Sjögren's, scleroderma, myositis & Raynaud's flashcards
23 high-yield MRCP Part 1 cards from the Intellect Medicos Rheumatology course. Try to answer each question before you open it — then study them properly with spaced repetition, which brings each card back just before you would forget it.
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Which two antibodies are characteristic of primary Sjögren's syndrome?
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Anti-Ro (SSA) and anti-La (SSB).
Why? Anti-Ro is positive in about 70%, anti-La in about 30%; ANA and RF are also often positive.
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Which bedside test measures reduced tear production in Sjögren's syndrome?
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Schirmer's test.
Why? A filter-paper strip in the lower eyelid wets less than normal over 5 minutes. Salivary gland biopsy shows lymphocytic infiltration.
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Which malignancy is increased in Sjögren's syndrome?
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B-cell non-Hodgkin lymphoma (especially MALT lymphoma of the parotid).
Why? Chronic B-cell activation drives lymphoma; persistent parotid swelling should raise concern.
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Which type of renal tubular acidosis occurs in Sjögren's syndrome?
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Distal (type 1) renal tubular acidosis.
Why? Lymphocytic infiltration of the kidney impairs distal acid secretion, causing hypokalaemia and possible renal stones.
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Which drug stimulates saliva production in Sjögren's syndrome?
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Pilocarpine (a muscarinic agonist).
Why? Artificial tears and saliva are first-line for sicca symptoms; hydroxychloroquine helps arthralgia and fatigue.
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Limited cutaneous systemic sclerosis (CREST) is associated with ______ antibodies, and diffuse cutaneous disease with anti-Scl-70 (anti-topoisomerase I) antibodies.
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anti-centromere
Why? Limited disease affects distal skin and progresses slowly; diffuse disease involves proximal skin early with more lung and renal involvement.
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Limited cutaneous systemic sclerosis (CREST) is associated with anti-centromere antibodies, and diffuse cutaneous disease with ______ antibodies.
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anti-Scl-70 (anti-topoisomerase I)
Why? Limited disease affects distal skin and progresses slowly; diffuse disease involves proximal skin early with more lung and renal involvement.
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What is the main late organ complication of limited cutaneous systemic sclerosis?
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Pulmonary arterial hypertension.
Why? Interstitial lung disease and renal crisis are more typical of diffuse disease.
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Which antibody in systemic sclerosis is most associated with scleroderma renal crisis?
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Anti-RNA polymerase III.
Why? Renal crisis presents with accelerated hypertension and rising creatinine, usually in early diffuse disease.
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What is the treatment of scleroderma renal crisis?
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An ACE inhibitor (e.g. captopril), even if creatinine is rising.
Why? ACE inhibitors are life-saving here and transformed survival in renal crisis.
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Name two drug classes used for pulmonary arterial hypertension in systemic sclerosis.
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Endothelin receptor antagonists (e.g. bosentan) and PDE-5 inhibitors (e.g. sildenafil).
Why? Both reduce pulmonary vascular resistance. ILD in SSc is treated with mycophenolate or cyclophosphamide.
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Which antibody is diagnostic of mixed connective tissue disease?
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Anti-U1 RNP.
Why? MCTD overlaps SLE, systemic sclerosis and polymyositis features; Raynaud's and puffy hands are common.
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Which myositis antibody marks the antisynthetase syndrome (ILD, mechanic's hands, arthritis)?
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Anti-Jo-1.
Why? Anti-Jo-1 is an anti-tRNA-synthetase antibody, seen more often in polymyositis and linked to lung involvement.
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In dermatomyositis, anti-______ is the most specific antibody, while anti-TIF1-γ is strongly associated with underlying malignancy.
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Mi-2
Why? Antibody profile guides the intensity of the cancer search and predicts the clinical phenotype.
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In dermatomyositis, anti-Mi-2 is the most specific antibody, while anti-______ is strongly associated with underlying malignancy.
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TIF1-γ
Why? Antibody profile guides the intensity of the cancer search and predicts the clinical phenotype.
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What is the gold-standard investigation for dermatomyositis or polymyositis?
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Muscle biopsy.
Why? CK, EMG, MRI and myositis antibodies support the diagnosis, but biopsy confirms inflammatory myopathy.
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A 58-year-old woman has proximal muscle weakness, a violaceous rash on her upper eyelids and raised papules over her knuckles. CK is 4,000 U/L. Besides starting treatment, what must be done at diagnosis?
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Screen for malignancy (e.g. CT chest, abdomen and pelvis).
Why? Dermatomyositis is associated with cancers such as lung, ovarian, pancreatic and colorectal.
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In dermatomyositis, violaceous papules over the MCP and PIP joints are ______, and purple periorbital discoloration with oedema is the heliotrope rash.
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Gottron's papules
Why? Other skin signs: shawl and V sign, mechanic's hands and dilated nailfold capillaries.
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In dermatomyositis, violaceous papules over the MCP and PIP joints are Gottron's papules, and purple periorbital discoloration with oedema is the ______.
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heliotrope rash
Why? Other skin signs: shawl and V sign, mechanic's hands and dilated nailfold capillaries.
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What is the first-line treatment of polymyositis and dermatomyositis?
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Corticosteroids (with a steroid-sparing agent such as methotrexate, azathioprine or mycophenolate).
Why? IVIG is used for refractory disease.
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What is the first-line drug for Raynaud's phenomenon not controlled by keeping warm?
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Nifedipine (a dihydropyridine calcium-channel blocker).
Why? Second-line options are PDE-5 inhibitors (sildenafil) or topical nitrates; IV iloprost is used for digital ulcers or critical ischaemia.
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Which investigation best distinguishes secondary from primary Raynaud's?
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Nailfold capillaroscopy (abnormal, dilated capillaries in secondary disease).
Why? Secondary Raynaud's suggests a connective tissue disease such as systemic sclerosis; check ANA and other autoantibodies too.
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Which commonly prescribed cardiovascular drug class can cause or worsen Raynaud's phenomenon?
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Beta-blockers.
Why? Unopposed alpha-mediated vasoconstriction worsens peripheral vasospasm; ergotamine is another drug cause.