Differentials
Embolic disease
SIGNS / SYMPTOMS
Often affects only 1 limb but can affect more. Recent chest pain suggesting a myocardial infarction.
INVESTIGATIONS
In embolic disease, an echocardiogram may show a valve lesion or thrombus; ECG and troponin may show evidence of myocardial ischemia.
Echocardiogram, ECG, and troponin blood tests show no dysrhythmia and no evidence of recent myocardial infarction in Buerger disease.
Duplex imaging may reveal the embolized thrombus.
Hypercoagulable state
SIGNS / SYMPTOMS
Multiple limbs affected. Known history or family history of thrombophilia disease.
INVESTIGATIONS
Coagulation profile: PT, PTT, and INR may be elevated.
Thrombophilia screen: may be positive for protein C, protein S, or antithrombin III deficiencies.
Raynaud phenomenon (RP)
SIGNS / SYMPTOMS
Painful extremities associated with cold weather exposure and a lack of skin loss.
Onset of symptoms with limb immersion in cold water.
INVESTIGATIONS
Antinuclear antibody, CBC, erythrocyte sedimentation rate, metabolic panel, and urinalysis may be normal in primary RP and abnormal in secondary RP.
Atherosclerosis
SIGNS / SYMPTOMS
Claudication history. Absent femoral pulses.
Associated with hypercholesterolemia, hypertension, and diabetes.
INVESTIGATIONS
Atherosclerotic plaques seen on radiologic imaging of arteries.
Rheumatoid vasculitis
SIGNS / SYMPTOMS
Usually known longstanding rheumatoid arthritis.
Active symmetric arthritis lasting >6 weeks, most commonly affecting the metacarpophalangeal (MCP), proximal interphalangeal (PIP), and metatarsalphalangeal (MTP) joints.
Skin rash is the most common vasculitic manifestation.
INVESTIGATIONS
Rheumatoid factor positive in up to 70% of cases.
Anti-cyclic citrullinated peptide (anti-CCP) positive in 70-80% of cases.
Joint x-rays reveal erosions.
Systemic lupus erythematosus
SIGNS / SYMPTOMS
Patients may have skin manifestations including malar, photosensitive, or discoid skin rash, mouth or nose ulcers, or diffuse patchy alopecia.
Other features include constitutional symptoms (e.g., fever, fatigue, weight loss), lymphadenopathy, musculoskeletal symptoms, Raynaud phenomenon, and symptoms of CNS, gastrointestinal cardiopulmonary, or hematological involvement.
INVESTIGATIONS
Antinuclear antibodies, dsDNA, and Smith antigen are positive.
Granulomatosis with polyangiitis (formerly known as Wegener granulomatosis)
SIGNS / SYMPTOMS
Classic triad consists of upper and lower respiratory tract involvement and pauci-immune glomerulonephritis, producing renal symptoms. Involvement of cutaneous, ocular, musculoskeletal, and peripheral nervous system tissue is also common.
Constitutional symptoms include fatigue, malaise, fever, night sweats, anorexia, and weight loss.
INVESTIGATIONS
Positive cANCA (cytoplasmic pattern on immunofluorescence testing) combined with positive proteinase 3 antibody testing by enzyme immunoassay (EIA).
Positive pANCA (perinuclear pattern on immunofluorescence testing) combined with positive myeloperoxidase antibody testing by EIA.
CREST syndrome
SIGNS / SYMPTOMS
Acronym for calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.
Disease typically limited to distal upper and lower extremities; may involve face and neck.
Esophageal dysmotility produces dysphagia and GERD. Sclerodactyly produces bright shiny skin of hands and feet.
Other features include dilated nailbed capillaries, symmetrical swelling of the fingers with reduced range of motion, contractures affecting joints in the hand, claw hand deformities, digital ulcers, and carpal tunnel syndrome.
INVESTIGATIONS
Anticentromere antibody may be positive and is strongly suggestive of the disease.
Serum antinuclear antibody may be positive but is not specific.
Serum extractable nuclear antigens may be positive.
CBC with peripheral fragmentation of red blood cells is associated with systemic disease.
Serum creatinine elevated in presence of renal crisis.
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