Orthopaedics & rheumatology Flashcards
What are spondyloarthritidies?
Group of inflammatory arthritidies affecting the spine and peripheral joints w/o production of RFs and associated with HLA-B27 allele. Are seronegative (no rheumatoid factor or anti-CCP).
Characteristics of spondyloarthritidies
Axial arthritis Sacroiliitis Assymetrical large joint arthritis Enthesitis Dactylitis Iritis IBD
Name 6 types of spondyloarthritidies
Ankylosing spondylitis Psoriatic arthritis Reactive arthritis Enteropathic arthritis Undifferentiated spondyloarthritis Childhood spondyloarthritis
Define ankylosing spondylitis
Low back pain and stiffness for over 3 months with limitation of spinal movement and limitation of chest expansion, leads to radiographical changes in the spine & sacroiliac joints. Seronegative. Associated with HLA-B27 gene.
Symptoms of ankylosing spondylitis
- Inflammatory back pain (early morning stiffness, improves with movement)
- Progressive loss of spinal movements
- Thoracic kyphosis and neck hyperextension
- Iritis / uveitis
- Enthesitis
- Presents in late teens/early 20s. Males more common. Positive family hx
Investigations for ankylosing spondylitis
- Pelvic X-ray shows: sacroiliitis (sclerosis, erosions, loss of joint space, fusion at sacroiliac joint)
- HLA-B27
- Spine x-ray : vertebra show corner erosions, squaring syndesmophytes and get ‘bamboo spine’
Management for ankylosing spondylitis
Exercise/physio NSAIDs Intra-articular steroid injections Anti-TNF if severe Bisphosphonates
Define psoriatic arthritis
inflammatory arthritis associated with psoriasis. Tests for rheumatoid factor usually negative. Arthritis can occur before the psoriasis. Affects small joints of the hands. See psoriatic nail changes.
rx for psoriatic arthritis
NSAIDs Sulfasalazine methotrexate ciclosporin anti-TNF
Define reactive arthritis
aseptic arthritis that occurs subsequent to an extra-articular infection, typically of the GI or GU tracts. Pain in knees, feet, toes, hips and ankles.
Bacteria that often are the cause of reactive arthritis
chlamydia trachomatis, campylobacter, salmonella, shigella and yersinia.
What are the 3 symptoms with Reiter’s syndrome
Reactive arthritis
Urethritis
Conjunctivitis / mucocutaneous lesions
Define enteropathic arthritis
Inflammatory arthritis associated with IBD
Define bursitis
Inflammation of a bursa (jelly like sack that contains a small amount of synovial fluid, they lie between a tendon and bone or skin to act as a friction buffer).
Common bursa that become inflamed in bursitis (5)
Suprapatellar - knee Sub-acromial (sub deltoid) - shoulder Trochanteric - hip Retrocalcaneal – back of the heel Olecranon – elbow
Symptoms of bursitis
Pain at site of bursa, worse on palpation
Active range of movement decreased
How is bursitis diagnosed?
Clinically
Rx for bursitis
- NSAIDs / paracetamol
- Corticosteroid injection
- Surgery to remove bursa (only in refractory cases)
(abx, surgical debridement if septic bursitis)
What is compartment syndrome?
A pathological condition characterised by elevated interstitial pressure in a closed osteofascial compartment that results in microvascular compromise (restriction of capillary blood flow).
Symptoms of compartment syndrome
Pain greater than clinical findings Pain on passive muscle stretch Warm, erythematous swollen limb Increased CRT Week / absent peripheral pulses
(6 P’s of limb ischaemia)
Rx for compartment syndrome
Elevate limb
Remove bandages/casts
Fasciotomy
Name the crystal arthropathies
Gout Pseudogout (calcium pyrophosphate dehydrate crystal deposition disease - CPPD OR chondrocalcinosis)
What is gout?
An inflammatory disease in which monosodium rate crystals deposit in joints making them red, hot, tender and swollen within hours
Risk factors for gout
Male
High consumption of purine rich foods e.g. red/organ meat, seafood
Alcohol
Use of diuretics, aspirin, ciclosporin, tacrolimus
Renal impairment
Increased cell turnover due to lymphoma, leukaemia, psoriasis, Haemolysis and tumour lysis syndrome
Symptoms of gout
Rapid severe onset of pain
Joint stiffness
Mostly affects joints in feet, first metatarsophalangeal joint (PODAGRA)
Swelling / joint effusion
Tophi - over extensor surface joints, urate deposits in pinna and tendons
Renal disease - radiolucent stones and interstitial nephritis
Ix for gout
Polarised light microscopy = negatively birefringent needle shaped crystals
High serum urate
Xray = punched out erosion in juxta-articular bone, reduced joint space
Mx for gout
Acute gout -
- NSAIDs (diclofenac or indomethacin)
- Colchicine
- Use steroids in renal impairment
Prevention of gout -
- Weight loss
- Avoid prolonged fasts and alcohol excess
- Allopurinol (xanthine oxidase inhibitor)
What is pseudo gout?
A rheumatic disorder caused by the accumulation of crystals of calcium pyrophosphate dehydrate in connective tissues. Knee joint mostly affected.
What are the 2 types of pseudo gout?
Acute CPPD = acute monoarthropathy. Affects knee joint. Usually spontaneous and self limiting. Elderly patients.
