Rheum Flashcards
Systemic sclerosis
Clinical
Progressive tissue fibrosis
Vascular dysfunction
Systemic: fatigue, weakness
Skin: telangiectasia, sclerodactyly, digital ulcers, calcinosis cutis
Extremities: arthralgias, contractures, myalgias
Gastrointestinal: esophageal dysmotility, dysphagia, dyspepsia
Vascular: Raynaud phenomenon
Systemic sclerosis
Complications
Lung: interstitial lung disease, pulmonary arterial HTN
Kidney: HTN, scleroderma renal crisis (oliguria, thrombocytopenia, MAHA)
Heart: myocardial fibrosis, pericarditis, pericardial effusion
Systemic sclerosis
Diagnosis
Antinuclear antibody
Anti-topoisomerase I (anti-Scl-70) antibody
Anticentromere antibody
Systemic sclerosis - Limited cutaneous
Scleroderma on trunk & UE
Prominent internal organ involvement
Scleroderma renal crisis Myocardial ischemia & fibrosis Interstitial lung disease
Anti-Scl-70 (topoisomerase-1) antibodies
Anti-RNA polymerase III antibodies
Worse prognosis
Systemic sclerosis - Diffuse cutaneous
Scleroderma on head & distal UE Prominent vascular manifestations Raynaud phenomenon Cutaneous telangiectasia Pulmonary arterial hypertension
CREST syndrome Anticentromere antibodies Better prognosis
Sarcoid
Young adults, African Americans
Constitutional symptoms Cough, dyspnea & chest pain Extrapulmonary findings: Skin lesions Anterior/posterior uveitis Löfgren syndrome Parotid gland swelling
Sarcoid
Findings
Imaging Bilateral hilar adenopathy Pulmonary reticular infiltrates Laboratory Hypercalcemia/hypercalciuria Elevated serum ACE level Biopsy: noncaseating granulomas
Sjogren
Diagnosis
Objective signs of decreased lacrimation (eg, Schirmer test)
Positive anti-Ro (SSA) &/or anti-La (SSB)
Salivary gland biopsy with focal lymphocytic sialoadenitis
Classification: primary if no associated CTD, secondary if comorbid CTD (eg, SLE, RA, scleroderma)
Sjogren
Extraglandular
features
Raynaud phenomenon Cutaneous vasculitis Arthralgia/arthritis Interstitial lung disease Non-Hodgkin lymphoma
Sjogren
Exocrine features
Keratoconjunctivitis sicca
Dry mouth, salivary hypertrophy
Xerosis
Polymyalgia rheumatica
Clinical features
Almost exclusively in patients age ≥50
Rapid-onset pain & stiffness in shoulders & hips ± neck involvement
Fatigue, weight loss, low-grade fever
~10% associated with GCA (eg, headache, jaw claudication, visual symptoms)
- Strength remains normal
- Morning stiffness, gelling (stiffness with inactivity), aching pain, and limited range of motion of the shoulders, hips, and/or neck
- Patients also often have systemic symptoms (eg, fatigue, fever, weight loss).
Polymyalgia rheumatica
Diagnosis
Elevated acute-phase markers (eg, ESR, CRP)
Temporal artery biopsy if symptoms of GCA
Polymyalgia rheumatica
Treatmennt
Very rapid response to oral glucocorticoids
Polymyositis
Clinical features
Proximal muscle weakness (eg, increasing difficulty climbing up stairs)
Pain mild/absent
Polymyositis
Diagnosis
Elevated muscle enzymes (eg, creatine kinase, aldolase, AST)
Autoantibodies (ANA, anti-Jo-1)
Biopsy: Endomysial infiltrate, patchy necrosis