Rheumatology Flashcards
What is ankylosing spondylitis associated with? What are the other seronegative spondyloarthropathies?
Ankylosing spondylitis is a HLA-B27 associated spondyloarthropathy
No production of Rheumatoid Factor + all associated with HLA-B27**
PEAR HEADS
Psoriatic arthritis
Enteropathic arthritis
Ankylosing spondylitis
Reactive arthritis
HLA B27 allele
Enthesitis
Axial, asymmetrical, oligoarthritis
Dactilytis
Seronegative
Who does ankylosing spondylitis present in?
It typically presents in males (sex ratio 3:1) aged 20-30 years old.
How does ankylosing spondylitis present?
- typically a young man who presents with lower back pain and stiffness of insidious onset
- stiffness is usually worse in the morning and improves with exercise
- the patient may experience pain at night which improves on getting up
?anterior chest pain (costochondritis)
?SOB (pulm. fibrosis)
?Eye pain
Osteoporosis (60%)
What would you find on clinical examination in ankylosing spondylitis?
- reduced lateral flexion
- reduced forward flexion - Schober’s test - a line is drawn 10 cm above and 5 cm below the back dimples (dimples of Venus). The distance between the two lines should increase by more than 5 cm when the patient bends as far forward as possible
- reduced chest expansion
What are the other features related to ankylosing spondylitis?
- Apical fibrosis
- Anterior uveitis
- Aortic regurgitation
- Achilles tendonitis
- AV node block
- Amyloidosis
- and cauda equina syndrome
- peripheral arthritis (25%, more common if female)
What investigations should be done in ankylosing spondylitis? What would be expect?
- Inflammatory markers (ESR, CRP) are typically raised although normal levels do not exclude ankylosing spondylitis.
- HLA-B27 is of little use in making the diagnosis as it is positive in: 90% of patients with ankylosing spondylitis + 10% of normal patients
- Plain x-ray of the sacroiliac joints is the most useful investigation in establishing the diagnosis. Radiographs may be normal early in disease
- If the x-ray is negative for sacroiliac joint involvement in ankylosing spondylitis but suspicion for AS remains high, the next step in the evaluation should be obtaining an MRI. Signs of early inflammation involving sacroiliac joints (bone marrow oedema) confirm the diagnosis of AS and prompt further treatment.
- Spirometry may show a restrictive defect due to a combination of pulmonary fibrosis, kyphosis and ankylosis of the costovertebral joints.
What would we see in an X ray for ankylosing spondylitis?
sacroiliitis: subchondral erosions, sclerosis
squaring of lumbar vertebrae
‘bamboo spine’ (late & uncommon)
syndesmophytes: due to ossification of outer fibers of annulus fibrosus
chest x-ray: apical fibrosis
- Syndesmophytes
- Squaring of vertebral bodies
Fusion of bilateral sacroiliac joints
Dagger sign
Single line of radiodense line related to ossification of supraspinous and infraspinous ligaments
Bamboo spine
Fusion of sacroiliac joint
How should ankylosing spondylitis be managed?
- NSAIDs first line
- physiotherapy
- encourage regular exercise such as swimming
- Osteoporosis screen every 2 yrs
- Flare episodes — consider a flare management plan tailored to the person’s needs, preferences, and circumstances including access to car
- the disease-modifying drugs which are used to treat rheumatoid arthritis (such as sulphasalazine) are only really useful if there is peripheral joint involvement
- the 2010 EULAR guidelines suggest: ‘Anti-TNF therapy should be given to patients with persistently high disease activity despite conventional treatments’ (greater risk of skin cancer)
What are the ididopathic inflammatory myopathies?
Polymyositis and dermatomyositis (striated muscle inflammation)
What are the features of the myopathies? What is it caused by? What is it associated with? Who is affected by it?
- proximal muscle weakness +/- tenderness
- Raynaud’s
- respiratory muscle weakness
- interstitial lung disease: e.g. fibrosing alveolitis or organising pneumonia
- dysphagia, dysphonia
- inflammatory disorder causing symmetrical, proximal muscle weakness
- thought to be a T-cell mediated cytotoxic process directed against muscle fibres
- may be idiopathic or associated with connective tissue disorders
- associated with malignancy
- typically affects middle-aged, female:male 3:1
What is the difference between polymyositis and dermatomyositis?
