Musculoskeletal Flashcards
1
Q
Define amyloidosis.
A
Heterogenous group of diseases characterized by extracellular deposition of amyloid fibrils.
Can be systemic or localised - e.g. pancreatic islets of Langerhans, cerebral cortex, cerebral blood vessels, bones and joints
Pancreatic Islets of Langerhans - T2DM
Cerebral Cortex - Alzheimer’s
Cerebral Blood Vessels - amyloid angiopathy
Bones & Joints - long-term dialysis caused by B2 microglobulin
2
Q
Explain the aetiology / risk factors of amyloidosis.
A
Amyloid fibrils are polymers comprising low-molecular-weight subunit proteins.
Amyloid fibril subunits are derived from proteins that undergo conformational changes to adopt anti-parallel B-pleated sheet configuration.
Amyloid fibril subunits associated with GAGs and serum amyloid P-component (SAP), and their sdeposition progressively disrupts the structure and function of nromal tissue.
Classification:
- AA - serum amyloid A protein - e.g. Chronic inflammatory (RA, seronegative arthritides, Crohn’s, familial Mediterranean fever), chronic infections (TB, bronchiectasis, osteomyelitis), malignancy (Hodgkin’s disease, renal cancer)
- AL - monoclonal immunoglobulin light chains fibril protein - e.g. subtle monoclonal plasma cell dyscrasias, multiple myeloma, Waldenstrom’s macroglobulinaemia, B-cell lymphoma
- ATTR (familiar amyloid polyneuropath) - genetic-variant transthyretin - autosomal dominantly transmitted muttaions in the gene for transthyretin (TTR), variable penetrance
Risk factors:
- Monoclonal gammopathy of undetermined significance (MGUS)
- Inflammatory polyarthropathy
- Chronic infections
- IBD
3
Q
Summarise the epidemiology of amyloidosis.
A
AA = 1-5% incidence among patients with chronic inflammatory disease
AL = estimated annual incidence of about 3,000 cases in US, 300-600 cases in UK
Hereditary - 5% of patients with systemic amyloidosis
4
Q
Recognise the presenting symptoms of amyloidosis.
A
Renal
- Proteinuria
- Nephrotic syndrome
- Renal failure
Cardiac
- Restrictive cardiomyopathy
- Heart failure
- Arrythmia
- Angina - due to accumulation of amyloid in coronary arteries
GI
- Macroglossia - characteristic of AL
- Hepatomegaly
- Splenomegaly
- Gut dysmotility
- Malabsorption
- Bleeding
Neurological
- Sensory and motor neuropathy
- Autonomic neuropathy - symptoms of bowel or bladder dysfunction, postural hypotension
- Carpal tunnel syndrome
Skin
- Waxy skin
- Easy brusing
- Purpura around the eyes - characteristic of AL
- Plaques
- Nodules
Joints
- Painful asymmetrical large joint
- Shoulder pad sign - enlargement of the anterior shoulder
Haematological
- Bleeding diathesis - factor X deficiency due to binding on amyloid fibrils primarily in the liver and spleen and reduce synthesis of coagulation factors in patients with advanced liver disease
5
Q
Recognise the signs of amyloidosis on physical examination.
A
Renal
- Proteinuria
- Nephrotic syndrome
- Renal failure
Cardiac
- Restrictive cardiomyopathy
- Heart failure
- Arrythmia
- Angina - due to accumulation of amyloid in coronary arteries
GI
- Macroglossia - characteristic of AL
- Hepatomegaly
- Splenomegaly
- Gut dysmotility
- Malabsorption
- Bleeding
Neurological
- Sensory and motor neuropathy
- Autonomic neuropathy - symptoms of bowel or bladder dysfunction, postural hypotension
- Carpal tunnel syndrome
Skin
- Waxy skin
- Easy brusing
- Purpura around the eyes - characteristic of AL
- Plaques
- Nodules
Joints
- Painful asymmetrical large joint
- Shoulder pad sign - enlargement of the anterior shoulder
Haematological
- Bleeding diathesis - factor X deficiency due to binding on amyloid fibrils primarily in the liver and spleen and reduce synthesis of coagulation factors in patients with advanced liver disease
6
Q
Identify appropriate investigations for amyloidosis and interpret the results.
A
- Tissue biopsy - congo red stain, immunohistochemistry (diagnose amyloidosis, identify amyloid fibril protein)
- Urine (proteinuria, free immunoglobi light chains in AL)
- Blood (CRP, ESR, RF, Ig levels, serum protein electrophoresis, LFTs, U&E, SAA levels)
- 123I-SAP Scan - radiolabeled SAP localizes to the deposits enabling quantitative imaging of amyloidotic organs throughout the body
7
Q
Define osteomyelitis.
A
An inflammatory condition of bone caused by an infecting organism, most commonly Staphylococcus aureus.
8
Q
Explain the aetiology / risk factors of osteomyelitis.
A
Usually involves single bone, but rarely affects multiple sites.
Occurs in peripheral or axial skeleton.
Stage on the aetiology of infection, pathogenesis, extent of bone involvement, duration and host factors particular to the individual patients.
Either haematogenous or contigous-focus.
Risk factors:
- Previous osteomyelitis
- Penetrating injury
- IV drug misuse
- Diabetes
- HIV infection
- Recent surgery
- Distant or local infections
- Sickle cell anaemia
- RA
- CKD
- Immunocompromising conditions
- URTI
- Varicella infection
9
Q
Summarise the epidemiology of osteomyelitis.
A
The annual incidence of osteomyelitis was less than eleven cases per 100,000 person-years until the sixth decade of life. Thereafter, incidence rates increased steeply with age, corresponding to a roughly 50% increase in incidence per decade of life.
10
Q
Recognise the presenting symptoms of osteomyelitis.
A
- Risk factors
- Limp or reluctance to weight-bear
- Non-specific pain at site of infection
- Malaise and fatigue
- Local back pain associated with systemic symptoms
- Paravertebral muscle tenderness and spasm
- Local inflammation, tenderness, erythema or swelling
- Fever
- Spinal cord or nerve root compression
- Wound drainage, acute or old healed sinuses
- Scars, previous flaps, fracture fixation
- Reduced range of movement
- Reduced sensation in diabetic foot infection
- UTI symptoms
- Torticollis
- Skin or other infections, recent episodes of Staphylococcus aureus bloodstream infection, indwelling catheter
- Limb deformity
- Tenderness to percussion
- Meningitis
11
Q
Recognise the signs of osteomyelitis on physical examination.
A
- Risk factors
- Limp or reluctance to weight-bear
- Non-specific pain at site of infection
- Malaise and fatigue
- Local back pain associated with systemic symptoms
- Paravertebral muscle tenderness and spasm
- Local inflammation, tenderness, erythema or swelling
- Fever
- Spinal cord or nerve root compression
- Wound drainage, acute or old healed sinuses
- Scars, previous flaps, fracture fixation
- Reduced range of movement
- Reduced sensation in diabetic foot infection
- UTI symptoms
- Torticollis
- Skin or other infections, recent episodes of Staphylococcus aureus bloodstream infection, indwelling catheter
- Limb deformity
- Tenderness to percussion
- Meningitis
12
Q
Identify appropriate investigations for osteomyelitis and interpret the results.
A
1st Line:
- FBC - HIGH WCC
- ESR - HIGH
- CRP - HIGH
- Blood culture - may be positive, indicating infecting organism and microbial sensitivities
- Plain XR of affected area
Results of XR - ACUTE DISEASE
- Initially normal
- Osteopenia 6-7 days after infection onset
- Evidence of bone destruction, cortical breaches and periosteal reaction
- Involucra and sequestra sometimes seen
- Diffuse osteopenia developing later due to disuse of affected limb
- Joint effusion in local joints
Results of XR - DISCITIS
- Lacteral spin radiographs show late changes at 2-3 weeks into illness
- Decreased intervertebral space
- Erosion of vertebral plate
Results of XR - VERTEBRAL OSTEOMYELITIS:
- Localised rarefication (thinning) of vertebral body
- Anterior bone destruction later on
Results of XR - CHRONIC DISEASE
- Intramedullary scalloping
- Cavities
- Cloacae seen
- Fallen leaf sign when a piece of endosteal sequestrum detached and fallen into medullary canal
Consider:
- Bone samples and bone biopsy - positive, other pathology shown
- PCR
- MALDI-TOF mass spectrometry - match reference strains
- Swabs
- Urine microscopy, culture and sensitivities
- Histology 0 infecting organisms, acute or chronic inflammatory cells, dead bone, active bone resorption, small sequestra, malignancy
- Probe-to-bone test - may reach bone, rule in osteomyelitis in high-risk patient with diabetes
- Bone MRI - high signal on T2 images, fat suppression sequences, changes in children within 3-5 days of onset, vertebral bone changes
- US - collections, subperiosteal abscesses, adjacent joint infusions
- CT scan - bone destruction, sequestra, abscess
- Radionuclide scan - increased uptake of radioactive injectate in infected sites
- Bone scintigraphy - hot spots of infection, positive 24hrs after onset
- Echocardiogram - valvular vegetations
- CXR - show primary or reatcive TB
- Mantoux test - positive for Mycobacteruim TB
13
Q
Define septic arthrtis.
A
The infection of 1 or more joints caused by pathogenic inoculation of microbes.
