Related Experiment Video
Updated: Sep 9, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Rituximab treatment for connective tissue diseases associated-pulmonary hypertension: a systematic literature review
Amany Touil1,2, Selma Bouden2,3, Sarra Maazaoui1,2
1Department of Respiratory Diseases and Interventional Endoscopy, Pavillon 2, Abderrahmen Mami Pneumology and Phthisiology Hospital, Ariana, Tunisia.
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.
Related Concept Videos
Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers
TKIs, such as imatinib (Gleevec), are particularly effective in tackling the growth and mitogenic factors that become upregulated in PAH patients. These factors contribute to the...
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...
Connective Tissue Cell Types
Fat cells (adipocytes), smooth muscle cells (myoblasts), and bone cells (osteoblasts) are some connective tissue cell types. Some immune system cells...
Pulmonary Hypertension: Classification and Pathogenesis
There are various classifications for PH, each relating to different underlying causes and also...
Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies
Rheumatic Heart Disease III: Medical Management

