
  • Osteosarcoma
  • Ewing sarcoma
  • Chondrosarcoma
210
Q

What are the most common sources of secondary bone tumours?

A
  • Breast cancer
  • Prostate cancer
  • Lung cancer
  • Thyroid cancer
  • Kidney cancer
211
Q

What are the clinical manifestations of bone tumours?

A
  • Local bone pain, swelling and fractures.
  • Worse pain at night
  • Dull-deep pain which is swollen and tender.
  • Press against spinal nerves
  • vascular necrosis
  • Pulmonary symptoms
  • Easy bruising
  • Mobility issues
  • Systemic symptoms
212
Q

What are the risk factors for primary bone tumours?

A
  • Previous chemotherapy
  • Previous cancer
  • Paget’s disease
  • benign bone lesions
213
Q

What are the investigations for primary bone tumours?

A
  • FIRST LINE - X-ray
  • GOLD STANDARD - Biopsy
  • FBC
  • ESR
  • ALP
  • Lactate dehydrogenase
  • Ca levels
  • U&E
  • CT of abdomen and pelvis
214
Q

What is the management for primary bone tumours?

A
  • General symptomatic management: analgesics and anti-inflammatory drugs. Biphosphonates may help.
  • Benign tumours: can be surgically removed to reduce pain and the risk of fractures.
  • Malignant tumours: radiotherapy, chemotherapy, hormone-therapy and surgery depending on the type and spread of the tumour.
215
Q

What is vasculitis?

A
  • Inflammation of a blood vessel (arteries or veins), which is characterised by the presence of an inflammatory infiltrate and destruction of the vessel wall.
  • Vessel wall destruction leads to aneurysm, rupture and stenosis: resulting in perforation and haemorrhage into tissues
216
Q

What is giant cell arteritis?

A
  • Inflammation of the lining of your arteries. Sometimes called temporal arteritis as it commonly affects the temporal branch of the carotid artery.
  • Often co-exists with Polymyalgia Rheumatica (PMR)
217
Q

What are the clinical manifestations of giant cell arteritis?

A
  • Malaise
  • Dyspnoea
  • Weight loss
  • Morning stiffness
  • Unequal or weak pulse

Due to arteries in the head being affected:
- Headache
- Scalp tenderness (pain when combing hair)
- Tongue/ jaw claudication - pain when chewing
- Amaurosis fugax - unable to see from one or both eyes
- Sudden unilateral blindness

218
Q

What are the investigations of giant cell arteritis?

A
  • Elevated ESR and CRP
  • Elevated ALP
  • Elevated platelets
  • Reduced Hb
  • ANCA -ve
219
Q

What is the diagnostic criteria for giant cell arteritis?

A

GCA can be diagnosed whenthree of the following criteriaare met:

  • Age at disease onset ≥50 years
  • New headache
  • Temporal artery abnormality
  • Elevated ESR(> 50 mm/hr)
  • Abnormal temporal artery biopsy(typical vasculitis features)
220
Q

What is the management for giant cell arteritis?

A
  • Steroids - dampen down immune response
    • Prednisolone
    • IV methylprednisolone if evolving visual loss
  • PPI
  • Ca2+ and Vitamin D
  • Bisphosphonate
221
Q

What is polyarteritis nodosa?

A

Necrotising vasculitis that causes aneurysms and thrombosis in medium sized arteries, leading to infarction in affected organs with severe systemic symptoms.

222
Q

What are the clinical manifestations of polyarteritis nodosa?

A
  • Fever, malaise, weight loss and myalgia
  • Hypertension
  • Haematuria
  • Proteinuria
  • Abdominal pain
  • GI bleeding
  • Skin lesions
  • Rash - Livedo reticularis (pink blue mottling caused by stasis of blood in skin venules)
  • Punched-out ulcers
  • Nodules
223
Q

What are the investigations for polyarteritis nodosa?

A
  • Bloods
    • Elevated WCC
    • Eosinophilia
    • Anaemia
    • Raised ESR and CRP
    • ANCA -ve
  • Renal or mesenteric angiogram - can see string of beads pattern due to fibrotic aneurysms
  • Renal biopsy
224
Q

What is the management for polyarteritis nodosa?

A
  • Steroids e.g. prednisolone in combination with immunosuppressive drugs e.g. azathioprine or cyclophosphamide
  • Control BP
  • Hep B should be treated after treatment with steroids
225
Q

What are the risk factors for Granulomatosiswith Polyangiitis?

A
  • Genetic risk
  • Infections
  • Certain medications
  • Alpha-1 antitrypsin
  • Environmental exposures
226
Q

What is Granulomatosiswith Polyangiitis (Wegener’s granulomatosis)?

A
  • A multi-system disorder of unknown causes characterised by necrotising granulomatous inflammation and vasculitis of small and medium vessels.
  • It classically affects: the upper respiratory tract, the lower respiratory tracts and the kidneys.
227
Q

What are the investigations for Granulomatosiswith Polyangiitis (Wegener’s granulomatosis)?

A
  • cANCA +ve
  • ESR and CRP raised
  • Raised WCC
  • Urinalysis - to look for proteinuria and haematuria
  • Biopsy of affected organs
228
Q

What is the management for Granulomatosiswith Polyangiitis (Wegener’s granulomatosis)?

A
  • Steroids combined with cyclophosphamide/ rituximab
  • Methotrexate/ azathioprine/ rituximab usually used for maintenance
  • Plasma exchange in patients with severe renal disease or pulmonary haemorrhage
229
Q

What is microscopic polyangiitis?

A

A necrotising vasculitis affecting small and medium vessels. (Similar to granulomatosis with polyangiitis).

230
Q

What are the clinical manifestations of microscopic polyangiitis?

A
  • Glomerulonephritis
  • Pulmonary haemorrhage (30%)
231
Q

What are the investigations of microscopic polyangiitis?

A

pANCA +ve

232
Q

What is the management for microscopic polyangiitis?

A
  • Steroids e.g. prednisolone combined with cyclophosphamide
  • Methotrexate, rituximab and azathioprine for maintenance

Phase 2a Sheffield Medicine (10 decks)

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