Rapidly progressive crescentic glomerulonephritis

Abstract

pulmonary edema. Hemodialysis was initiated and continued throughout her admission. Renal ultrasound showed normal kidney size with echogenicity consistent with parenchymal disease. Serologic tests were ordered and a renal biopsy was performed. The renal biopsy demonstrated involvement of essentially all glomeruli by necrosis, sclerosis, or both. Approximately 90% of glomeruli had cellular or fibrocellular crescents (Fig. 1A). There was focal disruption of Bowman’s capsule. Most glomeruli had prominent adjacent periglomerular inflammation, including occasional multinucleated giant cells. A few small foci of granulomatous inflammation were present but were centered on identifiable glomeruli (Fig. 1C). Arteries and arterioles had moderate sclerosis as well as focal necrotizing vasculitis (Fig. 1D). Immunofluorescence microscopy demonstrated intense linear staining of glomerular basement membranes CASE PRESENTATION (GBMs) for immunoglobulins (IgG) (Fig. 1B), kappa light A 77-year-old white woman was found unresponsive on the chains, and lambda light chains, and moderate granular staining floor of her home. Emergency medical technicians were called, for C3. There was no staining for IgA, IgM, or C1q. There was and she was transported to the University of North Carolina marked staining of crescents for fibrin. Electron microscopy Hospitals. She recovered consciousness and gave a history of revealed extensive disruption of glomerular basement memrecent anorexia, lower extremity weakness and paresthesias, branes and Bowman’s capsule, segmental fibrinoid necrosis, and difficulty walking. Her medical history was significant for and no immune-complex-type electron-dense deposits. rectal adenocarcinoma 9 years earlier that was successfully Serology demonstrated the presence of high-titer myelotreated with radiation, chemotherapy, and partial colectomy. peroxidase-specific antineutrophil cytoplasmic autoantibodies A recent colonoscopy showed no recurrence. She had been (MPO-ANCA) and high-titer anti-GBM antibodies. Testing for diagnosed with a myeloproliferative disorder with features of proteinase 3–specific ANCA (PR3-ANCA) and antinuclear essential thrombocytosis, and hydroxyurea (500 mg/day) had autoantibodies (ANA) was negative. been initiated. The patient received hemodialysis, plasmapheresis, and pulse Physical examination revealed an elderly, thin, lethargic methylprednisolone. Cyclophosphamide was not instituted bewoman who was afebrile and had a blood pressure of 140/60 cause of the poor prognosis for recovery of renal function and mm Hg, guaiac-negative stool, no rash, 1 pitting edema to the overall frail status of the patient. During the entire 3-week the knees, and bilateral foot drop. Computerized tomography admission, she produced less than 50 mL urine/day. Her lower (CT) identified a small subdural hematoma. Laboratory data extremity neuropathy improved and her lungs cleared. After included blood urea nitrogen (BUN), 158 mg/dL; serum creatiPermacath placement, she was discharged to a nursing home nine, 10.8 mg/dL (0.6 mg/dL 2 months prior to admission); on low-dose oral prednisone and scheduled to receive hemodipotassium, 6.3 mmol/L; glucose, 101 mg/dL; albumin, 2.3 g/dL; alysis. white blood cell count, 23.3 10/L (neutrophil count, 19.3 10/L); red blood cell count, 3.5 10/L; and platelet count, 799 10/L. Urinalysis revealed 2 protein, 4 blood, and nuDISCUSSION merous dysmorphic red blood cells. Chest radiographs showed Dr. J. Charles Jennette (Brinkhous Distinguished Professor and Chair of Pathology and Laboratory Medicine, and Professor of Medicine, The Medical School, The Nephrology Forum is funded in part by grants from Amgen, Incorporated; Merck & Co., Incorporated; Dialysis Clinic, IncorpoThe University of North Carolina at Chapel Hill, Chapel rated; and Bristol-Myers Squibb Company. Hill, North Carolina, USA): The patient had rapidly progressive glomerulonephritis clinically and crescentic glo