Treatment of Churg‐Strauss syndrome without poor‐prognosis factors: A multicenter, prospective, randomized, open‐label study of seventy‐two patients

Abstract

Results. The mean ؎ SD followup was 56.2 ؎ 31.7 months. Among the 72 patients studied, 93% achieved remission with CS therapy alone, and 35% relapsed, mainly during the first year of treatment. Among the 19 patients randomized to additional immunosuppression because of treatment failure or relapse, 5 of 10 receiving AZA and 7 of 9 receiving pulse CYC achieved remission, but the difference was not statistically significant. Survival rates in all patients at 1 and 5 years were 100% and 97%, respectively. At the end of followup, 79% of the patients whose disease was in remission required lowdose CS therapy, mainly to control respiratory disease. CS-related adverse events were observed in 31% of the 72 patients.Conclusion. In CSS patients with an FFS of 0, survival was excellent, confirming the predictive value of the FFS in this disease. First-line therapy with CS achieved remission in most patients, but relapses were common, and one-third of them required additional immunosuppressive therapy. AZA or pulse CYC was fairly effective in treating CS-resistant disease or major relapses. Over the long term, most patients continued to take oral CS, which might explain the high rate of CS-related adverse events.Churg-Strauss syndrome (CSS), which is also called allergic granulomatosis and angiitis, is characterized by necrotizing eosinophilic vasculitis affecting the small vessels and extravascular granulomas. The major clinical manifestations are asthma, hypereosinophilia, and extrapulmonary manifestations of systemic vasculitis. Antineutrophil cytoplasmic antibodies (ANCAs), ClinicalTrials.gov identifier: NCT00399399.

Churg–Strauss syndrome: retrospective study in Burgundian population in France in past 10 years

“…Thirteen patients out of 31 (42%; IC=25-59%) did not have the adequate initial treatment according to current recommendations' based on the FFS score [4][5][6][7]. Globally, the proportion of inadequate treatment is in decline from 1982 to 2008: the patients are thus currently better treated.…”

“…We have considered as a flare-up all new visceral attack related to Churg-Strauss vasculitis hitherto latent or asymptomatic as well as any known visceral attack requiring a rise of the corticosteroid therapy strictly of more than 50% or the addition of an immunosuppressive treatment. The FFS (Five Factor Score) evaluating the vasculitides prognostic at ANCA (Anti-cytoplasm antibodies of polynuclear neutrophils) 6 [3] has been calculated during a diagnosis and compared to the initial treatment received by the patients and to the therapeutic recommendations based on this score [4][5][6][7]. The remission rate (free from flare-up for over 6 months) to a one-year treatment has also been counted.…”

Churg Strauss Sindrom Kod Šesnaestogodišnje Djevojčice

“…U bronhoalveolarnom lavatu (BAL) eozinofilija se može naći u 33% slučajeva. U tretmanu Churg-Strauss sindroma se uglavnom koriste kortikosteroidi koji su u većini slučajeva dovoljna terapija (16). U manje od 20% slučajeva potrebni su citotoksčni lijekovi kao što su mikofenolat mofetil, ciklofosfamid, azatioprim i drugi (16,17).…”

Antineutrofil citoplazmatikus antitest asszociált vasculitisek korszerű kezelése

“…A szteroiddal kezelt MPA-s vagy EGPA-s betegek relapsusrátája közel 50%-os, de a 7 éves túlélési arány 79%. A diagnózis felállításakor adott immunszuppresszív kezelés csökkentheti a relapsusokat, azonban a vizsgála-tok eredményei szerint a ciklofoszfamid mellékhatásai ebben az esetben meghaladják a terápiás előnyt [28]. Olyan kevésbé toxikus szerek, mint az azathioprin kipró-bálása a CHUSPAN 2 francia vizsgálat keretében folyamatban van.…”