Pauci-immune glomerulonephritis

Synonyms

Rapidly progressive glomerulonephritis (RPGN), crescentic glomerulonephritis, ANCA associated glomerulonephritis.

Definition

Pauci-immune glomerulonephritis is the most common type of RPGN, and refers to the paucity or absence of immune deposits seen on immunofluorescence. Other histologic features include crescents and necrotizing glomerular inflammation. Pauci-immune glomerulonephritis may present in association with small vessel vasculitis, systemic disease, or limited to the kidneys. Antineutrophil cytoplasmic antibodies (ANCAs) are positive in the majority of patients, and thus this glomerular disease is often referred to as ANCA-associated glomerulonephritis. These diseases include Wegener's granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome.

Symptoms of pauci-immune glomulonephritis include myalgias, hematuria, proteinuria, oligouria or anuria, weight loss, and fever. Most patients have renal impairment at the time of diagnosis. Patients may have other...