Chronic CPPD = destructive changes like in OA. Poly-arthritis.
Risk factors for pseudo gout?
Older age OA DM Hypothyroidism Hyperparathyroidism Haemochromatosis Wilson's disease
Ix for pseudo gout
Polarised light microscopy = positively birefringent rhomboid-shaped crystals
X-ray = chondrocalcinosis. Soft tissue calcium deposition
Raised WCC
Tx for pseudo gout
Acute attacks = analgesia, NSAIDs, steroids (intra-articular or systemic), ice packs, colchicine
Chronic CPPD = joint replacement therapy, paracetamol, colchicine maintenance therapy
Define fibromyalgia
Chronic condition, more common in women (F>M, 10:1), causes widespread muscle pain, extreme tenderness and sleep disturbance
Symptoms of fibromyalgia
- Chronic pain
- Diffuse tenderness on examination
- Fatigue unrelieved by rest
- Sleep disturbance
- Mood disturbance
- Cognitive dysfunction / concentration difficulty
- Headaches
- Numbness / tingling sensations
- Stiffness
- Sensitivity to sensory stimuli
Risk factors for fibromyalgia
• Neurosis – depression, anxiety, stress • FH fibromyalgia • Rheumatological conditions • Middle aged • Female • Associated conditions o Chronic fatigue syndrome o Irritable bowel syndrome o Chronic headache syndromes
Ix for fibromyalgia
- Clinical diagnosis: presence of chronic (>3 months) widespread body pain and associated symptoms such as fatigue and sleep disturbances.
Normal ESR, CRP, TFT, FBC, RF, Anti-CCP, antinuclear antibody, Vit D level
MX fibromyalgia
Education CBT Graded exercise programmes Amitryptyline / pregablin SNRIs (venlafaxine / duloxetine)
What classes as a hip fracture?
any fracture of the femur distal to the femoral head and proximal to a level a few centimetres below the lesser trochanter.
Risk factors for osteoporosis
Age + SHATTERED
- Steroids
- Hyperthyroidism / hyperparathyroidism
- Alcohol and cigarettes
- Thin (BMI <22)
- Testosterone low
- Early menopause
- Renal / liver failure
- Erosive / inflammatory bone disease e.g. RA or myeloma
- Dietary calcium low / malabsorption, DM
Signs of hip fracture
- Shortened and externally rotated leg
- Pain in affected hip, groin or thigh
- Inability to weight bear or move the hip
- History of fall or trauma
Classification of hip fractures
Intra or extra capsular
Garden classification for intracapsular fractures
What should you look at on an x-ray for a fractured hip?
Shenton’s line
Intra or extra capsular
Displaced or non displaced
Osteopenic?
What are the rules for when to x-ray an ankle to look for fractures?
Ottawa ankle rules
- X-ray ankle if pain in malleolar zone + in any of:
o Tenderness along distal 6cm of posterior tibia or fibula including posterior tip of malleoli
o Inability to bear weight both immediately and in ED for four steps
Classification for ankle fractures
Weber classification for lateral malleolus fractures
• Type A = below the syndesmosis
• Type B = at the level of the syndesmosis
• Type C = above the level of the syndesmosis
What classification is used for tibial plateau fractures?
Schatzker classification
Define Lyme disease
zoonotic infection transmitted to humans through the bite of infected ticks
Causative organism of Lyme disease
borrelia burgdorferi
Symptoms of Lyme disease
Erythema migrans (target lesion) - rash 1-2 weeks after tick bite with central clearing Constitutional symptoms Arthritis Lymphadenopathy Hepatitis Bell's palsy CNS involvement AV block / my-pericarditis
Ix for Lyme disease
Enzyme immunoassay
Skin biopsy culture (takes 8+ weeks)
Antibiotic for Lyme disease
doxycycline (use IV benzylpenicillin if complications)
Define osteoarthritis
degenerative joint disorder in which there is progressive loss of hyaline cartilage.
Not an inflammatory joint disorder, is ‘wear and tear’.
Classification of osteoarthritis
- Primary = no underlying cause
- Secondary = obesity / joint abnormality
Symptoms of osteoarthritis
- Pain with activities and weight bearing. Pain at night is unusual, except in advanced OA
- Functional difficulties e.g., knee giving way or locking
- Knee, hip, hand or spine involvement
- Bony deformities
- Limited ROM
- Malalignment
- Crepitus, stiffness
What hand deformities do you get with osteoarthritis?
o Bouchard’s nodes: enlargement of PIP joints
o Heberden’s nodes: enlargement of DIP joints
o Squaring at base of the thumb (first carpometacarpal joint)
o Fixed flexion deformity
X-ray changes seen in osteoarthritis
o L = loss of joint space
o O = osteophytes
o S = subchondral sclerosis (increased density of the bone along the joint line)
o S = subchondral cysts (fluid filled holes in the bone aka geodes)
Management for osteoarthritis
- Analgesia
- Joint injection – local anaesthesia or steroids
- Surgical:
a. Arthroscopic washout
b. Arthroplasty
c. Osteotomy
d. Arthrodesis – joint fusion
Define osteomalacia
Metabolic bone disease characterised by defective bone mineralisation. There is incomplete mineralisation of the underlying mature organic bone matrix (osteoid) following growth plate closure in adults. There’s a normal amount of bone but it’s mineral content is low = excess uncalcified osteoid and cartilage.