- Dermatomyositis is a variant of the disease where skin manifestations are prominent, for example a purple (heliotrope) rash on the cheeks and eyelids
- polymyositis is a variant of the disease where skin manifestations are not prominent
What skin features are seen in dermatomyositis?
- photosensitive
- macular rash over back and shoulder
- heliotrope rash in the periorbital region
- Gottron’s papules - roughened red papules over extensor surfaces of fingers
- ‘mechanic’s hands’: extremely dry and scaly hands with linear ‘cracks’ on the palmar and lateral aspects of the fingers
- nail fold capillary dilatation
Heliotrope rash
Gottron’s papules
What investigations should be done? What would be expected?
- elevated creatine kinase
- other muscle enzymes (lactate dehydrogenase (LDH), aldolase, AST and ALT) are also elevated in 85-95% of patients
- EMG
- muscle biopsy - definitive
- anti-synthetase antibodies
- anti-Jo-1 antibodies are seen in pattern of disease associated with lung involvement, Raynaud’s and fever
- anti-Mi2
- antibodies to signal recognition particle (SRP)
- Malignancy screen
How should polymyositis and dermatomyositis be managed?
- Immunosuppressive and immunomodulatory therapies are frequently used but the optimal therapeutic regimen remains unclear.
- Steroids are the most important drugs. In mild disease, topical steroids may suffice. In more severe disease, high doses of systemic steroids are used and tapered off.
- Sun-blocking agents should be used.
- Encourage physical activity within reason to maintain muscular strength. This may involve consultation with a physiotherapist and occupational therapist.
- Evaluation of swallowing may be required and a speech and language therapist may help with difficulties of swallowing.
- Monitor creatine kinase and clinical response but treatment can improve the former without benefiting the latter.
What is systemic sclerosis?
Systemic sclerosis is a condition of unknown aetiology characterised by hardened, sclerotic skin and other connective tissues.
What are the patterns of systemic sclerosis?
- Limited Cutaneous systemic sclerosis
- Diffuse Cutaneous systemic sclerosis
- Scleroderma
What would the first sign of limited cutaneous systemic sclerosis? What is affected? What antibodies are found?
- first sign: Reynauds phenomenon
- scleroderma affects face and distal limbs predominately
- associated with anti-centromere antibodies
What is CREST syndrome?
Subtype of limited cutaneous systemic sclerosis
Calcinosis
Raynauds phenomenon
oEsophageal dysmotility
Sclerodactyly
Telangiectasia
Telangiectasia - CREST syndrome
What is affected in difuse cutaneous systemic sclerosis? What antibodies are associated with it? What are the complications associated with it? What is the prognosis?
- Scleroderma affects trunk and proximal limbs predominately
- Scl-70 antibodies
- Most common cause of death - respiratory involvement (80%): ILD + PAH
- Other complications: renal disease + hypertension
- Poor prognosis
Sclerodactyly
What is scleroderma? How does it manifest?
Scleroderma (without internal organ involvement)
tightening and fibrosis of skin
may be manifest as plaques (morphoea) or linear
What other antibodies are positive in systemic sclerosis?
ANA positive in 90%
RF positive in 30%
How is systemic sclerosis diagnosed?
The diagnosis of systemic sclerosis is confirmed when key features are present.
- Sclerodactyly
- Abnormal nail folds capillaries on capillaroscopy/dermatoscopy
- Specific autoantibodies (especially anti-Scl70 or anti-centromere antibody)
- Internal organ fibrosis and vascular damage
- Pulmonary function tests
- Transthoracic echocardiogram
- ECG
How is systemic sclerosis managed?
- Stop smoking
- For fatigue, weakness –> Hydroxychloroquine + gentle exercise + simple analgesia
- Antifibrotic therapies - topicals (tacrolimus, warm wax, corticosteroids), physiotherapies, systemic therapies (mycophenolate mofetil. methotrexate, cyclophosphamide…)
What is psoriatic arthropathy?
Psoriatic arthropathy is an inflammatory arthritis associated with psoriasis and is classed as one of the seronegative spondyloarthropathies.
What are the patterns of psoriatic arthritis?
- Symmetric polyarthritis (similar to RA) - most common type
- Asymmetrical oligoarthritis - typically affects hands and feet
- Sacroiliitis
- DIP joint disease
- Arthritis mutilans (telescoping fingers)
Arthritis mutilans
What are the extrarticular signs of psoriatic arthritis?
psoriatic skin lesions
periarticular disease - tenosynovitis and soft tissue inflammation resulting in:
* enthesitis: inflammation at the site of tendon and ligament insertion e.g. Achilles tendonitis, plantar fascitis
* tenosynovitis: typically of the flexor tendons of the hands
* dactylitis: diffuse swelling of a finger or toe
nail changes
* pitting
* onycholysis
Pencil in cup appearance - Psoriatic arthritis
How should psoriatic arthritis be managed?
Should be managed by a rheumatologist
Treatment is similar to that of rheumatoid arthritis (RA). However, the following differences are noted:
* mild peripheral arthritis/mild axial disease may be treated with ‘just’ an NSAID, rather than all patients being on disease-modifying therapy as with RA
* use of monoclonal antibodies such as ustekinumab (targets both IL-12 and IL-23) and secukinumab (targets IL-17)
* has a better prognosis than RA
What is osteomyelitis and how is it classified?
Osteomyelitis describes an infection of the bone. It may be subclassified into:
* haematogenous osteomyelitis
* non-haematogenous osteomyelitis
What causes haematogenous osteomyelitis? How many microbes cause it? Who is it seen in commonly? What bones are affected? What are the RFs?
- results from bacteraemia
- is usually monomicrobial
- most common form in children
- vertebral osteomyelitis is the most common form of haematogenous osteomyelitis in adults
- risk factors include: sickle cell anaemia, intravenous drug user, immunosuppression due to either medication or HIV, infective endoc
What causes non-haematogenous osteomyelitis? How many microbes cause it? Who is it seen in commonly? What bones are affected? What are the RFs?
- results from the contiguous spread of infection from adjacent soft tissues to the bone or from direct injury/trauma to bone
- is often polymicrobial
- most common form in adults
- risk factors include: diabetic foot ulcers/pressure sores, diabetes mellitus, peripheral arterial disease
What organism most commonly causes osteomyelitis?
Staph. aureus is the most common cause except in patients with sickle-cell anaemia where Salmonella species predominate
How would you manage osteomyelitis?
Investigations
* MRI is the imaging modality of choice, with a sensitivity of 90-100%
Management
* flucloxacillin for 6 weeks
* clindamycin if penicillin-allergic
What organism most commonly causes septic arthritis?
Most common organism overall is Staphylococcus aureus
What organism most commonly causes septic arthritis in young adults?
In young adults who are sexually active, Neisseria gonorrhoeae is the most common organism (disseminated gonococcal infection)
How does septic arthritis present?
- acute, swollen joint (most commonly knee)
- restricted movement in 80% of patients
- examination findings: warm to touch/fluctuant
- fever: present in the majority of patients
How would you manage a septic arthritis?
Investigations
* synovial fluid sampling is obligatory
* this should be done prior to the administration of antibiotics if necessary
* may need to be done under radiographic guidance
* blood cultures: the most common cause of septic arthritis is hematogenous spread
* joint imaging
Management
* intravenous antibiotics which cover Gram-positive cocci are indicated. The BNF currently recommends flucloxacillin or clindamycin if penicillin allergic
* antibiotic treatment is normally be given for several weeks (BNF states 4-6 weeks)
* patients are typically switched to oral antibiotics after 2 weeks
* needle aspiration should be used to decompress the joint
* arthroscopic lavage may be required
Sarcoidosis - bilateral hilar lymphadenopathy + interstitial infiltrates
What is sarcoidosis characterized by?
non-caseating granulomas.
Who is sarcoidosis seen in?
young adults and in people of African descent
How to patients with sarcoidosis present?
- acute: erythema nodosum, bilateral hilar lymphadenopathy, swinging fever, polyarthralgia
- insidious: dyspnoea, non-productive cough, malaise, weight loss
- skin: lupus pernio
- hypercalcaemia: macrophages inside the granulomas cause an increased conversion of vitamin D to its active form (1,25-dihydroxycholecalciferol)
What factors are associated with a poor prognosis in sarcoidosis?
- insidious onset, symptoms > 6 months
- absence of erythema nodosum
- extrapulmonary manifestations: e.g. lupus pernio, splenomegaly
- CXR: stage III-IV features
- black people
How do we investigate sarcoidosis?
- X ray
- ACE Levels (sensitivity 60% and specificity 70%)
- Spirometry - restrictive defect
- Tissue biopsy - non caseating granulomas
- Gallium-67 scan - not routinely used
- Bloods - calcium high in 10%
