title: [Wegener's granulomatosis. Diagnosis and follow-up of 7 cases].
abstract: Wegener's granulomatosis (WG) is a rare small vessels necrotizing and granulomatous systemic vasculitis which usually affects the respiratory-tract and the kidneys. Diagnosis is often difficult, but has become easier with antineutrophil cytoplasmic antibodies (ANCA) detection that may justify a more aggressive biopsy policy also in the elderly. Classic treatment with steroids and oral cyclophosphamide (CY) has proven to be of benefit, but side-effects are severe and frequent and the search for less toxic therapeutic schemes should be encouraged. We treated with intravenous pulses of CY (1 g/m2 monthly for 6 months, every two months for the following 6 and quarterly for another year) 5 of 7 patients with WG recently admitted to our institution. We obtained a quick, complete response in 4 of these patients, with no side effects, nor relapses, after a mean follow-up of 17 months. The only patient who did not respond was identified soon after the beginning of the treatment because of a poor reduction of ESR and could be shifted to oral administration of CY successfully. From our still limited experience CY intravenous pulses have proven to be safe and effective enough to advice its use as the first-choice treatment for WG.
authors.affiliation: Divisione di Nefrologia e Dialisi, Ospedale Mauriziano, Umberto I, Torino.
authors.forename: M
authors.initials: M
authors.lastname: Manganaro
authors.forename: M
authors.initials: M
authors.lastname: Bruno
authors.forename: R
authors.initials: R
authors.lastname: Pellerito
authors.forename: N
authors.initials: N
authors.lastname: Ravarino
authors.forename: B
authors.initials: B
authors.lastname: Torchio
authors.forename: A
authors.initials: A
authors.lastname: Tricerri
authors.forename: P
authors.initials: P
authors.lastname: Gabella
authors.forename: R
authors.initials: R
authors.lastname: De Giovanni
authors.forename: F
authors.initials: F
authors.lastname: Linari
mesh_terms: D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies / Q000097:blood; D015415:Biomarkers; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D014890:Granulomatosis with Polyangiitis / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies; D015415:Biomarkers; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide