[Kidney involvement in systemic necrotizing vasculitis].
Renal involvement occurs in 75% of the patients with systemic necrotizing small-vessel vasculitis ie microscopic polyangiitis, Wegener's granulomatosis or Churg-Strauss syndrome. Small-vessel vasculitis may also be limited to the kidney. The hallmark of small-vessel pauci-immune vasculitides consists of necrotizing glomerulonephritis and resultant crescent formation, without immune-complex deposits in vessel walls. The resulting renal manifestations consist of haematuria, proteinuria and rapidly progressive renal failure. ANCA testing has a 90% sensitivity for renal-associated pauci-immune small-vessel vasculitis. Kidney biopsy is required for demonstrating necrotizing vasculitis. Early identification and prompt treatment are mandatory to avoid early mortality and end-stage renal failure. Induction therapy combines corticosteroids and IV cyclophosphamide. Plasma exchange in indicated in the patients presenting with active renal lesions and serum creatinine > 500 micromol/L. Maintenance of immunosuppressive therapy is required for 18 months. Twenty to 50% of the patients relapse during follow-up, and close monitoring is warranted for early detection.
Chauveau
Dominique
D
Service de néphrologie et immunologie clinique, hôpital de Rangueil, Toulouse. chauveau.d@chu-toulouse.fr
Guittard
Joëlle
J
Mehrenberger
Marion
M
Pourrat
Jacques
J
D000305:Adrenal Cortex Hormones / Q000008:administration & dosage / Q000627:therapeutic use; D001706:Biopsy; D015267:Churg-Strauss Syndrome / Q000175:diagnosis* / Q000473:pathology; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D005500:Follow-Up Studies; D005921:Glomerulonephritis / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000473:pathology; D006417:Hematuria / Q000175:diagnosis; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D007668:Kidney / Q000473:pathology*; D015337:Multicenter Studies as Topic; D010951:Plasma Exchange; D011379:Prognosis; D011507:Proteinuria / Q000175:diagnosis; D012008:Recurrence; D051437:Renal Insufficiency / Q000209:etiology / Q000473:pathology / Q000628:therapy; D013997:Time Factors; D014657:Vasculitis / Q000175:diagnosis* / Q000473:pathology
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide