Rheumatology/locomotor Flashcards
What body parts are affected in:
A. Limited cutaneous sclerosis
B. Diffuse cutaneous sclerosis
C. Scleroderma
A. Limited: Face + distal limbs + CREST syndrome
B.Diffuse: trunk + proximal limbs + Interstitial lung disease/ pulmonary arterial hypertension
C. Scleroderma → only the skin is affected
Antibodies associated with:
A. Limited cutaneous sclerosis
B. Diffuse cutaneous sclerosis
- Limited: anti-centromere
- Diffused: anti-scl-70
What’s CREST syndrome?
CREST → a subtype of limited cutaneous systemic sclerosis
C - calcinosis
R - Raynould’s
E - oEsophageal dysmotility
S - sclerodactyly
T - telangiectasia
Antibodies in systemic sclerosis
- ANA positive in 90%
- RF positive in 30%
- anti-scl-70 antibodies associated with diffuse cutaneous systemic sclerosis
- anti-centromere antibodies associated with limited cutaneous systemic sclerosis
X-ray changes in RA
L - loss/narrowing of joint space
O - juxta-articular osteoporosis
S - subluxation
S - soft tissue swelling
X-ray changes in OA
L - loss/narrowing of joint space
O - osteophytes
S - subchondral sclerosis
S - subchondral cysts
What problems are associated with Diffuse Cutaneous Systemic Sclerosis?
Features of CREST syndrome plus many internal organs causing:
- Cardiovascular problems, particularly hypertension and coronary artery disease
- Lung problems, particularly pulmonary hypertension and pulmonary fibrosis
- Kidney problems, particularly glomerulonephritis and scleroderma renal crisis
What’s meant by scleroderma?
Scleroderma →hardening of the skin.
Appearance of:
- shiny, tight skin without the normal folds in the skin
- most notable on the hands and face
What’s meant by sclerodactyly?
Sclerodactyly → the skin changes in the hands
- skin tightens around joints → it restricts the range of motion in the joint and reduces the function of the joints
- skin hardens and tightens further → the fat pads on the fingers are lost
- The skin can break and ulcerate
Compare OA and RA
Management of systemic sclerosis
Steroids and immunosuppressants are usually started with diffuse disease and complications such as pulmonary fibrosis
Non-medical management involves:
- Avoid smoking
- Gentle skin stretching to maintain the range of motion
- Regular emollients
- Avoiding cold triggers for Raynaud’s
- Physiotherapy to maintain healthy joints
- Occupational therapy for adaptations to daily living to cope with limitations
Medical management focuses on treating symptoms and complications:
- Nifedipine can be used to treat symptoms of Raynaud’s phenomenon
- Anti-acid medications (e.g. PPIs) and pro-motility medications (e.g. metoclopramide) for gastrointestinal symptoms
- Analgesia for joint pain
- Antibiotics for skin infections
- Antihypertensives can be used to treat hypertension (usually ACE inhibitors)
- Treatment of pulmonary artery hypertension
- Supportive management of pulmonary fibrosis
Antibodies associated with SLE
- Anti-nuclear antibodies (ANA) positive 85% of SLE cases (but not specific)
- Anti-double stranded DNA (anti-dsDNA) is specific to SLE; positive in 70% of patients with SLE
- Anti-Smith (highly specific to SLE)
- Anti-centromere antibodies (most associated with limited cutaneous systemic sclerosis)
- Anti-Ro and Anti-La (most associated with Sjogren’s syndrome)
- Anti-Scl-70 (most associated with systemic sclerosis)
- Anti-Jo-1 (most associated with dermatomyositis)
Possible complications of SLE
- Cardiovascular disease → chronic inflammation in the blood vessels → hypertension and coronary artery diseas
- Infection (part of the disease process and secondary to immunosuppressants
- Anaemia of chronic disease → affects the bone marrow causing a chronic normocytic anaemia
- leukopenia, neutropenia, thrombocytopenia
- Pericarditis
- Pleuritis
- Interstitial lung disease → caused by inflammation in the lung tissue → pulmonary fibrosis
- Lupus nephritis → inflammation in the kidney → end-stage renal failure
- Neuropsychiatric SLE → inflammation in the central nervous system (optic neuritis, transverse myelitis or psychosis
- Recurrent miscarriage
- Venous thromboembolism → due to antiphospholipid syndrome occurring secondary to SLE
Treatment of SLE
First-line treatments are:
- NSAIDs
- Steroids (prednisolone)
- Hydroxychloroquine (first line for mild SLE)
- Suncream and sun avoidance for the photosensitive the malar rash
Other commonly used immunosuppressants in resistant or more severe lupus:
- Methotrexate
- Mycophenolate mofetil
- Azathioprine
- Tacrolimus
- Leflunomide
- Ciclosporin
Biological therapies (considered for patients with severe disease or where patients have not responded to other treatments):
- Rituximab is a monoclonal antibody that targets the CD20 protein on the surface of B cells
- Belimumab is a monoclonal antibody that targets B-cell activating factor
Name (4) types of small vessel vasculitis
- Henoch-Schonlein purpura
- Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
- Microscopic polyangiitis
- Granulomatosis with polyangiitis (Wegener’s granulomatosis)
Name (3) types of medium vessel vasculitis
- Polyarteritis nodosa
- Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
- Kawasaki Disease
Name (2) types of large vessel vasculitis
- Giant cell arteritis
- Takayasu’s arteritis
What’s the most important marker for vasculitis?