Causes of osteomalacia
- Vitamin D deficiency – malabsorption, poor diet, lack of sunlight
- Renal failure
- Drug induced – anticonvulsants (increased breakdown of 25-hydroxy-vitamin D)
- Liver disease
- Tumour induced osteomalacia
Risk factors for osteomalacia
- Old age
- Vitamin D and calcium deficient diet
- Lack of sunlight exposure
- Malabsorption syndromes – coeliac disease, chronic alcoholism, chronic pancreatitis
- Family hx osteomalacia
Symptoms of osteomalacia
- Asymptomatic
- Fatigue
- Bone pain
- Muscle weakness – esp. proximal muscle weakness (waddling gait & difficulty climbing stairs)
- Muscle aches
- Pathological / abnormal fractures
- Looser zones = fragility fractures that go partially through the bone
Ix for osteomalacia
- Bloods =
a. Serum 25-hydroxyvitamin D (vitamin D lab test) – low
b. Serum calcium – low
c. Serum phosphate – low
d. Serum ALP – may be high
e. PTH – may be high (secondary hyperparathyroidism)
f. U&Es – to check for renal failure - Imaging =
a. Xray = osteopenia (=more radiolucent bones) / looser fractures
b. DEXA scan = low bone mineral density
Mx for osteomalacia
Calcium + vitamin D supplements (colecalciferol + calcium carbonate)
define rickets
a childhood disease. There’s deficient mineralisation at the growth plate of long bones.
What is the difference between osteomalacia and rickets?
In osteomalacia, mineralisation is impaired at the bone matrix but in rickets, mineralisation is impaired at the growth plate (e.g., rickets can only happen in growing children before the fusion of the epiphyses).
Risk factors for rickets
- Inadequate sunlight exposure
- Breastfeeding – as breast milk is deficient in vitamin D
- Calcium or phosphate deficiency
- Family hx of rickets
- Drugs – antacids, loop diuretics, corticosteroids and anticonvulsants
Symptoms of rickets
- Bony deformities
- Bone pain
- Growth retardation
- Increased fracture risk
- Late closing of fontanelles
What are the bony deformities seen in rickets?
o bowlegs – bending of long bones
o Rachitic rosery – bead like distention of the bone-cartilage junctions in the ribs
o Marfan sign – distention of the epiphyseal plate of the distal tibia with widening and cupping of the metaphysis = looks like a double medial malleolus
o Craniotabes – softening of the skull
o Genu varum – deformity of the knee
Ix for rickets
- X-ray long bone = Widening of epiphyseal plate, Cupping, Splaying, Fraying of metaphysis, Looser zone fractures
- Serum calcium
- Serum PTH
- Serum 25-hydroxyvitamin D levels
- ALP
- U&Es
Mx for rickets
Calcium and vitamin D supplementation (calcium + colecalciferol)
Define osteomyelitis
Infection / inflammation in bone and bone marrow. Usually a bacterial infection.
What are the sources of infection for osteomyelitis?
1) local/contiguous (fracture site / during surgery)
2) Haematogenous (most common)
Most common causative organism for osteomyelitis
Staph aureus
Risk factors for osteomyelitis
- Open fractures
- Orthopaedic operations (particularly with prosthetic joints)
- Diabetes (particularly with diabetic foot ulcers)
- Peripheral arterial disease
- IVDU
- Immunosuppression
- Children – rich blood supply to growth plate (therefore usually affects metaphysis)
- Sickle cell anaemia
Symptoms of osteomyelitis
• In children o Limp o Reluctance to weight bear • Non-specific pain at site of infection • Signs of systemic infection - Fever, malaise, fatigue • Local inflammation, tenderness, erythema or swelling • Reduced ROM • Effusion in neighbouring joints
Ix for osteomyelitis
- Blood tests
o Raised CRP, ESR, WCC
o Blood cultures (+ve in 60%) - Bone cultures
- Imaging
o X-rays often don’t show changes, can’t be used to exclude osteomyelitis
o Potential x-ray signs (takes 10-14 days)
o MRI – for diagnosis
x-ray signs seen in osteomyelitis
Periosteal reaction – changes to the bone surface
Localised osteopenia – thinning of the bone
Destruction of areas of bone
Mx for osteomyelitis
- Antibiotics – 6 weeks for acute (flucloxacillin + rifampicin) & 3 months for chronic
- Surgical debridement
- Analgesia
- Osteomyelitis with prosthetic joint = may require a complete revision of surgery to replace prosthesis
Define osteoporosis
reduction in bone density. Reduced bone density leads to increased bone fragility and fracture risk. More common in elderly.
Define osteopenia
a less severe reduction in bone density than osteoporosis.
Risk factors osteoporosis
- Older age
- Female – post menopausal women are at risk as oestrogen is protective against osteoporosis
- Reduced mobility and activity
- Low BMI (<18.5)
- RA
- Alcohol and smoking
- Long term corticosteroids
- Medications – SSRIs, PPIs, antiepileptics, anti-oestrogens
Symptoms of osteoporosis
• Asymptomatic until fracture occurs
What is the FRAX tool used for?
