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A 41-year-old Venezuelan man with no past medical history was admitted last October for cough productive of blood-tinged sputum, fevers, weight loss of 25 lbs. during the previous three weeks. He was found to have acute renal failure, with a creatinine level of 3.59 mg/dL, active urinary sediment with many dysmorphic red blood cells (RBCs), no casts, proteinuria of about 1 g/day (spot). Chest X-ray and chest computed tomography (CT) showed b/l consolidation with cavitary lesions (see images). Renal ultrasound showed the isoechoic cortices, with the right kidney at 12.9 cm in length, left kidney at 12.7 cm in length, and no stone or hydronephrosis. A renal biopsy showed crescentic pauci-immune glomerulonephritis.
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Answer: B
The clinical history and laboratory results (active urinary sediment with many dysmorphic RBCs, no casts and non-nephrotic proteinuria), the radiological findings (cavitary lesions), and the crescentic pauci-immune glomerulonephritis on kidney biopsy confirmed the diagnosis of Wegener’s granulomatosis, or granulomatosis with polyangiitis. (In January 2011, the boards of directors of the American College of Rheumatology, the American Society of Nephrology, and the European League Against Rheumatism recommended that the name Wegener’s granulomatosis be changed to granulomatosis with polyangiitis.)
Anti-GBM and microscopic polyarteritis have similar symptoms, but the CT and X-ray do not reveal any cavitary lesions. However, fungal infection might create cavitary lesions in the lung, but it is not associated with crescentic pauci-immune glomerulonephritis.
Initial therapy with cyclophosphamide and glucocorticoids, which is preferred in the great majority of patients, induces remission in 85%-90% of patients, usually within two to six months. Two randomized trials have suggested that rituximab may be an effective alternative to cyclophosphamide for the initial treatment of patients who have newly diagnosed disease, but both studies are limited in the duration of follow-up. In this case, clinicians started with pulsed steroids, getting plasmapheresis and 100 mg oral cyclophosphamide. Pneumocystis carinii pneumonia prophylaxis during the initial treatment period also is suggested.
This case is presented by Miklos Z. Molnar, MD, PhD, and Kamyar Kalantar-Zadeh, MD, PhD, of Harbor-UCLA Medical Center in Torrance, Calif.
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