Occurs either by direct inoculation or haematogenous spread.
14
Q
Explain the aetiology / risk factors of septic arthritis.
A
Regard a hot, swollen, acutely painful joint with restriction of movement as septic arthritis until proven otherwise, even in the absence of fever and irrespective of microbiology and blood test results.
Risk factors:
- Underlying joint disease
- Prosthetic joint
- Age
- Immunosuppression
- Contiguous spread
- Exposure to ticks
- Previous intra-articular corticosteroid injection
- Recent joint surgery
- Low socioeconomic status
15
Q
Summarise the epidemiology of septic arthritis.
A
The estimated incidence of septic arthritis in developed countries is 6 cases per 100,000 population per year.
In patients with underlying joint disease or with prosthetic joints the incidence increases approximately 10-fold, to 70 cases per 100,000 of the population.
16
Q
Recognise the presenting symptoms of septic arthritis.
A
- Hot
- Swollen
- Painful
- Restricted
- Acute presentation
- Fever
- Large joint
- Prosthetic joint
- Single joint affected
- Symptoms are out of proportion to elsewhere disease activitiy
- Sexual activity - gonoccocal septic arthritis may present with polyarthralgia localising over one joint, fever, chills and skin lesions
- Erythema migrans
- Risk factors present
17
Q
Recognise the signs of septic arthritis on physical examination.
A
- Hot
- Swollen
- Painful
- Restricted
- Acute presentation
- Fever
- Large joint
- Prosthetic joint
- Single joint affected
- Symptoms are out of proportion to elsewhere disease activitiy
- Sexual activity - gonoccocal septic arthritis may present with polyarthralgia localising over one joint, fever, chills and skin lesions
- Erythema migrans
- Risk factors present
18
Q
Identify appropriate investigations for septic arthritis and interpret the results.
A
1st Line:
- Synovial fluid microscopy, Gram stain and polarising microscopy - ?micro-organisms, urate or pyrophosphate crystals
- Synovial fluid culture and sensitivities
- Synovial fluid WCC
- Blood culture and sensitivities
- WCC - elevated
- ESR - elevated
- CRP - elevated
- U&E - assess for sepsis and end-organ damage
- LFTs - assess for sepsis and end-organ damage
- Plain X-Ray - reveals degenerative changes or chondrocalcinosis (not diagnostic for septic arthritis)
- USS - presence of effusion to guide aspiration
Consider:
- Procalcitonin (PCT) - raised >0.5ng/mL more specific marker for bacterial infection than CRP, ESR or WCC
- MRI - ?associated osteomyelitis
- PCR
- Swabs for microscopy, culture and sensitivity
- Urine dipstick - organisms on microscopy, WCC, blood
- ELISA
- Synovial biopsy - ? myobacterium tuberculosis, fungi
- Calprotectin - high
19
Q
Define Sjogren’s Syndrome.
A
A systemic auto-immune disorder, characterised by keratoconjunctivitis sicca and xerostomia as a consequences of lymphocytic infiltration into the lacrimal salivary glands.
20
Q
Explain the aetiology / risk factors of Sjogren’s Syndrome.
A
Primary - occurs alone
Secondary - occurs along with another auto-immune disease - e.g. lupus, RA, systemic sclerosis
Risk factors:
- Female
- SLE
- RA
- Systemic sclerosis (scleroderma)
- HLA Class I markers
- Age peaks in 20-30s and after menopause
- Genetic inheritance
21
Q
Summarise the epidemiology of Sjogren’s Syndrome.
A
Sjogren syndrome is far from a rare disorder with an incidence approaching approximately one-half of that of rheumatoid arthritis (RA) or affecting 0.5% to 1.0% of the population. Between 400,000 and 3.1 million adults have Sjögren’s syndrome.
22
Q
Recognise the presenting symptoms of Sjogren’s Syndrome.
A
- Dry eyes - keratoconjunctivitis sicca
- Dry mount - xerostomia
- Dryness of skin, nose, throat, vagina
- Arthralgias
- Myalgias
- Peripheral neuropathies
- Lymphoma
- Fatigue
- Vasculitis
- Dental caries
- Increased oral fungal and bacterial infections
- Arthritis
- Kidney disease
- Corneal ulceration
- No salvia pool
- Enlarged salivary glands
- Facial pain
- Burning mouth syndrome
- History of VTE
- History of AA or dissection
23
Q
Recognise the signs of Sjogren’s Syndrome on physical examination.
A
- Dry eyes - keratoconjunctivitis sicca
- Dry mount - xerostomia
- Dryness of skin, nose, throat, vagina
- Arthralgias
- Myalgias
- Peripheral neuropathies
- Lymphoma
- Fatigue
- Vasculitis
- Dental caries
- Increased oral fungal and bacterial infections
- Arthritis
- Kidney disease
- Corneal ulceration
- No salvia pool
- Enlarged salivary glands
- Facial pain
- Burning mouth syndrome
- History of VTE
- History of AA or dissection
24
Q
Identify appropriate investigations for Sjogren’s Syndrome and interpret the results.
A
1st Line:
- Schirmer’s test - positive (filter paper placed in lower conjunctival sac, less than 5mm of paper is wetted after 5 mins)
- Anti-60 kD (SS-A) Ro and anti-La (SS-B) - positive
Consider:
- Sialometry - decreased
- Minor salivary gland biopsy - focus score 1 or greater
- Lissamine green test - score of 3 or more
- Fluorescein corneal staining test - score of 3 or more
- Parotid sialography - gross distortion of the normal pattern of parotid ductules coupled with significant retention of contrast material
- Salivary gland Technetium-99m pertechnetate scintigraphy - decreased uptake and secretion
- Skin biopsy - focal and segmental transmural necrotising inflammation in a medium-sized vessel (i.e., a small or medium-sized artery)
- Angiography - beading, aneurysm, or smooth, tapering vessel stenosi
- Urinalysis - may show abnormal levels of phosphate, calcium, potassium, glucose due to renal tubular acidosis
- Serum electrolytes - may show hypokalaemia with a normal anion gap; hyperchloraemic metabolic acidosis
- MRI salivary glands - inflammation of salivary glands
- US salivary glands - high salivary gland ultrasonography score
25
Define SLE.
A chronic multi-system disorder that most commonly affects women during their reproductive years.
26
Explain the aetiology / risk factors of SLE.
- Anti-nuclear antibodies + constitutional symptoms
- Involves skin and joints
- Serositis, nephritis, haematological cytopenias and neurological manifestations
Risk factors:
- Female sex
- Age 15-45 years
- African / Asian descent in Europe and US
- Drugs
- Sun exposure
- Family history of SLE
- Tobacco smoking
27
Summarise the epidemiology fo SLE.
The reported prevalence of systemic lupus erythematosus (SLE) in the United States is 20 to 150 cases per 100,000 [1-3]. In women, prevalence rates vary from 164 (white) to 406 (African American) per 100,000 [2].
28
Recognise the presenting symptoms of SLE.
- Malar (butterfly) rash
- Photosensitive rash
- Discoid rash
- Fatigue
- Weight loss
- Fever
- Oral ulcers
- Alopecia
- Arthralgia / artritis
- Fibromyalgia
- Raynaud's phenomenon
- Chest pain and SOB
- Venous or arterial thrombosis
- Hypertension
- Signs of nephrosis (e.g. oedema)
- Lymphadenopathy
- Abdominal pain, vomiting or diarrhoea
- Nose ulcers
- Poorly localised proximal limb inflammatory pain with weakness
- Dysrhythmias - e.g. tachycardia
- Conduction defects or unexplained cardiomegaly
- CNS signs - seizures, CNS abnormalities, cognitive defects, psychosis
- Dysphagia
29
Recognise the signs of SLE on physical examination.
- Malar (butterfly) rash
- Photosensitive rash
- Discoid rash
- Fatigue
- Weight loss
- Fever
- Oral ulcers
- Alopecia
- Arthralgia / artritis
- Fibromyalgia
- Raynaud's phenomenon
- Chest pain and SOB
- Venous or arterial thrombosis
- Hypertension
- Signs of nephrosis (e.g. oedema)
- Lymphadenopathy
- Abdominal pain, vomiting or diarrhoea
- Nose ulcers
- Poorly localised proximal limb inflammatory pain with weakness
- Dysrhythmias - e.g. tachycardia
- Conduction defects or unexplained cardiomegaly
- CNS signs - seizures, CNS abnormalities, cognitive defects, psychosis
- Dysphagia
30
Identify appropriate investigations for SLE and interpret the results.
1st Line:
- FBC and differential - anaemia, leukopenia, thrombocytopenia, pancytopenia
- Activated PTT - anti-phospholipid antibody patients have prolonged PTT
- U&E - high urea, high creatinine
- ESR and CRP - high
- Anti-nuclear antibodies, dsDNA, Smith antigen - positive
- Urinalysis - haematuria, casts (red cell, granular, tubular or mixed), proteinuria
- CXR - pleural effusion, infiltrates, cardiomegaly
- ECG - exclude other causes
Consider:
- Blood and urine cultures - exclude infection
- Antiphospholipid antibodies - positive
- Coombs test - positive
- 24-hour urine collection for protein or spot urine for protein/creatinine ratio - proteinuria
- Complement levels - complement consumption
- Creatinine phosphokinase - elevated
- Plain XR of affected joints - inflammation, non-erosive arthritis
- Renal USS - exclusion
- Chest CT - lung fibrosis, effusions
- PFT - restrictive pattern
- Pleural aspiration - exudate
- Brain MRI - white matter changes
- Echocardiography - pericarditis, pericardial effusion, pulmonary hypertension
- Skin biopsy - immune deposits at the dermal-epidermal junction on immunofluorescence or non-specific inflammation
- Renal biopsy - immune deposits, mesangial hypercellularity, focal, segmental or global glomerulonephritis
- TSH - exclusion
31
Define systemic sclerosis.