- Fracture risk assessment tool
- Gives a prediction of the risk of a fragility fracture over the next 10 years.
- This is usually the first step in assessing someone’s osteoporosis risk
- Input = age, BMI, co-morbidities, smoking, alcohol, family hx, bone mineral density (from DEXA scan)
- Gives 10-year probability as a % of a:
o Major osteoporotic fracture
o Hip fracture
How do you measure someones bone mineral density and why?
- Bone mineral density is measured using a DEXA scan
- DEXA – dual energy x-ray absorptiometry
- DEXA scans measure how much radiation is absorbed by the bones = shows how dense the bone is
- Bone mineral density can be measured at any location on the skeleton – do the hip for the classification & management of osteoporosis
- Bone density scores:
o Z score = number of SDs the patient’s bone density falls below the mean for their age
o T score = number of SDs the patient’s bone density falls below the mean for a healthy young adult - T score at the hip = classification for osteoporosis
How is bone mineral density used to classify the severity of osteoporosis?
T score =
>-1 = normal
-1 to -2.5 osteopenia
How do you assess someone for osteoporosis?
- FRAX assessment for:
a. Women >65
b. Men >75
c. Younger patients with fragility fractures, hx of falls, low BMI, long term steroids, endocrine disorders, RA - FRAX outcome without bone mineral density:
a. Low risk = reassure, follow up in 5 years
b. Intermediate risk = DEXA scan and recalculate FRAX score with results
c. High risk = offer treatment - FRAX outcome with bone mineral density
a. Treat
b. Lifestyle advice and reassure, follow up in 5 years
Management for osteoporosis
- Lifestyle advice
- Bisphosphonates
- Alternative to bisphosphonates = denoscumab, strontium ranelate, raloxifene, HRT
Lifestyle advice for osteoporosis
o Activity and exercise o Healthy weight o Adequate calcium and vitamin D intake (calcichew-D3) o Avoid falls o Stop smoking o Reduce alcohol
How do bisphosphonates work?
o Interfere with osteoclasts, reduces their activity, preventing the resorption of bone
Side effects of bisphosphonates
Reflux and oesophageal erosions – need to take on an empty stomach sitting upright for 30 minutes before moving or eating
Atypical fracture (femoral)
Osteonecrosis of the jaw and external auditory canal
Examples of bisphosphonates and how they are taken
alendronate (weekly oral)
risedronate (weekly oral)
zoledronic acid (yearly IV)
Define pathological fracture
a fracture that occurs in abnormal bone, because of a normally insignificant stress e.g. minor exertion or minor trauma.
Causes of pathological fractures
- Metastatic tumours – breast, lung, thyroid, kidney, prostate
- Generalised bone disease
o Osteogenesis imperfecta
o Osteoporosis
o Metabolic bone disease
o Myelomatosis
o Paget’s disease
o Osteomalacia - Local benign conditions
o Chronic osteomyelitis
o Bone cyst - Primary malignant tumours
o Chondrosarcoma
o Osteosarcoma
o Ewing’s tumour
Common locations for pathological fractures
spine (vertebral compression fractures), hip, wrist
Define polymyalgia rheumatica
inflammatory rheumatological syndrome that manifests as pain and morning stiffness involving the neck, shoulder girdle and/or pelvic girdle in people >50 yo. Peripheral MSK involvement may be present. Occurs as an isolated condition or with giant cell arteritis.
Who does polymyalgia rheumatica affect?
usually affects adults >50 yo, women and Caucasians
What other condition is polymyalgia rheumatica associated with?
Giant cell arteritis
Symptoms of polymyalgia rheumatica
- Bilateral shoulder pain that may radiate to the elbow
- Bilateral pelvic girdle pain
- Worse with movement
- Interferes with sleep
- Stiffness for at least 45 mins in the morning
- Systemic symptoms = weight loss, fatigue, low grade fever, low mood
- Upper arm tenderness
- Carpel tunnel syndrome
- Pitting oedema
How is polymyalgia rheumatica diagnosed?
clinical presentation + response to steroids (very responsive)
Mx for polymyalgia reumatica
15mg prednisolone daily
if they dont respond after a week, its not PMR. Taper dose after 3-4 weeks
Patient information for long term steroid use
Don’t STOP
- Don’t = patients will become steroid dependent after 3 weeks of treatment so should not stop taking them abruptly due to the risk of adrenal crisis
- S = sick day rules
- T = treatment card
- O = osteoporosis – consider bone protection (bisphosphates + calcium + vitamin D)
- P = proton pump inhibitor
Define rheumatoid arthritis
an autoimmune condition that causes chronic inflammation of the synovial lining of the joints, tendon sheaths and bursa. Is an inflammatory arthritis. Is a symmetrical polyarthritis. Primarily affects the small joints of the hands and feet.
Epidemiology of RA
Women (3x more common)
Middle age
+ve family hx
Genetic associations with RA
HLA DR4
HLA DR1
Joint symptoms of RA
- Active symmetrical arthritis lasting >6 weeks
a. Pain, swelling, stiffness
b. Commonly affected joints = wrist, ankle, MCP, PIP joints and cervical spine. Can affect larger joints (knees, shoulders, elbows)
c. Rapid onset e.g., can be overnight
d. Pain worse after rest and improves with activity
e. Morning stiffness
Bony deformities seen in RA
a. Swan neck deformity – hyperextended PIP with flexed DIP
b. Boutonniere’s deformity – hyperextended DIP with flexed PIP
c. Ulnar deviation – fingers point outwards from MCP joints
d. Z shaped deformity to thumb
e. Subluxation of MCP
What can happen to the cervical spine in RA?
