In anti-GBM antibody-mediated glomerulonephritis, variable granular or linear C3 staining is present, and the latter pattern may be continuous or discontinuous

In anti-GBM antibody-mediated glomerulonephritis, variable granular or linear C3 staining is present, and the latter pattern may be continuous or discontinuous. and anti-GBM disease is extremely rare. The correct diagnosis of membranous nephropathy with anti-GBM relies on a combination of renal biopsy findings and serological screening. Early diagnosis is needed to improve the renal dysfunction. Keywords: Membranous nephropathy, Anti-glomerular basement membrane disease, Rapidly progressive glomerulonephritis Background Membranous nephropathy (MN) is usually a common cause of nephrotic syndrome in adults, and it is characterized by the presence of subepithelial immune complexes followed by match activation, basement membrane damage, and proteinuria. Fibrinoid SP600125 necrosis and crescent formation are rarely encountered with MN. MN with crescents may occur as a dual glomerulopathy with superimposed antineutrophil cytoplasmic antibody (ANCA)-associated crescentic glomerulonephritis or, less often, anti-glomerular basement membrane (anti-GBM) antibodies. The combination of MN and anti-GBM disease has been well documented since the first statement in 1974 [1]. This dual glomerulopathy likely represents the coincidental occurrence of two individual disease processes, MN followed by anti-GBM disease or the simultaneous presence SP600125 of the two diseases [2C8]. Occasionally, anti-GBM followed by MN has also been reported in individual cases [9C12]. We statement 3 cases on MN with anti-GBM disease and analyzed their clinical and pathological characteristics. The correct diagnosis of MN with anti-GBM disease should rely on a combination of renal biopsy findings and serological screening. Case presentation Case 1 A 67-year-old woman with severe edema and anuria for 14?days was admitted to our hospital. On admission, she experienced pitting edema in her lower legs, shifting dullness in her stomach, which is a sign of ascites, and body weight gained of 2C5?kg. She experienced an upper respiratory tract contamination and hemoptysis at home. Her body temperature was 36.4?C, her pulse was 80 breaths/minute, and her blood pressure was 164/87?mmHg. Urinalysis revealed 4?+?protein with numerous red blood cells in the sediment. Her hemoglobin level was 89?g/L, and her white blood cell count was 15.89??109/L, with 87.7% neutrophils, 5.8% lymphocytes, 6.3% monocytes, 0% eosinophils, and 0.2% basophils. Her platelet count was 291??109/L. Serum albumin level was 25?g/L. Renal function deteriorated rapidly, and her serum creatinine level was 7.79?mg/dL (normal range 0.7C1.4?mg/dL). Anti-GBM was positive, and the titer was?>?200 RU/ml (negative range, less than 9 units). Serum M-type phospholipase A2 receptor antibody (anti-PLA2R) was positive (1:10 positive, indirect immunofluorescence assay). Her C-reactive protein level was 265.02?g/dl, and her SP600125 anti-streptolysin-O and anti-streptokinase titers were within normal ranges. Her serum cholesterol was slightly elevated (5.96?mmol/L). Antineutrophil cytoplasmic antibodies (ANCAs) were negative. Assessments for anti-nuclear antibody (ANA), double-strand DNA (ds-DNA), extractable nuclear antibody (ENA), anti-SSA, anti-SSB, hepatitis B or C and human immunodeficiency computer virus were unfavorable. Immunoglobulins (IgG, IgA, SP600125 and IgM) and C3 and C4 levels were normal. Computed tomography (CT) of the chest revealed lung congestion and exudation. Renal ultrasound revealed that this sizes of both kidneys were normal (right, 10.5??4.9?cm; left, 11.4??4.7?cm), and the thickness of the cortex in both kidneys was normal (0.8?cm). Renal biopsy was performed for diagnosis. Light microscopy revealed crescentic glomerulonephritis with 15 of 17 glomeruli showing circumferential cellular crescent formation with fibrinoid necrosis. The glomerular capillary walls experienced spikes on PAM staining. Diffuse interstitial cell infiltration was observed with moderate tubular degeneration and interstitial fibrosis. Light microscopy pathology indicated the presence of crescentic glomerulonephritis (Fig.?1). Immunofluorescence exhibited coexistent finely granular and linear depositions of IgG, IgG subclass except IgG4, C3 and and light chains along the glomerular capillary walls. However, PLA2R and IgG4 staining was granular along SP600125 the glomerular capillary walls (Fig.?2). Electron microscopy revealed irregularly thickened glomerular basement membranes, rupture of the GBM and subepithelial electron-dense deposits. Podocytes revealed diffuse foot-process effacement and microvillous transformation (Fig.?1). Open in a separate windows Fig. 1 A Circumferential cellular crescent formation accompanying fibrin deposits (HE??200). B Transmission electron Rabbit Polyclonal to ARSE microscopy image showing diffuse foot process effacement of the podocytes and subepithelial immunocomplex deposits (?4000) Open in a separate window Fig. 2 A Direct immunofluorescence reaction with FITC (fluorescein isothiocyanate)-labeled anti-IgG (notice the simultaneous granular and linear staining). B Indirect immunofluorescence reaction with FITC-labeled anti-IgG3.