A multi-system, autoimmune disease, characterised by functional and structural abnormalities of small blood vessels, fibrosis of skin and internal organs, production of autoantibodies.
32
Explain the aetiology / risk factors of systemic sclerosis.
Unknown.
2 TYPES:
- Limited cutaneous SSc
- Diffuse cutaneous SSc
Limit - less severe internal organ involvement, better prognosis
Risk factors:
- Family history
- Immune dysregulation - e.g. positive ANA
- Exposure to environmental substances and toxins - e.g. silica dust, solvents
33
Summarise the epidemiology of systemic sclerosis.
Scholarly articles for epidemiology of systemic sclerosis
Epidemiology of systemic sclerosis - Nikpour - Cited by 114
Epidemiology of systemic sclerosis: incidence, … - Barnes - Cited by 310
Epidemiology of systemic sclerosis (scleroderma) - MEDSGER JR - Cited by 343
A 2019 epidemiology study reported that based on 39 publications, the prevalence of systemic sclerosis in Europe and North America was 7.2-33.9 cases per 100,000 individuals, with an annual incidence rate of 0.6-2.3 cases per 100,000 individuals. Systemic sclerosis is rare in the resident population of Japan and China.
34
Recognise the presenting symptoms of systemic sclerosis.
- Raynaud;'s phenomenon
- Digital pits or ulcers
- Swelling of the hands and feet
- Skin thickening
- Loss of function of hands
- Sclerodactyly
- Heartburn reflux and dysphagia
- Bloating
- Faecal incontinence
- Athralgias and myalgias
- Abnormal nail-fold capillaroscopy
- Telangiectasia
- Subcutaneous calcinosis
- Dyspnoea
- Dry crackles at lung bases
- Tendon friction rub
- Abrupt onset moderate / marked hypertension
- Fatigue
- Dry cough
- Decreased exercise tolerance
- Weight loss
- Inflammatory arthritis
- Proximal muscular weakness (inflammatory myositis)
- Synovitis
- Increased accentuation of the pulmonic component of S2 heart sound
- Signs of anaemia
35
Recognise the signs of systemic sclerosis on physical examination.
- Raynaud;'s phenomenon
- Digital pits or ulcers
- Swelling of the hands and feet
- Skin thickening
- Loss of function of hands
- Sclerodactyly
- Heartburn reflux and dysphagia
- Bloating
- Faecal incontinence
- Athralgias and myalgias
- Abnormal nail-fold capillaroscopy
- Telangiectasia
- Subcutaneous calcinosis
- Dyspnoea
- Dry crackles at lung bases
- Tendon friction rub
- Abrupt onset moderate / marked hypertension
- Fatigue
- Dry cough
- Decreased exercise tolerance
- Weight loss
- Inflammatory arthritis
- Proximal muscular weakness (inflammatory myositis)
- Synovitis
- Increased accentuation of the pulmonic component of S2 heart sound
- Signs of anaemia
36
Identify appropriate investigations for systemic sclerosis and interpret the results.
1st Line:
- Serum auto-antibodies - positive ANA
- FBC - microcytic anaemia (GI bleed), microangiopathic haemolytic anaemia (MAHA) in scleroderma renal crisis
- Urea and serum creatinine - high in scleroderma renal crisis
- ESR and CRP - high
- Urine microscopy - proteinuria, cells or casts in scleroderma renal crisis
- Complete PFTs - spirometry, lung volumes and diffusing capacity measurement - interstitial lung disease = low FVC, low DLCO plus restrictive pattern , pulmonary hypertension, disproportionate drop in DLCO compared with FVC
- ECG - cardiac involvement
- Echocardiogram - pulmonary hypertension, rise in RVSP, pericardial effusion, RV or LV diastolic dysfunction present
- CXR- interstitial lung disease (bi-basilar interstitial infiltrates), cardiomegaly, RHF
- Barium swallow - diminished oesophageal peristalsis and gastroparesis, diminished muscle tone in lower oesophagus, reflux of barium, strictures
Consider:
- CT chest - interstitial lung disease (ground glass opacities, thickened interstitium, interstitial fibrosis), traction bronchiectasis, honey-combing
- OGD + biopsy - oesophageal inflammation, ulceration, strictures, gastric antral vascular ectasia, Barrett's metaplasia, adenocarcinoma may be present
- Serum muscle enzymes - elevated in scleroderma myopathy
- Electromyogram /nerve conduction studies - inflammatory myositis
- Muscle biopsy - inflammatory myositis
37
Define sarcoidosis.
Multisystem granulomatous inflammatory disorder.
38
Explain the aetiology / risk factors of sarcoidosis.
Unknown.
Transmissibel agents - e.g. viruses, atypical mycobacterium, Propionibacterium acnes
Environmental triggers
Genetic factors
Unknown antigen presented on MHC Class II complex of macrophages to CD4 Th1 lymphocytes, which accumulate and release cytokines (IL-1, IL-2).
Formation of NON-CASEATING granulomas in a variety of organs.
39
Summarise the epidemiology of sarcoidosis.
Uncommon.
20-40 year olds.
Africans.
Females.
Variable prevalence
UK 16 in 100,000
Highest in Irish women.
40
Recognise the presenting symptoms of sarcoidosis.
General
- Fever
- Malaise
- Weight loss
- Bilateral parotid swelling
- Lymphadenopathy
- Hepatosplenomegaly
Lungs
- Breathlessness
- Cough - usually unproductive
- Chest discomfort
- Minimal clinical signs - e.g. fine inspiratory crackles
Musculosksletal
- Bone cysts - e.g. dactylitis in phalanges
- Polyarthralgia
- Myopathy
Eyes
- Keratoconjunctivitis sicca - dry eyes
- Uveitis
- Papilloedema
Skin
- Lupus pernio - red-blue infiltrations of the nose, cheeky, ears, terminal phalanges
- Erythema nodosum
- Maculopapular eruptions
Neurological
- Lymphocytic meningitis
- Space occupying lesions
- Pituitary infiltration
- Cerebellar ataxia
- Cranial nerve palsies - e.g. bilateral facial nerve palsy
- Peripheral neuropathy
Heart
- Arrhythmia
- Bundle branch block
- Pericarditis
- Cardiomyopathy
- Congestive cardiac failure
41
Recognise the signs of sarcoidosis on physical examination.
General
- Fever
- Malaise
- Weight loss
- Bilateral parotid swelling
- Lymphadenopathy
- Hepatosplenomegaly
Lungs
- Breathlessness
- Cough - usually unproductive
- Chest discomfort
- Minimal clinical signs - e.g. fine inspiratory crackles
Musculosksletal
- Bone cysts - e.g. dactylitis in phalanges
- Polyarthralgia
- Myopathy
Eyes
- Keratoconjunctivitis sicca - dry eyes
- Uveitis
- Papilloedema
Skin
- Lupus pernio - red-blue infiltrations of the nose, cheeky, ears, terminal phalanges
- Erythema nodosum
- Maculopapular eruptions
Neurological
- Lymphocytic meningitis
- Space occupying lesions
- Pituitary infiltration
- Cerebellar ataxia
- Cranial nerve palsies - e.g. bilateral facial nerve palsy
- Peripheral neuropathy
Heart
- Arrhythmia
- Bundle branch block
- Pericarditis
- Cardiomyopathy
- Congestive cardiac failure
42
Identify appropriate investigations for sarcoidosis and interpret the results.
Bloods
- High serum ACE
- Hight Ca2+
- High ESR
- WCC low due to lymphocyte sequestration in lungs
- Immunoglobulins - polyclonal hyperglobulinaemia
- LFTs - high AlkPhos & GGT
24h Urine Collection
- Hypercalciuria
CXR
- Stage 0 - may be clear
- Stage 1 - bilateral hilar lymphadenopathy
- Stage 2 - bilateral hilar lymphadenopathy with pulmonary infiltration and paratracheal node enlargement
- Stage 3 - Pulmonary infiltration and fibrosis
High-Resolution CT Scan
- For diffuse lung involvement
67-Gallium Scan
- Shows areas of inflammation (classically parotids and around eye)
Pulmonary Function Tests
- Reduced FEV1, FVC and gas transfer
- Restrictive pattern
Bronchoscopy and Bronchoalveolar Lavage
- Raised lymphocytes
- Raised CD4:CD8 ratio
Transbronchial Lung Biopsy (or Lymph Node Biopsy)
- Non-caseating granulomas composed of epithelioid cells (activated macrophages)
- Multinucleate Langhans cells
- Mononuclear cells (lymphocytes)
43
Define ankylosing spondylitis.
Axial spondyloarthritis (AxSpa) = a chronic progressive inflammatory arthropathy.