- Atlantoaxial subluxation –
a. The axis (C2) and odontoid peg shifts within the atlas (C1) – can cause spinal cord compression = emergency
b. Important if patient having a GA – do MRI scan in pre-operative assessment to visualise changes
Systemic features of RA
a. Fatigue
b. Weight loss
c. Flu like illness
d. Muscle aches and weakness
Extra articular features of RA
a. Rheumatoid nodules – pink/red with rubbery texture. Check elbows.
b. Vasculitic lesions – skin rashes
c. Pleuritic chest pain
d. Scleritis / uveitis
e. Caplan’s syndrome – pulmonary fibrosis with pulmonary nodules
f. Bronchiolitis obliterans – inflammation causing small airway destruction
g. Felty’s syndrome – RA, neutropenia and splenomegaly
h. Secondary sjogren’s syndrome – Sicca syndrome
i. Anaemia of chronic disease
j. Cardiovascular disease – pericarditis + pericardial effusion
k. Lymphadenopathy
l. Carpel tunnel syndrome – bilateral
m. Amyloidosis
What is palindromic rheumatism?
self-limiting short episodes of inflammatory arthritis with joint pain, stiffness and swelling that only affects a few joints. Last 1-2 days then resolves. If positive for RF and anti-CCP = usually progression to full RA
Ix for RA
- Rheumatoid factor – autoantibody present in 70% of RA patients. RF is usually IgM.
- Anti CCP antibodies (cyclic citrullinated peptide antibodies) – specific to RA and often pre-date the development of RA
- Inflammatory markers – CRP and ESR
- X-ray hands and feet –
a. Joint destruction and deformity
b. Soft tissue swelling
c. Periarticular osteopenia
d. Bony erosions - US scan joints – synovitis
Diagnosis criteria RA
Diagnosis criteria: >6 score is RA
- Joints involved = more and smaller joints score higher
- Serology – RF and anti-CCP
- Inflammatory markers – ESR and CRP
- Duration of symptoms = more than 6 weeks
What score is used to monitor RA?
DAS28 score = disease activity score.
- Assessment of 28 joints
- Points are given for: swollen joints, tender joints, ESR/CRP result
What score is used to monitor functional ability with RA?
HAQ = health assessment questionnaire
- Measures functional ability
- Good for checking response to treatment
What makes the prognosis with RA worse?
- Younger onset
- Male
- More joints and organs affected
- Presence of RF and anti-CCP
- Erosions on x-ray
Mx for RA
- Multidisciplinary team involvement
- Quick referral – any adult with persistent synovitis, even if seronegative. Urgent referral if small joints of hands and feet, multiple joints or symptoms present for 3+ months
- Flare ups = short course steroids, NSAIDs
- DMARDs
a. 1st line = methotrexate, leflunomide or sulfasalazine. Hydroxychloroquine in mild disease
b. 2nd line = use 2 of above in combination
c. 3rd line = methotrexate + TNF inhibitor
d. 4tj line = methotrexate + rituximab - Orthopaedic surgery for joint deformities
How does methotrexate work?
Interferes with metabolism of folate
How should methotrexate be taken?
Once weekly
Also take folic acid once weekly on a different day to methotrexate
Side effects of methotrexate
Pulmonary fibrosis Teratogenic Mouth ulcers and mucositis Liver toxicity Bone marrow suppression and leukopenia
How does leflunomide work?
Interferes with production of pyrimidine (component of RNA and DNA)
Side effects of leflunomide
Peripheral neuropathy Increased BP Rashes Teratogenic Mouth ulcers and mucositis Liver toxicity Bone marrow suppression and leukopenia
What DMARDs are safe in pregnancy
Sulfasalazine
Hydroychloroquine
Side effects of sulfasalazine
Temporary male infertility – reduces sperm count
Bone marrow suppression
Hydroxychloroquine side effects
Nightmares
Reduced visual acuity – macular toxicity
Liver toxicity
Skin pigmentation
Name anti-TNF drugs
Adalimumab Infliximab Etanercept Golimumab Certolizumab
Side effects of anti -TNF drugs
Vulnerability to severe infections and sepsis
Reactivation of TB and hep B
What type of drug is rituximab?
Anti-CD20 drug (monoclonal antibody)
Side effects of anti-CD20 drugs (e.g. rituximab)
Vulnerability to severe infections and sepsis Night sweats Thrombocytopenia Peripheral neuropathy Liver and lung toxicity
Define sarcoidosis
a chronic granulomatous disorder of unknown cause, commonly affecting the lungs, skin, and eyes. Characterised by accumulation of lymphocytes and macrophages and the formation of non-caseating granulomas in the lungs and other organs.
