Nephrology Question 28 Instructor

A patient presents with hemoptysis and acute renal failure. What would be included in the differential diagnosis?

This presentation suggests the presence of a pulmonary-renal syndrome, which is a combination of acute glomerulonephritis with alveolar hemorrhage.

The pulmonary-renal syndrome can be caused by a variety of conditions, including Goodpasture’s syndrome which is associated with autoantibodies to the glomerular and alveolar basement membranes, and various forms of primary systemic vasculitis associated with serum positivity for antineutrophil cytoplasmic antibodies (ANCA), such as microscopic polyangiitis, Wegener’s granulomatosis and, less commonly, Churg-Strauss syndrome. This syndrome can be seen less commonly in association with systemic lupus erythematosus, systemic sclerosis, antiphospholipid syndrome, drugs (including propylthiouracil and hydralazine), Behçet’s disease, Henoch–Schönlein purpura, IgA nephropathy and mixed cryoglobulinaemia.

The majority of cases of pulmonary-renal syndromes are associated with ANCAs. The antigen targets in ANCA-associated disease are proteinase-3 and myeloperoxidase. The antigen target in Goodpasture’s syndrome is the alpha-3 chain of type IV collagen.

For a more detailed, free full text review try:

Bench-to-bedside review: Pulmonary–renal syndromes – an update for the intensivist. Critical Care 2007, 11:213 (PubMed)

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