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Article Abstract
Objectives: Current guidelines suggest treating poor-prognosis eosinophilic granulomatosis with polyangiitis (EGPA) with a combination of glucocorticoids (GCs) plus cyclophosphamide (CYC). However, there is little data to support the need for the addition of CYC. The objective of this study was to compare GCs plus CYC to GCs alone as induction therapy in poor-prognosis EGPA.
Methods: We emulated a target trial using observational data from a European multicenter retrospective database. We included patients with newly diagnosed EGPA with a 1996 Five Factor Score (FFS) of at least 1, treated with GCs or GCs plus CYC between June 1985 and November 2018. Propensity score analysis was used to adjust for potential confounders. Primary outcome was relapse at 12months. Secondary outcomes included major relapse at 12months and GC-dependent asthma and/or ear nose and throat (ENT) manifestations at 24months.
Results: A total of 209 patients were included: 47 % were male and the mean age at diagnosis was 52 (±16 years); 26 % were treated with GCs alone and 74 % with GCs plus CYC. After adjustment, the risk of relapse (hazard ratio [HR]: 0.24, 95%CI [0.08-0.67], p = 0.007), major relapse (HR: 0.24, 95%CI [0.07-0.85], p = 0.026) and the proportion of GC-dependent asthma and/or ENT manifestations (odds ratio:0.30, 95%CI [0.14-0.66], p = 0.003) were lower in the GCs plus CYC group compared to the GCs alone group.
Conclusion: This target trial emulation study shows that the addition of CYC to GCs reduces the risk of vasculitis relapse and the rate of GC-dependent asthma and/or ENT manifestations in patients with poor-prognosis EGPA.
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Article Synopsis
- The study aimed to assess the effectiveness of combining glucocorticoids (GCs) with cyclophosphamide (CYC) for treating patients with poor-prognosis eosinophilic granulomatosis with polyangiitis (EGPA) compared to using GCs alone.
- Data from a European multicenter database were analyzed, involving 209 patients and focusing on relapse rates and other related outcomes over 12 to 24 months.
- Results indicated that adding CYC significantly reduced the risk of relapse and related complications compared to GCs alone, suggesting a beneficial role for CYC in the treatment of poor-prognosis EGPA.
A retrospective study of infantile-onset Takayasu arteritis: experience from a tertiary referral center in China.
Front Cardiovasc Med
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Department of Rheumatology and Immunology, Children's Hospital of Nanjing Medical University, Nanjing, China.
Objective: Takayasu artery (TAK) is a chronic inflammatory disease that mainly affects the aorta and its major branches and is rarely reported in infants. We aimed to summarize the clinical features of infant TA (I-TA) in a tertiary care center.
Methods: We performed a retrospective study involving 10 infants diagnosed with TAK.