Who gets sarcoidosis
Women
Black
2 incidence spikes - young adulthood then around 60 yo
Symptoms of sarcoidosis
- Lung symptoms – in over 90% o Mediastinal lymphadenopathy o Pulmonary fibrosis o Pulmonary nodules - Systemic symptoms o Fever o Fatigue o Weight loss - Liver – in around 20% o Liver nodules o Cirrhosis o Cholestasis - Eyes – in around 20% o Uveitis o Conjunctivitis o Optic neuritis - Skin – in around 15% o Erythema nodosum – tender red nodules on the skins caused by inflammation of the subcutaneous fat o Lupus pernio – raised purple skin lesions commonly on cheeks and nose o Granulomas develop in scar tissue - Heart – in around 5% o Bundle branch block o Heart block o Myocardial muscle involvement - Kidneys – in around 5% o Kidney stones – due to hyperalcaemia o Nephrocalcinosis o Interstitial nephritis - CNS – in around 5% o Nodules o Pituitary involvement – diabetes incipidus o Encephalopathy - Peripheral nervous system – in around 5% o Facial nerve palsy o Mononeuritis multiplex - Bones – in around 2% o Arthralgia o Arthrtitis o Myopathy
What is lofgren’s syndrome?
• Specific presentation of sarcoidosis • Characterised by triad: o Erythema nodosum o Bilateral hilar lymphadenopathy o Polyarthralgia
Ix for sarcoidosis
- Bloods
a. Raised serum ACE
b. Hypercalcaemia
c. Raised serum soluble interleukin-2 receptor
d. Raised CRP
e. Raised immunoglobulins - Imaging
a. CXR – hilar lymphadenopathy
b. CT thorax – hilar lymphadenopathy and pulmonary nodules
c. MRI head – CNS involvement
d. PET scan – active inflammation of affected areas - Histology = GOLD STANDARD FOR DIAGNOSIS
a. Bronchoscopy, ultrasound guided biopsy of mediastinal lymph nodes for histology
b. Histology shows = non-caseating granulomas with epithelioid cells
Mx sarcoidosis
- No/mild symptoms = no treatment
- Oral steroids for 6 to 24 months (+ bisphosphonates)
- 2nd line = methotrexate or azathioprine
- Lung transplant in severe pulmonary disease
Define septic arthritis
infection within a joint (native joint or join replacement)
EMERGENCY
Local or haematogenous spread
Causative organisms of septic arthritis
- STAPH AUREUS – most common
- Gonococcus – common in young sexually active (Gram staining = gram negative diplococcus)
- Group A strep e.g., streptococcus pyogenes
- Haemophilus influenza
- E. coli
Risk factors for septic arthritis
- Joint disease – RA
- Immunosuppression
- Prosthetic joints
Presentation of septic arthritis
- Usually affects a single joint
- Hot red swollen painful joint, acute onset
- Stiffness and reduced ROM
- Systemic symptoms: fever, lethargy, and sepsis
Ix for septic arthritis
- Joint aspiration for MCS – high WCC (mostly polynuclear leukocytes e.g., neutrophils)
- Raised ESR, CRP, WCC
- Blood cultures
- X-ray
Mx septic arthritis
- Abx
o Check local policy
o Empirical IV abx given until sensitivities known
o For 3-6 weeks
o Flucloxacillin (vancomycin if penicillin allergy/MRSA/prosthetic joint) + rifampicin - Joint washout under GA
- Splint joint
Complications of septic arthritis
- Osteomyelitis
- Arthritis
- Ankylosis (fusion)
Causes of mono arthritis
Septic arthritis
Crystal arthritis - gout, CPPD
Osteoarthritis
Trauma - haemarthrosis
Causes of oligoarthritis (<5 joints)
Crystal arthritis Psoriatic arthritis Reactive arthritis Ankylosing spondylitis Osteoarthritis
Symmetrical polyarthritis causes
RA
Osteoarthritis
Viruses - hep A, hep B, hep C, mumps
Assymetrical polyarthritis causes
Reactive arthritis
Psoriatic arthritis
Define systemic lupus erythematous
multisystemic inflammatory autoimmune connective tissue disease. Systemic (affects many organs). Often has relapsing remitting course, with flares then periods where symptoms are improved. Chronic inflammation means patients have a shortened life expectance – cardiovascular disease and infection = leading causes of death.
Characteristics of SLE
A RASH POINts an MD
- A = arthritis (peripheral joints)
- R = renal (proteinuria and high BP)
- A = ANA (+ve in 90%) – antinuclear antibodies – antibodies against normal proteins in the cell nucleus
- S = serositis – pleuritis and pericarditis
- H = haematological – autoimmune haemolytic anaemia, low WCC, thrombocytopenia
- P = photosensitivity
- O = oral ulcers
- I = immune phenomenon (anti-dsDNA, anti-Sm, anti-phospholipid)
- N = neurological – seizures, psychosis
- M = malar rash (facial erythema sparing the nasolabial folds)
- D = discoid rash
Ix for SLE
- Autoantibodies – ANA, anti-dsDNA, anti-smith
- FBC – normocytic anaemia
- C3 and C4 levels – decrease in active disease
- CRP and ESR – increase with active inflammation
- Immunoglobulins – raised due to activation of B cells with inflammation
- Urinalysis & urine protein: creatinine ratio
- Renal biopsy
Mx for SLE
- NSAIDs
- Steroids – prednisolone
- Hydroxychloroquine – mild SLE
- Sun cream and sun avoidance
- Severe SLE
a. Methotrexate
b. Mycophenolate mofetil
c. Azathioprine – inhibits purine synthesis. Do TPMT test first to check for individuals prone to azathioprine toxicity. SEs: bone marrow depression, nausea/vomiting, pancreatitis, increased risk of non-melanoma skin cancer. Safe in pregnancy.
d. Tacrolimus
e. Leflunomide
f. Ciclosporin
g. Rituximab
h. Belimumab (monoclonal antibody that targets b cell activating factor)
What is systemic sclerosis?
