title: Renal involvement in Churg-Strauss syndrome.
abstract: BACKGROUND
  Churg-Strauss syndrome (CSS) is a rare disorder characterized by asthma, eosinophilia, and systemic vasculitis. Renal involvement is not regarded as a prominent feature, and its prevalence and severity vary widely in published reports that usually refer to small series of selected patients.
  
  
  METHODS
  We examined the prevalence, clinicopathologic features, and prognosis of renal disease in 116 patients with CSS.
  
  
  RESULTS
  There were 48 men and 68 women with a mean age of 51.9 years (range, 18 to 86 years). Signs of renal abnormalities were present in 31 patients (26.7%). Rapidly progressive renal insufficiency was documented in 16 patients (13.8%); urinary abnormalities, 14 patients (12.1%); and chronic renal impairment, 1 patient. There were 3 additional cases of obstructive uropathy. Sixteen patients underwent renal biopsy, which showed necrotizing crescentic glomerulonephritis in 11 patients. Other diagnoses were eosinophilic interstitial nephritis, mesangial glomerulonephritis, and focal sclerosis. Antineutrophil cytoplasmic antibody (ANCA) was positive in 21 of 28 patients (75.0%) with nephropathy versus 19 of 74 patients without (25.7%; P < 0.001). In particular, all patients with necrotizing crescentic glomerulonephritis were ANCA positive. After a median follow-up of 4.5 years, 10 patients died (5 patients with nephropathy) and 7 patients developed mild chronic renal insufficiency. Five-year mortality rates were 11.7% (95% confidence interval, 3.9 to 33.3) in patients with nephropathy and 2.7% (95% confidence interval, 0.7 to 10.7) in those without (P = 0.10).
  
  
  CONCLUSIONS
  Renal abnormalities are present in about one quarter of patients with CSS. The prevailing picture is ANCA-associated necrotizing crescentic glomerulonephritis; however, other forms of nephropathy also may occur. Outcome and long-term follow-up usually are good.
authors.affiliation: Dipartimento Area Medica, Azienda Ospedaliera Ospedale San Carlo Borromeo, Milan, Italy. renato.sinico@oscb.sined.net
authors.forename: Renato Alberto
authors.initials: RA
authors.lastname: Sinico
authors.forename: Lucafrancesco
authors.initials: L
authors.lastname: Di Toma
authors.forename: Umberto
authors.initials: U
authors.lastname: Maggiore
authors.forename: Cinzia
authors.initials: C
authors.lastname: Tosoni
authors.forename: Paolo
authors.initials: P
authors.lastname: Bottero
authors.forename: Ettore
authors.initials: E
authors.lastname: Sabadini
authors.forename: Gaia
authors.initials: G
authors.lastname: Giammarresi
authors.forename: Bruno
authors.initials: B
authors.lastname: Tumiati
authors.forename: Gina
authors.initials: G
authors.lastname: Gregorini
authors.forename: Alberto
authors.initials: A
authors.lastname: Pesci
authors.forename: Stefano
authors.initials: S
authors.lastname: Monti
authors.forename: Genesio
authors.initials: G
authors.lastname: Balestrieri
authors.forename: Giovanni
authors.initials: G
authors.lastname: Garini
authors.forename: Filomena
authors.initials: F
authors.lastname: Vecchio
authors.forename: Carlo
authors.initials: C
authors.lastname: Buzio
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology; D015267:Churg-Strauss Syndrome / Q000150:complications* / Q000276:immunology; D005260:Female; D006801:Humans; D007674:Kidney Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011379:Prognosis; D012189:Retrospective Studies
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic