Sindrome di Churg Strauss

Objective: To estimate the prevalences and the incidences of these 4 vasculitides in a Parisian suburban population during the year 2000 using capture – recapture analysis.

Study design: Cases were collected in Seine – Saint-Denis County, a northern suburb of Paris, which has 1,382,928 inhabitants, 1,093,515 of whom are ³15 years old. The study period encompassed the entire calendar-year 2000. The cases were identified by 2 separate survey sources: 1) mailing with follow-up letter to all the general practitioners (n=1119); 2) mailing with telephone follow-up to the departments of internal medicine, rheumatology, nephrology, pneumology and hemodialysis of all the public hospitals and 2 big private clinics (n=20). The medical charts of all such identified cases were reviewed and diagnoses confirmed according to the Chapel Hill Consensus Conference nomenclature. The point prevalences and incidences were estimated by 2-source capture – recapture analysis using Chapman and Seber’s formula; the 95% confidence intervals (95% CI) were calculated using the Poisson distribution.

Results: The response rates were: 52% for general practitioners and 100% for hospital departments. Of the 59 cases identified (PAN, n=20; MPA, n=11; WG, n =21; CSS, n=7), 17 (29%) cases were retrieved from both sources (matches). The mean age was 59.1 yr (range: 19 – 84 yr) and the male/female ratio was 1.27. Ten diagnoses were made during 2000 (PAN, n=1; MPA, n=4; WG, n=4; CSS, n=1); 0 source matches. The table gives the estimated prevalences and year 2000 incidences (95% CI) in the ³15-year-old population.

Objective: This french multicenter prospective study compared the efficacies and safety of CS and 6 vs 12 IV pulse CY in CSS patients with at least 1 of the following five factors associated with a poor outcome: creatininemia > 120 mM, proteinuria > 1 g/d, GI tract, myocardial and/or CNS involvement (FFS ³ 1). Patients and Methods: Patients with CSS, diagnosed according to the 1990 ACR criteria and FFS ³ 1 at baseline, were enrolled between July 1994 and December 2000. The regimen consisted of CS (prednisone initially 1 mg/kg bw/d then gradually tapered) and randomly assigned 6 vs 12 pulses of monthly IV CY (0.6 g/m2 ). Only patients who had been followed for at least 6 months were considered for this abstract. Results: For the 39 enrolled patients (16 females, 23 males; mean age: 51.6 ± 15.2 yr) mean follow-up was 30.9 ± 20.7 mo; ANCA were detected in 15/39 (38.5%) patients. CY pulses were administrered for 6 or 12 mo to, respectively, 18 and 21 patients; 83.3 and 76.2% of them, respectively entered complete remission (CR). Four patients (2 in each arm) died on day 46, 54, 449 and 546 of cardiac failure (n = 2), infectious complications (n = 1) or uncontrolled vasculitis (n = 1). Neither side effects nor relapse rates differed significantly between the 2 arms. At the last update, 31/39 (79.5%) patients were in CR. Conclusion: Based on these preliminary results at 4 years, no significant differences in terms of CR, relapse or side effects were found for CSS patients with FFS ³ 1 given CS and 6 or 12 IV CY pulses.

Objective: This multicenter prospective trial tested the efficacy of CS alone in CSS patients without poor-prognostic factors at baseline, as assessed with the FFS: creatininemia > 120 mM, proteinuria > 1 g/d, GI tract, myocardial and/or CNS involvement. Patients and Methods: Patients with CSS diagnosed according to the1990 ACR criteria, and FFS =0 at baseline, were enrolled by the FVSG from July 1994 to December 2000. The regimen consisted of CS alone (prednisone initially 1 mg/kg bw/d then gradually tapered); immunosuppressants (6 mo of oral azathioprine (AZA) 2 mg/kg/d or monthly IV cyclophosphamide (CY) (0.6 g/m2 ) were randomized only in the case of uncontrolled vasculitis, relapse or when it was not possible to taper prednisone to < 20 mg/d. Only patients who had been followed at least 6 mo were considered for this abstract. Results: Forty-three patients (24 females, 19 males; mean age: 49.9 ± 14.9 yr) were enrolled. Mean follow-up was 32 ± 19.1 mo; ANCA were detected in 14/43 (32.6%) patients. Complete remission (CR) was obtained in 33/43 (76.7%) patients; 23/33 (69.7%) patients experienced mostly mild relapses requiring transient increases of CS doses. Five patients required CS doses > 20 mg/d; 5 other never achieved CR. The outcomes for patients randomized to receive either IV CY (n = 8) or oral AZA (n=7) did not differ significantly. At the last update, 36/43 patients were in CR; 1 patient allergic to AZA died of methotrexate-induced pneumonia. Conclusions: These preliminary results indicate that CSS patients with FFS = 0 at baseline can be safely treated with CS alone. Outcomes were comparable for patients who required AZA or IV CY adjunction.