Non-radiographic axial spondyloarthritis (nr-axSpA) = axial inflammation visible on MRI that has not caused substantial erosive damage to sacroiliac joints.
44
Explain the aetiology / risk factors of ankylosing spondylitis.
SERONEGATIVE SPONDYLOARTHROPATHY
- e.g. psoriatic arthritis, enteropathic arthritis, reactive arthritis.
= heterogenous group of conditions with overlapping clinical manifestations and associated with HLA-B27.
Mainly affects axial spine and sacroiliac joints.
Peripheral joints, entheses (tendon or ligaments attached to bone), and extra-articular sites (e.g. eye, bowel) are affected.
May lead to spinal fusion, following radiographical changes in spine and sacroiliac joints.
(Bamboo spine)
Risk factors:
- HLA-B27
- ERAP1 and IL23R genes
- Positive family history
- Male sex
- Klebsiella pneumoniae
45
Summarise the epidemiology of ankylosing spondylitis.
Epidemiology. Recent population estimates indicate that the prevalence of AS in the United States is approximately 0.2-0.5%. Based on data from multiple countries, the age- and sex-adjusted incidence of AS is 0.4-14 per 100,000 person-years.
46
Recognise the presenting symptoms of ankylosing spondylitis.
- Inflammatory back pain
- Iritis/uveitis
- Enthesitis
- Presentation in late teens and early 20s
- Male sex
- Positive family history of AS
- Fatigue
- Sleep disturbance
- Tenderness at sacroiliac joint
- Dyspnoea
- Loss of lumbar lordosis
- Peripheral joint involvement
- Kyphosis
- Psoriasis
- Symptoms of IBD
47
Recognise the signs of ankylosing spondylitis on physical examination.
- Inflammatory back pain
- Iritis/uveitis
- Enthesitis
- Presentation in late teens and early 20s
- Male sex
- Positive family history of AS
- Fatigue
- Sleep disturbance
- Tenderness at sacroiliac joint
- Dyspnoea
- Loss of lumbar lordosis
- Peripheral joint involvement
- Kyphosis
- Psoriasis
- Symptoms of IBD
48
Identify appropriate investigations for ankylosing spondylitis and interpret the results.
1st Line
- Pelvic XRay - sacroiliitis
Consider:
- HLA -B27 - positive or negative (not diagnostic!)
- MRI - bone marrow oedema on T2-weighted sagittal STIR image
- Spinal X Ray (lateral) - erosions, squaring, sclerosis, syndesmophytes, bridging syndesmophytes in the cervical & lumbar & thoracic spine, bamboo spine
- USS - enthesitis
49
Define gout.
Characterized by:
- Hyperuricaemia
- Deposition of urate srystals
- Acute inflammatory arthritis
- Tophi around joints
- Joint destruction
- Renal glomerular, tubular and interstitial disease
- Uric acid urolithiasis
50
Explain the aetiology / risk factors of gout.
Commonly affects:
- 1st toe
- Foot
- Ankle
- Knee
- Fingers
- Wrist
- Elbow
(can affect any joint)
Risk factors:
- Older age
- Male sex
- Menopausal status
- Consumption of meat, seafood, alcohol
- Use of diuretics
- Use of ciclosporin or tacrolimus
- Use of pyrazinamide
- Use of aspirin
- Genetic susceptibility
- High cell turnover rate
- Obesity
- Adiposity and insulin resistance
- Exogenous insulin
- Hypertension
- Renal insufficiency
- DM
- Hyperlipidaemia
- Family history of gout
51
Summarise the epidemiology of gout.
Recent reports of the prevalence and incidence of gout vary widely according to the population studied and methods employed but range from a prevalence of <1% to 6.8% and an incidence of 0.58–2.89 per 1,000 person-years. Gout is more prevalent in men than in women, with increasing age, and in some ethnic groups
52
Recognise the presenting symptoms of gout.
- Acute onset of severe joint pain
- Swelling
- Effusion
- Warmth
- Erythema
- Tenderness of involved joint
- Joint stiffness
- Foot joint distribution
- Few affected joints
- Tophi over extensor surface joints (elbows, knees, Achilles tendons)
- Family history of gout
53
Recognise the signs of gout on physical examination.
- Acute onset of severe joint pain
- Swelling
- Effusion
- Warmth
- Erythema
- Tenderness of involved joint
- Joint stiffness
- Foot joint distribution
- Few affected joints
- Tophi over extensor surface joints (elbows, knees, Achilles tendons)
- Family history of gout
54
Identify appropriate investigations for gout and interpret the results.
1st line:
- Arthrocentesis with synovial fluid analysis - strongly NEGATIVE birefringent needle-shaped crystals under polarised light, WBC > 2.0 / 10^9/L
Investigations to consider:
- Serum uric acid level - >420 micromol/L in men, >360 micromol/L in women
- USS - erosions, tophi, double contour line
- DECT - erosions, tophi, double countour line
- XR - periarticular erosions with overhanging edge or punched out appearance
55
Define psuedogout.
= Calcium Pyrophosphate deposition
Associated with both acute and chronic arthritis.
ACUTE = acute inflammatory arthritis of one or more joints, affecting knees, wrists, shoulders, ankles, elbows or hands
CHRONIC = mimics osteoarthritis or RA, associated with variable degrees of inflammation.
56
Explain the aetiology / risk factors of pseudogout.
Caused by deposition of calcium pyrophosphate (CPP) crystals.
Underlying metabolic conditions associated - e.g. hyperparathyroidism, hemochromatosis.
Risk factors:
- Advanced age
- Injury
- Hyperparathyroidism
- Haemachromatosis
- Family history of CPPD
- Hypomagnesaemia
- Hypophosphatasia
- Gout
57
Summarise the epidemiology of pseudogout.
The frequency of pseudogout varies with age. The annual incidence of acute attacks of arthritic pain and swelling is about 1.3 per 1000 adults, but nearly 50% of adults develop radiographic changes typical of CPPD by age 80 years.
58
Recognise the signs of pseudogout on physical examination.
- Presence of risk factors
- Painful and tender joints
- Osteoarthritis-like involvement of joints (wrists, shoulders)
- Sudden worsening of osteoarthritis
- Red and swollen joints
- Joint effusion and fluctuance
- Fever and malaise
59
Identify appropriate investigations for pseudogout and interpret the results.
1st:
- Arthrocentesis with synovial fluid analysis - intracellular or extracellular POSITIVELY birefringent rhomboid-shaped crystals under polarised light, fluids often bloody
- XR of affected joints - linear, stippled radio-opaque deposits in fibro-cartilage or hyaline articular cartilage of joints, calcified tendons, subchondral cysts, progressive rapid joint degeneration or bony collapse, predominant involvement of patellofemoral joint in knee
- Serum calcium - normal or HIGH
- Serum PTH - normal or HIGH
- Iron studies - normal or HIGH
- Serum Mg - normal or LOW
- Serum AlkPhos - normal or LOW
Consider:
- Ultrasonography - calcified deposits in articular tissue
- CT scan - calcified deposits in articular cartilage
60
Define fibromyalgia.
A chronic pain syndrome diagnosed by the presence of widespread body pain.
CRITERIA:
- Widespread pain - front and back, right and left, both sides of diaphragm
- For at least 3 months
- Tenderness 11/18 designated tender point sites
- Fatigue + memory and sleep difficulties
61
Explain the aetiology / risk factors of fibromyalgia.
No single cause.
CNS-driven pain amplification syndrome.
Risk factors:
- Family history of fibromyalgia
- Rheumatological conditions
- Age between 20-60 years
- Female sex
- Presence of associated conditions
62
Summarise the epidemiology of fibromyalgia.
Chronic widespread pain, the cardinal symptom of fibromyalgia (FM), is common in the general population, with comparable prevalence rates of 7.3% to 12.9% across different countries. The prevalence of FM in the general population was reported to range from 0.5% to 5% and up to 15.7% in the clinic.
63
Recognise the presenting symptoms of fibromyalgia.
- Chronic pain
- Fatigue unrelieved by rest
- Sleep disturbance
- Mood disturbance
- Cognitive dysfunction
- Headaches
- Numbness / tingling sensations
- Stiffness
- Sensitivity to sensory stimuli - e.g. bright lights, odours, noises
64
Recognise the signs of fibromyalgia on physical examination.
Typically normal, often diffuse tenderness - may be assessed by counting number of tender points or by palpating several areas of the body.
65
Identify appropriate investigations for fibromyalgia and interpret the results.
1st line:
- Clinical diagnosis - chronic (>3 months), widespread body pain, fatigue, sleep disturbance
Consider:
- ESR / CRP - normal (exclude inflammatory muscular pain / fatigue)
- TFTs - normal (exclude hypothyroidism)
- FBC - normal (exclude anaemia)
- Rheumatoid factor - normal (exclude RA)
- Anti-CCP antibody - normal (exclude RA)
- Anti-nuclear antibody - normal (exclude SLE)
- Vit D level - normal (exclude deficiency)
66
Define giant cell arteritis.
A granulomatous vasculitis or large and medium-size arteries.
AKA - temporal arteritis, cranial arteritis, granulomatous arteritis.
67
Explain the aetiology / risk factors of giant cell arteritis.
Affects branches of external carotid artery.
Risk factors:
- Age > 50 years
- Female sex
- Genetic factors
- Smoking
- Atherosclerosis
- Environmental factors
68
Summarise the epidemiology of giant cell arteritis.
