Atypical antiglomerular basement membranes disease with nephrotic-range proteinuria, mesangial proliferation, and membranoproliferative glomerulonephritis pattern of injury.

Atypical antiglomerular basement membranes disease with nephrotic-range proteinuria, mesangial proliferation, and membranoproliferative glomerulonephritis pattern of injury. Saudi J Kidney Dis Transpl. 2017 Nov-Dec;28(6):1397-1403 Authors: AlSowailmi B, AlSowailmi G, Aloudah N, Alsaad KO, Elhassan E, Al Sayyari AA Abstract Antiglomerular basement membrane (anti-GBM) disease is an uncommon autoimmune disease characterized by the presence of IgG autoantibodies targeting the alpha-3 chain of type IV collagen. Some of the atypical forms of the disease have been described. Herein, we describe a case of atypical anti-GBM in a 27-year-old Saudi male who presented with lower limb edema, gross hematuria, elevated serum creatinine concentration, and nephrotic-range proteinuria. All serology tests were negative, except for anti-GBM which was weakly positive. Renal biopsy showed proliferative glomerulonephritis (GN) with nodular transformation of the glomerular tufts, mesangial hypercellularity (mesangial cell proliferation), segmental endocapillary hypercellularity and three incomplete cellular crescents, and recapitulating membranoproliferative GN pattern of glomerular injury. Direct immunofluorescence microscopy demonstrated diffuse, intense linear positivity for IgG and Kappa and Lambda light chains, and compatible with anti-GBM disease. The patient was treated with cyclophosphamide and corticosteroids in addition to therapeutic plasma excha...
Source: Saudi Journal of Kidney Diseases and Transplantation - Category: Urology & Nephrology Authors: Tags: Saudi J Kidney Dis Transpl Source Type: research