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Cause | Typical clinical presentation | Serologic and/or radiographic findings |
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SLE | Female, mean age of 27, often concurrent renal involvement, and other signs of connective tissue disease | ANA, anti-Smith and anti-dsDNA antibodies, hypocomplementemia (C3, C4, or CH50), and granular immune complex deposition on kidney biopsy by IF |
Granulomatosis with polyangiitis | Male, mean age of 50, and involvement of upper and lower respiratory tract and kidneys | c-ANCA, anti-PR3 antibody, nodules and cavities on chest radiograph and/or CT scan, and negative or pauci-IC deposition kidney biopsy by IF |
Microscopic polyangiitis | Associated focal segmental necrotizing glomerulonephritis with rare pulmonary involvement | p-ANCA, anti-MPO antibody, and negative kidney biopsy by IF |
Goodpasture syndrome | Male, age in 20 s, smoker, and pulmonary and renal involvement | IgG anti-GBM antibody and C3, and linear pattern on kidney biopsy by IF |
Antiphospholipid syndrome | Female, with the history of thrombotic events and miscarriage, may be in the setting of SLE or another systemic autoimmune disease | Anticardiolipin antibodies, anti-beta2 GPI antibodies, and/or lupus anticoagulant |
Mitral stenosis or regurgitation | Intermittent chest pain, palpitations, and peripheral edema | Elevated BNP or N-terminal pro-BNP, Kerley B lines on chest radiograph and/or CT scan, enlarged left atrium on echocardiography; may be unilateral |
Drug-related complication | History of exposure to following: abciximab, amiodarone, anticoagulants, carbimazole, crack cocaine, leflunomide, nitrofurantoin, penicillamine, propylthiouracil, sirolimus, TNF-α antagonist, and trimellitic anhydride | Clinical history and/or positive drug screening for cocaine abuse |
Isolated pauci-immune pulmonary capillaritis | Isolated pulmonary involvement | No significant serologic or radiographic findings |
Idiopathic pulmonary hemosiderosis | Children and young adults age < 30 years, chronic anemia, pulmonary fibrosis, and celiac disease (Lane-Hamilton syndrome) | Anti-tTG or antiendomysial antibodies (celiac disease) |
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