Systemic sclerosis = autoimmune inflammatory and fibrotic connective tissue disease. Cause unclear. Characterised by hardened, sclerotic skin and other connective tissues and affects the internal organs.
Most patients with scleroderma have systemic sclerosis however there’s a localised version of scleroderma that only affects the skin.
Is systemic sclerosis more common in women or men?
4x more common in women
3 types of sclerosis
- Limited cutaneous systemic sclerosis
- Diffuse cutaneous systemic sclerosis
- Scleroderma (no internal organ involvement)
What are the features of limited cutaneous systemic sclerosis?
a. Raynaud’s
b. Scleroderma affects face and distal limbs predominantly
c. Associated with ANTI-CENTROMERE ANTIBODIES
d. Subtype of limited cutaneous systemic sclerosis = CREST syndrome
What does CREST syndrome stand for?
i. Calcinosis
ii. Raynaud’s
iii. Oesophageal dysmotility
iv. Sclerodactyly
v. Telangiectasia
The symptoms seen in limited cutaneous systemic sclerosis
Features of diffuse cutaneous systemic sclerosis?
a. Scleroderma affects trunk and proximal limbs predominantly
b. Associated with SCL-70 ANTIBODIES
c. Most common cause of death = respiratory involvement (pulmonary hypertension and pulmonary fibrosis)
d. Other complications = renal disease (glomerulonephritis and scleroderma renal crisis) , coronary artery disease & HTN
e. Poor prognosis
Features of scleroderma
a. Tightening and fibrosis of skin
b. May manifest as plaques (morphoea) or linear
Antibodies seen in systemic sclerosis?
- Antinuclear antibodies (ANA) - Systemic sclerosis (+ve in 90%)
- Rheumatoid factor - Systemic sclerosis (+ve in 30%)
- Anti-scl-70 antibodies - Diffuse cutaneous systemic sclerosis, Associated with more severe disease
- Anti-centromere antibodies-Limited cutaneous systemic sclerosis
Symptoms of systemic sclerosis
- Scleroderma – hardening of the skin. Shiny tight skin without normal folds.
- Sclerodactyly – skin tightening around joints, restricting ROM and loss of fat pads
- Telangiectasia
- Calcinosis – calcium deposits under the skin, most commonly on the fingertips
- Raynaud’s – white, blue then red fingertips in response to cold
- Oesophageal dysmotility – connective dysfunction in the oesophagus. Swallowing difficulties, reflux and oesophagitis.
- Systemic and pulmonary HTN – connective tissue dysfunction in arteries
- Pulmonary fibrosis – gradual onset dry cough and SOB
- Scleroderma renal crisis – acute condition with severe HTN and renal failure
Ix for systemic sclerosis
- Autoantibodies – ANA, RF, anti-centromere and anti-scl-70
- Nailfold capillaroscopy – skin at the base of the nail is magnified and examined to look at the health of the peripheral capillaries.
Non-medical management for systemic sclerosis
o Avoid smoking o Gentle skin stretching o Regular emollients o Avoid cold triggers for Raynaud’s o Physiotherapy to maintain healthy joints o Occupational therapy
Medical management for systemic sclerosis
o Nifedipine for Raynaud’s
o PPIs and metoclopramide (pro-motility) for oesophageal dysmotility
o Analgesia for joint pain
o Antibiotics for skin infections
o Ant-HTN
o Tx for pulmonary artery HTN and pulmonary fibrosis
o Diffuse disease = steroids and immunosuppressants
What is osteochondritis dissecans?
pathological process affecting the subchondral bone (most often in the knee joint) with secondary effects on the joint cartilage, including pain, oedema, free bodies, and mechanical dysfunctions.
Affects children/young adults
Symptoms of osteochondritis dissecans
- Knee pain and swelling, typically after exercise
- Knee catching, locking and/or giving way: more constant and severe symptoms are associated with the presence of loose bodies
- Feeling a painful ‘clunk’ when flexing or extending the knee - indicating the involvement of the lateral femoral condyle
Ix for osteochondritis dissecans & findings
- X-ray (anteroposterior, lateral and tunnel views) - may show the subchondral crescent sign or loose bodies
- MRI - used to evaluate cartilage, visualise loose bodies, stage and assess the stability of the lesion
What is anti-phospholipid syndrome?
Definition = disorder associated with antiphospholipid antibodies where the blood is prone to clotting. Is a hyper-coagulable state.
Main associations = thrombosis and recurrent miscarriage.
Occurs on its own or secondary to SLE.
What 3 antibodies are associated with antiphospholipid syndrome?
