title: Factors influencing patient survival and renal function outcome in pulmonary-renal syndrome associated with ANCA (+) vasculitis: a single-center experience.
abstract: Small vessel vasculitides, usually associated with positive antineutrophil cytoplasmic antibodies (ANCA(+)) can cause pulmonary-renal syndrome (PRS). Data from 22 patients, (19 males), aged 28-76 yrs (mean 55), with PRS were analyzed retrospectively. Renal function was estimated at presentation, 1 month after treatment initiation and at the end of follow-up (mean 4.4 +/- 3.3 yrs). Thirteen out of 22 patients had PR3 (+) ANCA and 9/22 patients had MPO (+) ANCA. Mean serum creatinine (Cr) at diagnosis was 6.6 +/- 4.4 mg/dL (M +/- SD) and proteinuria 1.6 +/- 1.4 g/24 hr (M +/- SD). During the 1st month of treatment with corticosteroids and cyclophosphamide, renal function improved in 12 patients (54.5%) (serum Cr from 8.5 +/- 4.5 to 4.3 +/- 2.3 mg/dL, p=0.001) remaining stable thereafter, and renal function deteriorated in nine patients (41%) (serum Cr from 4.1 +/- 3 to 6.5 +/- 2.9 mg/dL, p=0.03); one patient (4.5%) died because of sepsis. At the end of the study, 11/22 patients (50%) had died, eight patients of these (73%) because of respiratory failure, three patients (13.6%) reached end-stage renal disease (ESRD), five patients (36.4%) remained stable, but with impaired renal function and finally three patients (13.6%) improved, achieving almost normal renal function. In multiple regression analysis, factors contributing to final serum Cr were: dialysis dependency at the time of diagnosis p=0.01, initial proteinuria p<0.0001, and percentage of cellular crescents p=0.003. Main differences between PR3 and MPO (+) patients were the chest CT findings (bilateral nodules in PR3 (+) and "ground glass" or fibrosis in MPO (+) patients) and the renal function improvement rate after treatment initiation (rapid decline in serum Cr in PR3 (+) patients). In conclusion, PRS with ANCA (+) is associated with increased mortality. If renal function improves during the 1st month of treatment, it usually remains stable thereafter. The presence of PR3-ANCA is associated with an early response to treatment, while MPO-ANCA vasculits seems to necessitate prolonged treatment.
authors.affiliation: Nephrology Department, Aristotelion University of Thessaloniki, Thessaloniki-Greece. mstangou@math.com
authors.forename: Maria
authors.initials: M
authors.lastname: Stangou
authors.forename: Afroditi
authors.initials: A
authors.lastname: Asimaki
authors.forename: Gerasimos
authors.initials: G
authors.lastname: Bamichas
authors.forename: Fontini
authors.initials: F
authors.lastname: Christidou
authors.forename: Nikolaos
authors.initials: N
authors.lastname: Zoumbaridis
authors.forename: Taisir
authors.initials: T
authors.lastname: Natse
authors.forename: Nikolaos
authors.initials: N
authors.lastname: Galanis
authors.forename: Pandora
authors.initials: P
authors.lastname: Christaki
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Patakas
authors.forename: Kostas
authors.initials: K
authors.lastname: Sombolos
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007668:Kidney / Q000473:pathology / Q000503:physiopathology*; D007674:Kidney Diseases / Q000188:drug therapy / Q000209:etiology* / Q000401:mortality / Q000473:pathology / Q000503:physiopathology*; D008171:Lung Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000401:mortality; D008297:Male; D008875:Middle Aged; D053146:Myeloblastin; D009195:Peroxidase / Q000097:blood; D013902:Radiography, Thoracic; D012189:Retrospective Studies; D012697:Serine Endopeptidases / Q000097:blood; D016019:Survival Analysis; D013577:Syndrome; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014657:Vasculitis / Q000097:blood / Q000150:complications* / Q000276:immunology* / Q000628:therapy
chemical_list: D000305:Adrenal Cortex Hormones; D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D009195:Peroxidase; D012697:Serine Endopeptidases; D053146:Myeloblastin