Antineutrophil cytoplasmic antibodies (ANCA)
Types of ANCA blood tests associated with vasculitis
There are two type of ANCA blood tests: p-ANCA and c-ANCA.
- p-ANCA (PR3 antibodies): Microscopic polyangiitis and Churg-Strauss syndrome
- c-ANCA (MPO antibodies): Wegener’s granulomatosis
Management of vasculitis
Rheumatology referral - to diagnose + treat
Combination of steroids and immunosuppressants.
Steroids can be administered to target the affected area:
- Oral (i.e. prednisolone)
- Intravenous (i.e. hydrocortisone)
- Nasal sprays for nasal symptoms
- Inhaled for lung involves (e.g. Churg-Strauss syndrome)
Immunosuppressants that are used include:
- Cyclophosphamide
- Methotrexate
- Azathioprine
- Rituximab and other monoclonal antibodies
(4) classic features of Henoch-Schonlein Purpura
- purpura
- joint pain
- abdominal pain
- renal involvement
Management of Henoch Schonlein Purpura
- typically supportive → simple analgesia, rest and proper hydration
- abdominal pain usually settles within a few days
- patients without kidney involvement can expect to fully recover within 4-6 weeks
- a third of patients have a recurrence of the disease within 6 months
- 1% of patients will go on to develop end stage renal failure.
Another name for Churg-Strauss syndrome
Eosinophilic granulomatosis with polyangiitis
*small and medium vessel vasculitis
Characteristic features of Churg-Strauss syndrome
- lung and skin problems (but can affect other organs such as kidneys)
- often presents with severe asthma in late teenage years or adulthood
- a characteristic finding is elevated eosinophil levels on the full blood count
Characteristic features of microscopic polyangiitis
- renal failure
- it can also affect the lungs → shortness of breath and haemoptysis
Characteristic features of Wegener’s Granulomatosis
- affects the respiratory tract and kidneys
URTI affects:
- nose → nose bleeds (epistaxis) and crusty nasal secretions
- ears → hearing loss
- sinuses→ sinusitis
A classic sign in exams is the saddle-shaped nose due to a perforated nasal septum
LRTI:
- the lungs → a cough, wheeze and haemoptysis.
- A chest xray may show consolidation and it may be misdiagnosed as pneumonia
- kidneys → rapidly progressing glomerulonephritis
Features of Polyarteritis Nodosa
Polyarteritis nodosa (PAN) is a medium vessel vasculitis
- associated with hepatitis B but can also occur without a clear cause or with hepatitis C and HIV.
- affected vessels in: skin, gastrointestinal tract, kidneys and heart → renal impairment, strokes and myocardial infarction
(!) It is associated with a rash called livedo reticularis → mottled, purplish, lace like rash
Features of Kawasaki disease
Kawasaki disease is a medium vessel vasculitis. It affects young children, typically under 5 years of age. There is no clear cause.
Clinical features are:
- Persistent high fever > 5 days
- Erythematous rash
- Bilateral conjunctivitis
- Erythema and desquamation (skin peeling) of palms and soles
- “Strawberry tongue” (red tongue with prominent papillae)
A key complication is coronary artery aneurysms.
Treatment is with aspirin and IV immunoglobulins.
Features of Takayasu arteritis
Takayasu’s arteritis is a form of large vessel vasculitis.
It mainly affects:
- the aorta and it’s branches
- pulmonary arteries
These large vessels and their branches can swell and form aneurysms or become narrowed and blocked. This leads to it’s other name of “pulseless disease”.
Presentation: Japanese female below 40 y old; non-specific systemic symptoms (fever, malaise and muscle aches) or with more specific symptoms of arm claudication or syncope.
Diagnosis:CT or MRI angiography. Doppler ultrasound of the carotids can be useful in detecting carotid disease.
