The pathophysiologic effects of FR167653 were investigated in a model of crescentic glomerulonephritis induced by a small dose of nephrotoxic serum in Wistar-Kyoto rats. The rats developed crescentic glomerulonephritis by 6 d after the administration of serum. The subcutaneous administration of FR167653 (32 mg/kg) markedly decreased the severity of the renal damage. In a group of rats treated w...

Pauci-immune crescentic glomerulonephritis (CrGN) is one of the most common causes of rapidly progressive glomerulonephritis. The majority of patients with pauciimmune CrGN have circulating antineutrophil cytoplasmic autoantibodies (ANCA). Approximately 10% of systemic vasculitides patients test negative for ANCA. Majority of the ANCA negative pauciimmune CrGN described in the literature have n...

Antineutrophil cytoplasmic antibodies are associated with pauci-immune small-vessel vasculitis and crescentic glomerulonephritis. Cathelicidin LL37 is the human member of a family of antimicrobial peptides that are released from activated neutrophils and monocytes at sites of acute inflammation. Zhang and colleagues evaluated serum levels of cathelicidin LL37 and interferon-alpha in patients wi...

INTRODUCTION A ntiglomerular basement membrane (GBM) disease is an autoimmune disease that classically presents as a rapidly progressive crescentic glomerulonephritis, with or without pulmonary hemorrhage, and typically does not relapse. The anti-GBM autoantibodies, typically polyclonal IgG1 and IgG4, bind to the noncollagenous 1 domain of the alpha-3 chain of type IV collagen that is present o...

Hypocomplementemic urticarial vasculitis (HUV) is the rare immune complex vasculitis, affecting small vessels and associated with anti-C1q antibodies, presenting with urticaria and hypocomplementemia. Glomerulonephritis, arthritis, obstructive pulmonary disease, and ocular inflammation are common in HUV. HUV may present as an idiopathic disease or be a part of systemic autoimmune or autoinflamm...

Cogan syndrome is a systemic disease manifesting interstitial keratitis, sensorineural hearing loss, tinnitus, and rotatory vertigo. Renal complications of this syndrome are very rare. We encountered an adolescent with Cogan syndrome complicated by aortitis and anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis. At the age of 14, the patient showed proteinuria in a screen...