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Anti-neutrophil cytoplasmic antibody vasculitis is a vasculitis of small vessels and can cause damage to many organs, most commonly the kidneys, upper and lower respiratory tract, gastrointestinal tract, skin, and nervous system. It is characterized by IgG antibodies that are directed against components of the cytoplasm of neutrophils. According to the immunofluorescence pattern, anti-neutrophil cytoplasmic antibodies are divided into antibodies with cytoplasmic immunofluorescence pattern, and antibodies with perinuclear immunofluorescence pattern. Anti-glomerular basement antibodies can be associated with the combined impairment of the lungs and kidneys (Goodpasture’s syndrome) or with isolated renal impairment (anti-glomerular basement membrane disease). The diagnosis of Goodpasture’s syndrome and anti-glomerular basement disease requires demonstration of anti-glomerular basement antibodies either in the serum or the kidney. Renal biopsy should be performed in every patient, because the accuracy of serologic assays is variable. The most common clinical manifestation in patients with anti-neutrophil cytoplasmic antibodies and anti-glomerular basement antibodies is acute renal failure due to rapidly progressive glomerulonephritis. Authors present a 49-year-old female with renal failure, without pulmonary impairment, who had positive anti-neutrophil cytoplasmic antibodies in serum and a rapidly progressive glomerulonephritis with deposits of IgG antibodies along the glomerular basement membrane.