Crescentic glomerulonephritis without immune deposits: Clinicopathologic features

Abstract

Of 46 patients with acute crescentic glomerulonephritis involving 20 to 90% of glomeruli, 16 had no definable systemic disease and no significant glomerular immune deposits by immunofluorescent or electron microscopy. Anti-GBM antibody and circulating immune complexes were further excluded by radioimmunoassay and Raji cell assay in all patients tested. Clinical features included a 10:6 male:female ratio, mean age of 58 years (range, 13-77), disease duration of less than 3 months, rapidly deteriorating renal function, and frequent pulmonary manifestations. Nine patients had oliguria, serum creatinine concentrations over 6 mg/100 ml, and required dialysis, but three of these patients subsequently recovered renal function. These three patients and seven patients with creatinine concentrations of less than 6 mg/100 ml have not progressed to chronic renal failure. In this series, idiopathic acute crescentic glomerulonephritis without immune deposits was more common than was immune complex or anti-GBM nephritis. The clinical, laboratory, and pathologic characteristics of these patients were similar to those reported in anti-GBM and immune-complex-induced glomerulonephritis. These observations expand the spectrum of rapidly progressive crescentic glomerulonephritis. They suggest that glomerular immune deposits may be less important than other factors in determining the extent of renal injury and subsequent clinical course in crescentic glomerulonephritis.

Kidney dendritic cell activation is required for progression of renal disease in a mouse model of glomerular injury

“…Experimental studies proposed a role particularly in crescentic and pauci-immune GN (2,5,(23)(24)(25)(26)(27)(28). However, the exact mechanisms by which the distinct T cell types contribute to GN are only partially understood.…”

Anti-Neutrophil Cytoplasmic Antibodies and Effector CD4+ Cells Play Nonredundant Roles in Anti-Myeloperoxidase Crescentic Glomerulonephritis

“…In most autoantibody-mediated diseases, a key feature is the presence of the autoantibody at the sites of tissue injury. Paradoxically, ANCA-associated crescentic GN is uniquely characterized by an absence or minimal presence of antibodies in affected glomeruli, hence the historical description of the lesion as "pauciimmune" (10). However, both neutrophils and extracellular MPO are present in glomeruli (11).…”

Pathogenesis of Vascular Inflammation by Anti-Neutrophil Cytoplasmic Antibodies

“…In 1978, Fauci et al (4) contended that "most of the vasculitic syndromes are caused by, or closely associated with, deposition of immune complexes in blood vessel walls." However, immunohistologic evaluation of vessels from patients with Wegener's granulomatosis, microscopic polyangiitis, and renal-limited vasculitis demonstrated an absence or paucity immunoglobulins (5,6). Therefore, pauci-immune small-vessel vasculitis and glomerulonephritis seemed to have a pathogenesis that differed from typical immune complex disease.…”