Most common form of systemic vasculitis in adults.
>50 years.
Women.
69
Recognise the presenting symptoms of giant cell arteritis.
- Presence of risk factors
- Headache
- Polymyalgia rheumatica symptoms - e.g. aching and stiffness in neck, shoulders, hips and proximal extremities, worsens after a period of inactivity and with movement
- Pain and swelling of distal joints
- Extremity claudication
- Cranial artery abnormalities
- Loss of vision
- Jaw claudication - pain chewing, unilateral or bilateral
- Superficial temporal artery tenderness, thickening or nodularity
- Absent temporal artery pulse
- Abnormal fundoscopy
- Systemic symptoms
- Cough, sore throat and hoarseness
- Bruit on auscultation
- Asymmetric blood pressure
- Shoulder tenderness
- Limited active range of movement of shoulders and hips
- Wrist and knee swelling
70
Recognise the signs of giant cell arteritis on physical examination.
- Presence of risk factors
- Headache
- Polymyalgia rheumatica symptoms - e.g. aching and stiffness in neck, shoulders, hips and proximal extremities, worsens after a period of inactivity and with movement
- Pain and swelling of distal joints
- Extremity claudication
- Cranial artery abnormalities
- Loss of vision
- Jaw claudication - pain chewing, unilateral or bilateral
- Superficial temporal artery tenderness, thickening or nodularity
- Absent temporal artery pulse
- Abnormal fundoscopy
- Systemic symptoms
- Cough, sore throat and hoarseness
- Bruit on auscultation
- Asymmetric blood pressure
- Shoulder tenderness
- Limited active range of movement of shoulders and hips
- Wrist and knee swelling
71
Identify appropriate investigations for giant cell arteritis and intepret the results.
1st line
- ESR - HIGH >50mm/hour by Westergren method
- CRP - HIGH
- FBC - normochromic, normocytic anaemia, normal WCC, HIGH platelets, leukocytosis
- LFTS - transaminases, AlkPhos
- Temporal artery ultrasound - wall thickening (halo sign), stenosis, occlusion
- Temporal artery biopsy - granulomatous inflammation, multinucleated giant cells, inflammatory infiltrate focal and segmental
Consider:
- Aortic arch angiography - stenosis or occlusion of subclavian, axillary or brachial arteries, smoothly tapered stenoses, aortitis (thickening or oedema of aortic wall)
- FDG-PET scan of head to mid-thigh - FDG uptake in aorta and major branches
- USS of upper extremity arteries - mural thickening, stenosis or occlusion
72
Generate a management plan for giant cell arteritis.
INITIAL
- Prednisolone - taper dose over 6-12 months
- Methylprednisolone pulse therapy (visual or neurological signs) - 1g IV OD for 3 days
CONFIRMED
- Prednisolone - taper dose over 6-12 months
- Aspirin - 75mg oral OD
- Osteoporosis prevention - calcium carbonate + ergocalciferol + alendronic acid / risedronate sodium
- Relapse - Toclizumab or Methotrexate
73
Identify the possible complications of giant cell arteritis and its management.
- Irreversible loss of vision due to optic nerve ischemia
- Aortic aneurysms
- Large vessel stenoses
- Stroke
74
Summarise the prognosis for patients with giant cell arteritis.
When giant cell arteritis is diagnosed and treated early, the prognosis is usually excellent. Your symptoms will likely improve quickly after beginning corticosteroid treatment, and your vision isn't likely to be affected.
75
Define idiopathic inflammatory myopathies (including polymyositis, dermatomyositis).
A heterogeneous group of sub-acute, chronic and acute diseases of skeletal muscle hat have in common the presence of moderate-to-severe proximal muscle weakness and inflammation on muscle biopsy.
76
Explain the aetiology / risk factors of idiopathic inflammatory myopathies (including polymyositis, dermatomyositis).
3 GROUPS:
- POLYMYOSITIS - autoimmune
- DERMATOMYOSITIS - autoimmune
- INCLUSION BODY MYOSITIS - autoimmune, degenerative
Risk factors:
- Children and age > 40yrs
- Exposure to high intensity of global UV radiation
- Genetic predisposition
- Female sex and/or black ethnicity - polymyositis and dermatomyositis
- Male sex and/or white ethnicity (inclusion body myositis)
- Lipid-lowering agents
- HIV
- Viral infections
- Non-viral infections
- Vaccination
- D-penicillamine
- Drugs or toxins
77
Summarise the epidemiology of idiopathic inflammatory myopathies (including polymyositis, dermatomyositis).
Idiopathic inflammatory myopathies have a reported incidence of 0.1 to 1 per 100,000 person-years and prevalence of 0.55 to 6 per 100,000 in the United States.
78
Recognise the presenting symptoms of idiopathic inflammatory myopathies (including polymyositis, dermatomyositis).
- Presence of risk factors
- Difficulty with motor tasks
- Muscle weakness
- Muscle atrophy
- Heliotrope rash with eyelid oedema - blue-purple discolouration of upper eyelids with oedema (dermatomyositis)
- Gottron's papules - erythema over the knuckles with raised violaceous scalp eruption (dermatomyositis)
- Frequent falls
- Fatigue and generalised malaise
- Weight loss
- SOB
- Fever
- Abnormal breath sounds
- Dysphagia
- Myalgia
- Arthralgia
- Palpitations
- Syncope
- Symptoms of MI
- Facial rash
- Erythematous rash
- Nail fold changes - dilation of capillary loops of periungual area (dermatomyositis)
- Facial muscle weakness
- Skin calcinosis
- Joint swelling
- Arrhythmias
- Signs of heart failure and/or MI
- Physical findings of malignancy
- Systemic signs of autoimmune disease
- Peripheral neuropathy
79
Recognise the signs of idiopathic inflammatory myopathies (including polymyositis, dermatomyositis) on physical examination.
- Presence of risk factors
- Difficulty with motor tasks
- Muscle weakness
- Muscle atrophy
- Heliotrope rash with eyelid oedema - blue-purple discolouration of upper eyelids with oedema (dermatomyositis)
- Gottron's papules - erythema over the knuckles with raised violaceous scalp eruption (dermatomyositis)
- Frequent falls
- Fatigue and generalised malaise
- Weight loss
- SOB
- Fever
- Abnormal breath sounds
- Dysphagia
- Myalgia
- Arthralgia
- Palpitations
- Syncope
- Symptoms of MI
- Facial rash
- Erythematous rash
- Nail fold changes - dilation of capillary loops of periungual area (dermatomyositis)
- Facial muscle weakness
- Skin calcinosis
- Joint swelling
- Arrhythmias
- Signs of heart failure and/or MI
- Physical findings of malignancy
- Systemic signs of autoimmune disease
- Peripheral neuropathy
80
Identify appropriate investigations for idiopathic inflammatory myopathies (including polymyositis, dermatomyositis) and interpret the results.
1st Line:
- CK - HIGH
- EMG - short duration, low amplitude, polyphasic units with early recruitment on voluntary activity, diffuse spontaneous activity with fibrillation and positive sharp waves at rest
- Muscle biopsy - polymyositis (endomysial inflammatory infiltrates, muscle necrosis, atrophy, muscle fibre regeneration), dermatomyositis (perifascicular atrophy, perivascular/ perimysial inflammation), inclusion body myositis (endomysial inflammatory infiltrate, fibre size variability, fibre necrosis, rimmed vacuoles)
- Aldolase - HIGH
- LDH - HIGH
- Alanine transaminases - HIGH
- Myoglobin - HIGH
Consider:
- ESR - HIGH
- ANA - HIGH
- Myositis-specific antibodies - type-specific antibodies
- Soluble CD30 - HIGH
- Type 1 interferon - HIGH
- MRI - increased signal or oedema seen in inflammatory processes, changes are not specific
- USS - image suggestive of inflammatory processes
81
Define osteoarthritis (including carpometacarpal, shoulder, spine, hip and knee).
The result of mechanical and biological events that destabilise the normal process of degeneration and synthesis of articular cartilage chondrocytes, ECM and subchondral bone.
82
Explain the aetiology / risk factors of osteoarthritis (including carpometacarpal, shoulder, spine, hip and knee).
Involves entire joint - articular cartilage, subchondral bone, pericapsular muscles, capsule and synovium.
Results in loss of cartilage, sclerosis and eburnation of subchondral bone, osteophytes and subchondral cysts.
Most commonly affects knee, hip, hands, lumbar and cervical spine.
Risk factors:
- Age > 50 yrs
- Female
- Obesity
- Genetic factors
- Knee malalignment
- Physically demanding occupation / sport
- High bone mineral density
83
Summarise the epidemiology of osteoarthritis (including carpometacarpal, shoulder, spine, hip and knee).
Approximately 1 in 5 adults (18.2%) over 45 years of age in England has osteoarthritis of the knee. The prevalence ranges from around 15% to 21% across local authorities in England. The rate of knee replacements in local authority areas ranges from 1/1,000 to 6/1,000 people over 45 years.
84
Recognise the presenting symptoms of osteoarthritis (including carpometacarpal, shoulder, spine, hip and knee).
Joint pain, stiffness + functional limitation.
Worse with activity.
- Presence of risk factors
- Pain
- Functional difficulties
- Knee, hip, hand or spinal involvement
- Bony deformities
- Limited range of motion
- Malalignment
- Tenderness
- Crepitus
- Stiffness
- Shoulder, elbow, wrist or ankle involvement
- Effusion
- Antalgic gait
85
Recognise the signs of osteoarthritis (including carpometacarpal, shoulder, spine, hip and knee) on physical examination.
Joint pain, stiffness + functional limitation.
Worse with activity.
- Presence of risk factors
- Pain
- Functional difficulties
- Knee, hip, hand or spinal involvement
- Bony deformities
- Limited range of motion
- Malalignment
- Tenderness
- Crepitus
- Stiffness
- Shoulder, elbow, wrist or ankle involvement
- Effusion
- Antalgic gait
86
Identify appropriate investigations for osteoarthritis (including carpometacarpal, shoulder, spine, hip and knee) and interpret the results.
Loss of joint space, subchondral sclerosis and osteophytes.
87
Identify appropriate investigations for osteoarthritis (including carpometacarpal, shoulder, spine, hip and knee) and interpret the results.
Loss of joint space, subchondral sclerosis and osteophytes.
1st line:
- XR of affected joints - new bone formation (osteophytes), joint narrowing, subchondral sclerosis, subchondral cysts
- CRP - normal
- ESR - normal
Consider:
- RF - negative
- Anti-CCP antibody - negative
- MRI - cartilage loss, bone marrow lesions, meniscal tears
88
Define polymyalgia rheumatica.
An inflammatory rheumatological syndrome that manifests as pain and morning stiffness involving neck, shoulder girdle, pelvic girdle in individuals >50 yrs.
89
Explain the aetiology / risk factors of polymyalgia rheumatica.
Associated with GCA.
Risk factors:
- >50 years
- GCA
- Female
90
Summarise the epidemiology of polymyalgia rheumatica.
Women.
Associated with GCA.
About 15% to 20% of patients with polymyalgia rheumatica (PMR) have giant cell arteritis (GCA); 40% to 60% of GCA patients have PMR.
91
Recognise the presenting symptoms of polymyalgia rheumatica.
Difficulty rising from seated or prone positions, varying degrees of muscle tenderness, shoulder / hip bursitis and/or oligoarthritis.
- Presence of risk factors
- Shoulder / hip girdle stiffness
- Shoulder / hip girdle pain
- Rapid response to corticosteroids
- Acute onset
- Low-grade fever
- Anorexia
- Weight loss
- Malaise
- Depression
- Asthenia - physical weakness, lack of energy
- Oligoarticular arthritis
92
Recognise the signs of polymyalgia rheumatica on physical examination.
Difficulty rising from seated or prone positions, varying degrees of muscle tenderness, shoulder / hip bursitis and/or oligoarthritis.
- Presence of risk factors
- Shoulder / hip girdle stiffness
- Shoulder / hip girdle pain
- Rapid response to corticosteroids
- Acute onset
- Low-grade fever
- Anorexia
- Weight loss
- Malaise
- Depression
- Asthenia - physical weakness, lack of energy
- Oligoarticular arthritis
93
Identify appropriate investigations for polymyalgia rhuematica and interpret the results.
1st line:
- ESR - HIGH
- CRP - HIGH
- FBC - variable
- USS - bursitis, joint effusions
Consider:
- TSH - normal or raised in hypothyroidism
- MRI - bursitis, joint effusions
- Serum protein electrophoresis - normal
- IL-6 - HIGH
- Fluorodeoxyglucose F18 PET scan - joint inflammation, arteritis
94
Generate a management plan for polymyalgia rheumatica.
INITIAL
- Corticosteroid - prednisolone, then methylprednisolone
- Calcium + VitD + Bisphosphonate - alendronic acid or risedronate sodium + colecalciferol + calcium carbonate/citrate
- NSAID - naproxen or ibuprofen
- Methotrexate
- Folic acid - decrease risk of methotrexate adverse effects (oral ulcers, bone marrow suppression)
- Toclizumab
RELAPSE
- Corticosteroid - increased dose
- Calcium + VitD + Bisphosphonate - - alendronic acid or risedronate sodium + colecalciferol + calcium carbonate/citrate
- Methotrexate + Folic Acid
- Toclizumab or Leflunomide
95
Identify the possible complications of polymyalgia rheumatica and its management.
- GCA
| - Affects ADLs significantly
96
Summarise the prognosis for patients with polymyalgia rheumatica.
With early diagnosis and correct therapy, patients have an excellent prognosis. The average length of disease is 3 years. However, exacerbations may occur if steroids are tapered too rapidly, and relapse is common, affecting up to 25% of all treated patients.
97
Define reactive arthritis.
An inflammatory arthritis that occurs after exposure to GI and GU infections.
98
Explain the aetiology / risk factors of reactive arthritis.
TRIAD - post-infectious arthritis, non-gonococcal urethritis and conjunctivitis.
SPONDYLOARTHROPIES
- e.g. psoriatic arthritis, ankylosing spondylitis, arthritis related to IBD, undifferentiated spondyloarthropathy.
Similar clinical, radiographic and laboratory features - e.g. spinal inflammation, HLA-B27
Asymmetrical oligoarticular arthritis.
Large joints of lower limb.
Risk factors:
- Male sex
- HLA-B27 genotype
- Preceding chlamydial or GI infection
- BCG immunotherapy
99
Summarise the epidemiology of reactive arthritis.
The condition occurs worldwide, predominantly affects young adults in the 20–40 age group, and affects men more so than women (ratio 3:1). The risk of developing reactive arthritis is about 50 times greater in those people who are HLA B27 positive. The incidence in Europe is estimated at 30/100 000.
100
Recognise the presenting symptoms of reactive arthritis.
History of antecedent genitourinary or dysenteric infection 1-4 weeks before onset.
- Presence of risk factors
- Peripheral arthritis
- Axial arthritis
- Constitutional symptoms - e.g. fever, fatigue, weight loss
- Enthesitis
- Mucous membrane involvement
- Skin rash - e.g. keratoderma blenorrhagia
- Circinate balanitis
- Ocular manifestations - conjunctivities, redness, tearing, sterile purulent discharge, photophobia
- Cardiac manifestations - aortitis, aortic regurg, heart failure, arrhythmias, scarring of conduction system
101
Recognise the signs of reactive arthritis on physical examination.
History of antecedent genitourinary or dysenteric infection 1-4 weeks before onset.
- Presence of risk factors
- Peripheral arthritis
- Axial arthritis
- Constitutional symptoms - e.g. fever, fatigue, weight loss
- Enthesitis
- Mucous membrane involvement
- Skin rash - e.g. keratoderma blenorrhagia
- Circinate balanitis
- Ocular manifestations - conjunctivities, redness, tearing, sterile purulent discharge, photophobia
- Cardiac manifestations - aortitis, aortic regurg, heart failure, arrhythmias, scarring of conduction system
102
Identify appropriate investigations for reactive arthritis.
1st Line:
- ESR - HIGH
- CRP - HIGH
- ANA - negative
- RF - negative
- Urogenital and stool cultures- negative unless patients tested very early after onset of infection
- Plain XR - sacroiliitis, enthesopathy
- Arthrocentesis with synovial fluid analysis - negative
Consider:
- HLA-B27 - positive or negative
- Nucleic acid amplification tests - Chlamydia trachomatis, Neisseria gonorrhoea (STI acquired reactive arthritsi)
- MRI - sacroiliitis or enthesopathy
- USS - synovial hypertrophy, increased vascularity
103
Define rheumatoid arthritis.
A chronic inflammatory condition affecting 1% of the population.
Erosive arthritis that requires early and aggressive treatment.
104
Explain the aetiology / risk factors of rheumatoid arthritis.
Small joints of hands and feet.
Can cause major loss of work, decreased quality of life, need for joint replacement surgery and mortality.
Risk factors:
- Genetic predisposition
- Smoking
105
Summarise the epidemiology of rheumatoid arthritis.
Most common inflammatory arthritis seen by physicians.
1% of population.
50-55 years.
106
Recognise the presenting symptoms of rheumatoid arthritis.
- Active symmetrical arthritis lasting >6 weeks
- Female
- Joint main
- Joint swelling
- Morning stiffness
- Swan neck deformity
- Boutonniere's deformity
- Ulnar deviation
- Rheumatoid nodules
- Vasculitic lesions
- Pleuritic chest pain
- Scleritis
- Uveitis
107
Recognise the signs of rheumatoid arthritis on physical examination.
- Active symmetrical arthritis lasting >6 weeks
- Female
- Joint main
- Joint swelling
- Morning stiffness
- Swan neck deformity
- Boutonniere's deformity
- Ulnar deviation
- Rheumatoid nodules
- Vasculitic lesions
- Pleuritic chest pain
- Scleritis
- Uveitis
108
Identify appropriate investigations for rheumatoid arthritis and interpret the results.
1st Line:
- RF - positive
- Anti-CCP antibody - positive
- Radiographs - erosions
- USS - synovitis of wrist and fingers
Consider:
- Disease activity score - affirmative
109
Define lumbosacral radiculopathy.
A disorder that causes pain in the lower back and hip, which radiates down the back of the thigh into the leg.
Describes a predictable constellation of symptoms occuring secondary to mechanical and/or inflammatory cycles compromising at least one of the lumbosacral nerve roots.
