Renal involvement in Churg-Strauss syndrome.
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.
Sinico
Renato Alberto
RA
Dipartimento Area Medica, Azienda Ospedaliera Ospedale San Carlo Borromeo, Milan, Italy. renato.sinico@oscb.sined.net
Di Toma
Lucafrancesco
L
Maggiore
Umberto
U
Tosoni
Cinzia
C
Bottero
Paolo
P
Sabadini
Ettore
E
Giammarresi
Gaia
G
Tumiati
Bruno
B
Gregorini
Gina
G
Pesci
Alberto
A
Monti
Stefano
S
Balestrieri
Genesio
G
Garini
Giovanni
G
Vecchio
Filomena
F
Buzio
Carlo
C
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
D019268:Antibodies, Antineutrophil Cytoplasmic