Rituximab treatment for connective tissue diseases associated-pulmonary hypertension: a systematic literature review.

INTRODUCTION

To evaluate the efficacy and safety of Rituximab (RTX) in the treatment of pulmonary hypertension (PH) in patients with connective tissue diseases (CTDs).

METHODS

A systematic review of the published literature was performed according to the PRISMA guidelines. Eligible articles were clinical trials, cohort studies, case series, and case reports of adult patients with CTD-PH treated with RTX. The selected languages were English and French.

RESULTS

Six studies fulfilled the selection criteria and two of them were prospective. Most patients were from the U.S.A. and Russia. The predominant sex was female (85.9%), and the age of the included patients ranged from 29 years to 67 years. PH was confirmed by right heart catheterization in 3 studies. The CTDs associated with PH were systemic sclerosis (3 cases), adult-onset Still's disease (one case), systemic lupus erythematosus (one case) and mixed connective tissue disease (one case). The mean number of infusions was 4.2. The mean follow-up period across all studies was 18.3 months. Clinical and paraclinical parameters before and after RTX infusions were evaluated and showed improvement. RTX for the treatment of CTD-PH appeared to be well tolerated.

CONCLUSIONS

RTX seemed to be effective and safe in the treatment of CTD-PH.Systematic review registration: PROSPERO, ID: CRD420251037839.