110
Explain the aetiology / risk factors of radiculopathy.
Damage caused by compression fo nerve roots exiting spine at levels L1-S4.
Most disc herniations occur posterolaterally, root that gets compressed is actually the root that exists the foramen below the herniated disc. Protrusion at L4/L5 will compress L5 root, protrusion at L5/S1 will compress S1 root.
Most common in lower back and neck = lumbar-sacral, cervical.
Majority of cases are benign and will resolve spontaneously.
Risk factors:
- Activities placing an excessive or repetitive load on spine
- Heavy labour
- Contact sports
- Age 45-64 yrs
- Smoking
- Mental stress
- Frequent lifting
- Driving - vibration of whole body
Causes:
- Lesions of intervertebral discs and degenerative disease of spine
- Herniated disc with nerve root compression
- Tumours
- Congenital abnormalities
- Scoliosis
- Osteomyelitis
111
Summarise the epidemiology of radiculopathy.
3-5% prevalence rate.
112
Recognise the presenting symptoms of radiculopathy.
- Tingling
- Radiating pain
- Numbness
- Paraesthesia
- Shooting pain
- Gait abnormalities
- Predictable patterns affecting corresponding dermatome or myotome.
Sciatica:
- Unilateral leg pain greater than lower back pain
- Leg pain follows dermatomal pattern
- Pain traveling below knee to foot or toes
- Numbness and paraesthesia
- Straight leg raise positive - induces more pain
113
Recognise the signs of radiculopathy on physical examination.
- Tingling
- Radiating pain
- Numbness
- Paraesthesia
- Shooting pain
- Gait abnormalities
- Predictable patterns affecting corresponding dermatome or myotome.
Sciatica:
- Unilateral leg pain greater than lower back pain
- Leg pain follows dermatomal pattern
- Pain traveling below knee to foot or toes
- Numbness and paraesthesia
- Straight leg raise positive - induces more pain
114
Identify appropriate investigations for radiculopathy and interpret the results.
DDX:
- Radicular syndrome/ sciatica
- Pseuoradicular syndrome
- Thoracic disc injuries
- Low back pain
- Cauda equina
- Inflammatory / metabolic causes - e.g. diabetes, ankylosing spondylitis, Paget's disease, Arachnoiditis, Sarcoidosis
- Trochanteric bursitis
- Intraspinal synovial cysts
CXR - presence of trauma or osteoarthritis, signs of tumour or infetcion
EMG - detect radiculopathies, limited utility in diagnosis
MRI - see if disc herniation and nerve root compression are present
115
Define spondylosis.
A degenerative process affecting the vertebral disc and facet joints that gradually develops with age.
116
Explain the aetiology / risk factors of spondylosis.
From any cause.
Most common cause is spinal osteoarthritis.
Affects vertebral bodies, neural foramina and facet joints.
If severe, can cause pressure on spinal cord or nerve roots with sensory or motor disturbances - e.g. pain, paresthesia, imbalance, muscle weakness.
Causes:
- Years of constant abnormal pressure being placed on vertebrae and discs between them
- Joint subluxation
- Stress induced by sports
- Acute or repetitive trauma
- Poor posture
Risk factors:
- Genetic tendency
- Obseity
- Sedentary lifestyle
- Lack of exercise
- Injury to spinal
- Spinal surgery
- Smoking
- Repetitive weight-bearing movements
- Mental health - anxiety, depression
- Psoriatic arthritis
117
Summarise the epidemiology of spondylosis.
The prevalence of radiographic spondylosis increases with age. It is present only in a small percentage of the population in the first few decades of life, but is common by the age of 65 years . In those with low back pain, prevalence ranges from 7 to 75%, depending on the diagnostic criteria
118
Recognise the presenting symptoms of spondylosis.
- Stiffness
- Mild pain following certain movements or long periods without moving
- Grinding or popping feeling when moving the spine
- Weakness in hands and legs
- Poor co-ordination
- Muscle spasms and pain
- Headaches
- Loss of balance and difficulty walking
- Loss of bladder or bowel control
119
Recognise the signs of spondylosis on physical examination.
- Stiffness
- Mild pain following certain movements or long periods without moving
- Grinding or popping feeling when moving the spine
- Weakness in hands and legs
- Poor co-ordination
- Muscle spasms and pain
- Headaches
- Loss of balance and difficulty walking
- Loss of bladder or bowel control
120
Define carpal tunnel syndrome.
A collection of symptoms and signs caused by compression of the median nerve in the carpal tunnel.
121
Explain the aetiology / risk factors of carpal tunnel syndrome.
Risk factors:
- Age over 30 years
- High BMI
- Female sex
- Alterations in carpal tunnel space
- Fractured wrist / carpal bones
- Square wrist
- RA
- DM
- Dialysis
- Pregnancy
- Congenital carpal tunnel stenosis
- Occupation involving exposure to repetitive bending, twisting or vibration of hands or wrists
- Mobility aids
- Smoking
- White ethnicity
- Thyroid disorders
- HRT
- Oophorectomy
- Occupational keyboard / computer use
- Physical inactivity
- Sports and activities
122
Summarise the epidemiology of carpal tunnel syndrome.
Female
40-60 years
Most common entrapment neuropathy.
1 in 25.
123
Recognise the presenting symptoms of carpal tunnel syndrome.
- Numbness and tingling of thumb and radial fingers
- Aching wrist
- Clumsiness
- Radiation in anterior wrist and forearm
- Night-time worsening
- Intermittent
- Gradual onset
- Weakness of thenar muscles - abductor pollicis brevis
- Normal reflexes
- Finger stiffness
- Cold sensitivity
- Atrophy of thenar eminence
124
Recognise the signs of carpal tunnel syndrome on physical examination.
Useful for ruling out other diagnoses.
125
Identify appropriate investigations for carpal tunnel syndrome and interpret the results.
1st Line:
- EMG - focal slowing of conduction velocity in median sensory nerves across carpal tunnel, prolongation of median distal motor latency, decreased amplitude of median sensory and/or motor nerves
Consider:
- USS - space-occupying lesion identified - e.g. ganglion cyst
- MRI - space-occupying lesion identified - e.g. ganglion cyst
126
# Define vasculitides.
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome)
- Granulomatosis with polyanigiitis
- Microscopic polyangitis
- Polyarteritis nodosa
- Takayasu arteritis
- Giant cell arteritis (GCA)
Vasculitis - the inflammation and necrosis of blood vessels.
Classification according to vessel size:
- LARGE = Giant cell arteritis, Takayasu's aortitis
- MEDIUM = Polyarteritis nodosa, Kawasaki's disease
- SMALL = Churg-Strauss Syndrome, microscopic polyangiitis, Henoch-Schonlein purpura, Wegener's granulomatosis, mixed essential cryoglobinaemia, relapsing polychondritis
Summary:
Large = GCA, TA
Medium - PN, KD
Small = CSS, MP, HSP, WG, MEC, RP
EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CSS)
- Triad of tissue eosinophilia, granulomatous inflammation + vasculitis
GRANULOMATOSIS WITH POLYANGIITIS (WG)
- Triad of upper and lower respiratory tract involvement and pauci-immune glomerulonephritis
MICROSCOPIC POLYANGIITIS
- Ill-defined
- Characterised by systemic, pauci-immune, necrotizing, small vessel vasculitis without evidence of necrotizing granulomatous inflammation
POLYARTERITIS NODOSA
- Necrotising inflammation of medium-sized or small arteries
- Without glomerulonephritis or vasculitis in arterioles, capillaries or venules
TAKAYASU'S ARTERITIS
- Affects aorta and main branches
- Can cause stenosis, occlusion and aneurysm formation
GIANT CELL ARTERITIS / TEMPORAL ARTERITIS
- Granulomatous inflammation of large arteries
- Branches of external carotid artery - temporal artery most common
127
Explain the aetiology / risk factors of vasculitides.
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome)
- Granulomatosis with polyanigiitis
- Microscopic polyangitis
- Polyarteritis nodosa
- Takayasu arteritis .
- Giant cell arteritis (GCA)
Unknown aetiology.
?Autoimmune origin.
Immune complex deposition in vessel walls triggers classical complement activation and inflammation.
EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CSS)
- May have perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) directed against myeloperoxidase (MPO)
- Risk factors - history of asthma, allergic rhinitis, sinusitis
GRANULOMATOSIS WITH POLYANGIITIS (WG)
- Anti-neutrophil cytoplasmic antibody (ANCA)
- Common involvement of cutaneous, ocular, MSK and peripheral nervous system tissue
- Small and medium vessels
- Risk factors - genetic (HLA-DP, alpha-1-antitrypsin, proteinase 3, ANCA), infection with Staph aureus, environmental triggers (silica)
MICROSCOPIC POLYANGIITIS
- p-ANCA triggering neutrophil degranulation and endothelial injury
- anti-MPO antibodies can cause necrotizing and crescentic glomerulonephritis
POLYARTERITIS NODOSA
- Only medium sized vessels
- Risk factors - HBV, 40-60 yrs, hairy cell leukaemia, blood transfusion pre-HBV screening, HCV, male
TAKAYASU'S ARTERITIS
- Symptoms due to claudication and stroke
- Diminished or absent pulses and hypertension is common
- Risk factors - genetic predisposition, female, <40, Asian
GIANT CELL ARTERITIS / TEMPORAL ARTERITIS
- Unknown
- Increasing age, genetic and ethnic background, infection
- Associated with HLA-DR4 and HLA-DRB1
128
Summarise the epidemiology of vasculitides.
