B cell depletion therapy is a potentially effective and safe adjuvant treatment for systemic sclerosis-pulmonary arterial hypertension (SSc-PAH). Future studies in these patients can confirm whether the identified biomarkers predict rituximab-responsiveness.

In an NIH-sponsored, multi-center, double-blinded, randomized, placebo-controlled, proof-of-concept trial, 57 SSc-PAH patients on stable-dose standard medical therapy received two infusions of 1000 mg of rituximab or placebo administered two weeks apart.

We randomized 57 subjects from 2010-2018. In the primary analysis, using data through week 24, the adjusted mean change in 6MWD at 24 weeks favored the treatment arm but did not reach statistical significance.

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