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Currently filtering by tag: Crescentic glomerulonephritis

Twitter Poll (August 22, 2018)

ANSWER: D Anti-GBM disease is a rare small vessel vasculitis that affects glomerular and pulmonary capillaries. The majority of patients develop widespread glomerular crescent formation, presenting with features of rapidly progressive GN, and 40-60% will have concurrent alveolar hemorrhage

Digging Deeper, Literally

This biopsy came from a 79-year-old patient. He had presented with acute renal failure. His serum creatinine was up to 2.0 mg/dL, from a normal baseline. He also had proteinuria, which was quantified as approximately 1 g. His serum albumin was low at 2.5 g/dL. The patient was anemic, and hemoglobin was 9.1. The patient had been investigated with an extensive serologic work up. ANA was negative, C3 was within normal limits, and SPEP was negative for monoclonal proteins. However, ANCA serologies were positive. Based on that, the patient had been treated with high doses of steroids for a few...

Hidden Treasures

This biopsy came to us due to acute renal failure and proteinuria. The patient was a gentleman in his late 70s, with elevated creatinine up to 2.0 mg/dl, and proteinuria quantified at about 1 gram per 24 hours. His serum albumin was remarkably low, at 2.5 g/dL. He was also anemic, with hemoglobin at 9.1 g/dL. ANCA serologies were positive and the patient had been treated with high doses of steroids for a few weeks prior to the biopsy. SPEP was negative for monoclonal proteins. ANA serology was negative. Fortunately for us, the biopsy was a great sample of renal...

Crescentic Glomerulonephritis with IgA Deposits

This biopsy was taken from a 58-year-old male with no significant past medical history, who presents with gross hematuria, serum creatinine of 2.5 mg/dl and a urine protein/creatinine ratio of 1.4 g/g. The biopsy shows segmental rupture of the capillary loops associated with fibrinoid necrosis of the glomerular tuft and cellular crescent formation (Fig 1 – Jones stain). Of note, no mesangial or endocapillary proliferation is present. Immunofluorescence (Fig 2) shows mesangial granular staining for IgA (3+), C3 (1+), kappa (2+) and lambda (3+). Electron microscopy (not shown) shows scattered mesangial electron-dense deposits. The differential diagnosis in patients with necrotizing and...

IgA with Crescents, MEST-C

This biopsy is from a young adult patient with IgA nephropathy (the immunofluorescence image in Fig. 2 shows 2+ mesangial IgA deposits). While both glomeruli show endocapillary hypercellularity, the arrow indicates one glomerulus with a cellular crescent (Fig. 1). The recently updated Oxford classification of IgA nephropathy recommends that the frequency of crescents (cellular and/or fibrocellular) be added as the “C” in the MEST-Cscore (see reference). Although randomized clinical trials are still needed to determine optimal therapy, a C1 score (crescents in <25% of glomeruli) identifies patients at risk for poor renal outcome if not treated with immunosuppression, and a...

Anti GBM Disease

This biopsy from an 80-year-old male with acute kidney injury shows glomeruli with frequent rupture of the capillary loops and Bowman’s capsule, associated with fibrinoid necrosis of the glomerular tuft and cellular crescent formation (Fig 1). Of note, there are scattered multinucleated giant cells within the involved glomeruli. Immunofluorescence shows diffuse and global bright linear staining of the capillary loops for IgG (Fig 2), kappa and lambda. These findings are diagnostic of anti-glomerular basement membrane disease.  Multinucleated giant cells are significantly more common in anti-glomerular basement membrane disease when compared to ANCA-associated glomerulonephritis, and may serve as a marker of...

Endocarditis

Crescentic glomerulonephritis is most commonly an autoimmune-related glomerulonephritis (e.g. ANCA, anti-GBM disease, lupus nephritis). However, a recent case series (reference below) found that more than 50% of cases of endocarditis associated glomerulonephritis show a crescentic pattern of glomerulonephritis without endocapillary proliferation. Further, 28% of the patients with endocarditis-associated glomerulonephritis had positive serologic studies for ANCA. Therefore, it is important to maintain a high index of suspicion for infective endocarditis associated glomerulonephritis considering the potential adverse outcome if a patient with endocarditis was mistakenly treated for ANCA-associated glomerulonephritis with cytotoxic agents in lieu of antibiotics. Reference: Boils CL, Nasr SH, Walker...

Renal Medullary Angiitis

This 56-year-old female presented with acute kidney injury, hematuria, and mild proteinuria. All serologic studies were negative except for antineutrophil cytoplasmic antibodies (ANCA), which showed a perinuclear pattern of staining. A renal biopsy shows focal necrotizing and crescentic glomerulonephritis (Fig 1) with the absence of significant staining by immunofluorescence, consistent with ANCA-associated glomerulonephritis. Interestingly, the biopsy also shows significant neutrophil-rich medullary angiitis (Fig 2 and Fig 3). Medullary angiitis is characterized by the presence of interstitial hemorrhage, polymorphonuclear leukocyte infiltration, and karyorrhectic debris within the renal medulla. The morphology of medullary angiitis may be quite variable, mainly related to the...