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome)
- Granulomatosis with polyanigiitis
- Microscopic polyangitis
- Poarteritis nodosa
- Takayasu arteritis
- Giant cell arteritis (GCA)
EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CSS) - 10.7-14 per million adults. No gender difference.
GRANULOMATOSIS WITH POLYANGIITIS (WG) - Descriptive epidemiological studies carried out primarily in European countries estimate a prevalence of WG ranging from 24 to 157 per million and annual incidence rates from 3 to 14 per million
MICROSCOPIC POLYANGIITIS
Annual incidence of 3.6 cases per million persons. The prevalence is one to three cases per 100,000 population.
POLYARTERITIS NODOSA
Rare disease, with an incidence of about 3-4.5 cases per 100,000 population annually
TAKAYASU'S ARTERITIS
- Women
- <40 yrs
GIANT CELL ARTERITIS
- 18 in 100,000
- M:F = 2-4:1
- 65-70 yrs
129
Recognise the presenting symptoms of vasculitides.
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome)
- Granulomatosis with polyanigiitis
- Microscopic polyangitis
- Poarteritis nodosa
- Takayasu arteritis
- Giant cell arteritis (GCA)
Features of All Disease:
- General - fever, night sweats, malaise, weight loss
- Skin - rash (vasculitic, purpuric, maculopapular, livedo reticularis)
- Joint - arthralgia, arthritis
- GI - abdominal pain, haemorrhage from mucosal ulceration, diarrhoea
- Kidney - glomerulonephritis, renal failure
- Lung - dyspnoea, cough, chest pain, haemoptysis, lung haemorrhage
- CVS - pericarditis, coronary arteritis, myocarditis, heart failure, arrhythmias
- CNS - mononeuritis multiplex, infarctions, meningeal involvement
- Eyes - retinal haemorrhage, cotton wool spots
EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CSS)
- Asthma
- Allergic rhinitis
- Sinusitis
- Eosinophilia
GRANULOMATOSIS WITH POLYANGIITIS (WG)
- Granulomatous vasculitis of upper and lower respiratory tract
- Nasal discharge
- Ulceration
- Deformity
- Haemoptysis
- Sinusitis
- Corneal thinning
- Glomerulonephritis
MICROSCOPIC POLYANGIITIS
- Non-specific with multiple organs affected
- Glomerulonephritis with no glomerular Ig deposits
POLYARTERITIS NODOSA
- Microaneurysms
- Thrombosis
- Infarctions (causing GI perforation)
- Hypertension
- Testicular pain
TAKAYASU'S ARTERITIS
- Constitutional upset
- Head or neck pain
- Tenderness over affected arteries - aorta and major branches
- Dizziness
- Fainting
- Low peripheral pulses
- Hypertension
GIANT CELL ARTERITIS
- Subacute onset, usually over a few weeks
- Scalp and temporal tenderness - pain on combing hair
- Jaw and tongue claudication
- Blurred vision
- Sudden blindness in one eye - amaurosis fugax
- Malaise, low-grade fever, lethargy, weight loss, depression
- Early morning pain and stiffness of the muscles of the shoulder and pelvic girdle - 40-60% of cases associated with PMR
130
Recognise the signs of vasculitides on physical examination.
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome)
- Granulomatosis with polyanigiitis
- Microscopic polyangitis
- Poarteritis nodosa
- Takayasu arteritis
- Giant cell arteritis (GCA)
Features of All Disease:
- General - fever, night sweats, malaise, weight loss
- Skin - rash (vasculitic, purpuric, maculopapular, livedo reticularis)
- Joint - arthralgia, arthritis
- GI - abdominal pain, haemorrhage from mucosal ulceration, diarrhoea
- Kidney - glomerulonephritis, renal failure
- Lung - dyspnoea, cough, chest pain, haemoptysis, lung haemorrhage
- CVS - pericarditis, coronary arteritis, myocarditis, heart failure, arrhythmias
- CNS - mononeuritis multiplex, infarctions, meningeal involvement
- Eyes - retinal haemorrhage, cotton wool spots
EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CSS)
- Asthma
- Allergic rhinitis
- Sinusitis
- Eosinophilia
GRANULOMATOSIS WITH POLYANGIITIS (WG)
- Granulomatous vasculitis of upper and lower respiratory tract
- Nasal discharge
- Ulceration
- Deformity
- Haemoptysis
- Sinusitis
- Corneal thinning
- Glomerulonephritis
MICROSCOPIC POLYANGIITIS
- Non-specific with multiple organs affected
- Glomerulonephritis with no glomerular Ig deposits
POLYARTERITIS NODOSA
- Microaneurysms
- Thrombosis
- Infarctions (causing GI perforation)
- Hypertension
- Testicular pain
TAKAYASU'S ARTERITIS
- Constitutional upset
- Head or neck pain
- Tenderness over affected arteries - aorta and major branches
- Dizziness
- Fainting
- Low peripheral pulses
- Hypertension
GIANT CELL ARTERITIS
- Swelling and erythema overlying the temporal artery
- Scalp and temporal tenderness
- Thickened non-pulsatile temporal artery
- Low visual acuity
131
Identify appropriate investigations for vasculitides and interpret the results.
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome)
- Granulomatosis with polyanigiitis
- Microscopic polyangitis
- Poarteritis nodosa
- Takayasu arteritis
- Giant cell arteritis (GCA)
EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CSS)
- FBC with differential - eosinophilia
- ANCA - positive
- CRP - high
- ESR- high
- Urea & creatinine - normal or high
- Urinalysis - normal or abnormal (may show glomerulonephritis - haematuria, proteinuria, RBC casts)
- PFT - reversible airway obstruction
- CXR- interstitial infiltrates or nodules
- Echo - left ventricular regional wall motion abnormlaities, intracardiac thrombus, pericardial effusion
GRANULOMATOSIS WITH POLYANGIITIS (WG)
- Urinalysis and microscopy - haematuria, proteinuria, dysmorphic RBC, RBC casts
- CT chest - lung nodules, infiltrates
- ANCA - cANCA, pANCA
- FBC and differential - anaemia
- Creatinine - high
- ESR - high
MICROSCOPIC POLYANGIITIS
- ESR - high
- CRP - high
- FBC - anaemia
- Creatinine - high
- pANCA with MPO specificity - positive
- Urinalysis - haematuria, proteinuria, RBC casts
- If neuropathy, EMG may reveal sensorimotor peripheral neuropathy
POLYARTERITIS NODOSA
- CRP - high
- ESR - high
- FBC - normocytic anaemia, high WBC, high platelet
- Complement - low
- Creatinine - high or normal
- Midstream urine - proteinuria (mild) or normal
- LFTs - high liver enzymes
- HBV - HbsAg positive and/or HbeAg positive
- HCV - positive anti-HCV antibodies
- Cryoglobulins - none
- Blood culture - no growth
- CK - normal or mildly elevated
- ANCA - negative
- ANA - negative
- Anti-dsDNA - negative
- RF - negative
- Anti-CCP - negative
- Lupus anticoagulant - negative
- IgG antiphospholipid antibodies - negative
- B2 glycoprotein - negative
- Fibrinogen - normal or high
- Conventional digital subtraction angiography - microaneurysms, vessel actasia, focal occlusive lesions in medium sized vessels
- Echo - normal
TAKAYASU'S ARTERITIS
- ESR- >50mm/hr with active disease
- CRP - high
- CTA - segmental narrowing or occlusion, dilation of affected vessels, aortic aneurysms seen, thickening of vessel walls seen
- MRA - segmental narrowing, occlusion or dilation of involved arteries, vessel wall inflammation
GIANT CELL ARTERITIS
- ESR - >50mm/hr
- CRP - high
- FBC - normochromic, normocytic anaemia, normal WBC, high platelet, mild leukocytosis
- LFTs - AST, ALT, ALP mildly high
- Temporal artery USS - wall thickening (halo sign), stenosis or occlusion
- Temporal artery biopsy - granulomatous inflammation in 50% of cases, multinucleated giant cells present, inflammatory infiltrate focal and segmental
- Aortic arch angiography - stenosis or occlusion of SC, axillary or proximal brachial arteries
132
Generate a management plan for Giant Cell Arteritis.
SUSPECTED
- No visual neurological symptoms - Prednisolone 4 weeks, taper over 6-12 months
- Visual or neurological symptoms - Methylprednisolone pulse therapy 1g IV for 3 days
CONFIRMED
- Prednisolone - 4 weeks, taper over 6-12 months
- Aspirin - 75mg OD oral
- Osteoporosis prevention - Calcium Carbonate + Ergocalciferol + Alendronic Acid / Risedronate Sodium
- Tocilizumab or Methotrexate
133
Identify the possible complications for giant cell arteritis and its management.
- Carotid artery or aortic aneurysms
- Thrombosis
- Recanalization or embolism to opthalamic artery
- Visual disturbances
- Amaurosis fugax - cannot see out of one or both eyes due to lack of blood flow to eyes
- Sudden monoocular blindness
134
Summarise the prognosis for patients with giant cell arteritis.
For most condition lasts for 2 years before complete remission.