- Lupus anticoagulant antibodies
- Anticardiolipin antibodies
- Anti-beta-2 glycoprotein I antibodies
Symptoms of antiphospholipid syndrome
- DVT / PE
- Stroke / MI / Renal thrombosis
- Recurrent miscarriages
- Stillbirth
- Pre-eclampsia
- Livedo reticularis – purple lace like rash that gives a mottled appearance to the skin
- Libmann-Sacks endocarditis – non-bacterial endocarditis
- Thrombocytopenia
What happens to the ATTP in antiphospholipid syndrome?
RAISED ATTP – this is paradoxical and the blood is more prone to clotting. Its due to an ex-vivo reaction of the lupus anticoagulant autoantibodies with phospholipids involved in the coagulation cascade.
Mx for antiphospholipid syndrome
- Low dose aspirin
- If VTE has occurred:
o Lifelong warfarin with a target INR of 2-3
o If recurrent VTE, INR target of 3-4
What is sjogren’s syndrome
autoimmune disorder effecting exocrine glands resulting in dry mucosal surfaces.
Can be primary or secondary to RA/other connective tissue disorders.
9x more common in women
symptoms of sjogrens syndrome
- Dry eyes – keratoconjunctivitis sicca
- Dry mouth
- Vaginal dryness
- Arthralgia
- Raynaud’s
- Sensory polyneuropathy
- Recurrent episodes of parotitis
- Renal tubular acidosis
Ix for sjogrens syndrome
- RF - +ve in 50%
- ANA - +ve in 70%
- Anti-Ro antibodies - +ve in 70% of patients with primary sjogren’s syndrome
- Anti-La antibodies - +ve in 30% of patients with primary sjogren’s syndrome
- Schirmer’s test – filter paper near conjunctival sac to measure tear formation
- Histology – focal lymphocytic infiltration
- Hypergammaglobinaemia
- Low C4
Mx for sjogrens syndrome
- Artificial saliva and tears
- Pilocarpine to stimulate saliva production
What type of cancer is there increased risk of if you have sjogrens syndrome?
- Increased risk of lymphoid malignancy (40-60 times)
What is Raynaud’s disease and phenomenon?
Raynaud’s phenomenon is characterised by an exaggerated vasoconstrictive response of the digital arteries and cutaneous arteriole to the cold or emotional stress.
It may be primary (Raynaud’s disease) or secondary (Raynaud’s phenomenon).
Secondary causes of raynaud’s
- connective tissue disorders
o scleroderma (most common)
o rheumatoid arthritis
o systemic lupus erythematosus - leukaemia
- type I cryoglobulinaemia, cold agglutinins
- use of vibrating tools
- drugs: oral contraceptive pill, ergot
- cervical rib
Mx for raynauds
Nifedipine (CCB)
2nd line - IV prostacyclin infusion (effects last for weeks/months)
What is giant cell arteritis
systemic vasculitis of the medium and large arteries. Typically presents with symptoms affecting the temporal arteries e.g., temporal arteritis.
What is temporal arteritis associated with?
Polymyalgia rheumatica
Symptoms of giant cell arteritis
• Headache
o Severe unilateral headache around temple and forehead
o Scalp tenderness on brushing hair
o Jaw claudication
o Blurred/double vision
o Irreversible painless complete sight loss can occur rapidly
• Systemic symptoms: o Fever o Muscle aches o Fatigue o Weight loss o Loss of apetite o Peripheral oedema
Ix for giant cell arteritis
- raised ESR
- temporal artery biopsy (multinucleated giant cells)
- FBC – normocytic anaemia and thrombocytosis
- LFTs – raised ALP
- CRP raised
- Duplex US of temporal artery – hypoechoic halo sign
Mx for giant cell arteritis
- Prednisolone 40-60mg
- Aspirin
- PPI (think steroids)
- Refer to: vascular surgeons, rheumatology, ophthalmology
Early and late complications of giant cell arteritis
- Early o Vision loss o Stroke - Late o Relapse of the condition o Steroid related side effects o Stroke o Aortitis leading to aortic aneurysm and aortic dissection
What is Paget’s disease?
is a disease of increased but uncontrolled bone turnover. It is thought to be primarily a disorder of osteoclasts, with excessive osteoclastic resorption followed by increased osteoblastic activity.
Clinical features of paget’s disease
- older male with bone pain and an isolated raised ALP
- bone pain (e.g. pelvis, lumbar spine, femur)
- classical, untreated features: bowing of tibia, bossing of skull
Ix for Paget’s disease
- bloods
- raised alkaline phosphatase (ALP)
- calcium and phosphate are typically normal. Hypercalcaemia may occasionally occur with prolonged immobilisation
- other markers of bone turnover include
- procollagen type I N-terminal propeptide (PINP)
- serum C-telopeptide (CTx)
- urinary N-telopeptide (NTx)
- urinary hydroxyproline
- x-rays
o osteolysis in early disease → mixed lytic/sclerotic lesions later - skull x-ray: thickened vault, osteoporosis circumscripta
- bone scintigraphy
o Increased uptake is seen focally at the sites of active bone lesions
Mx for Paget’s disease
- bisphosphonate (either oral risedronate or IV zoledronate)
- calcitonin is less commonly used now
Complications of paget’s disease
- deafness (cranial nerve entrapment)
- bone sarcoma (1% if affected for > 10 years)
- fractures
- skull thickening
- high-output cardiac failure
