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Updated: May 7, 2026

Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat
Published on: November 18, 2018
Pulmonary hypertension complicating connective tissue disease
Joseph P Lynch1, John A Belperio, Rajeev Saggar
1Division of Pulmonary, Critical Care Medicine, Clinical Immunology, and Allergy, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California.
Pulmonary hypertension (PH) complicates connective tissue disease (CTD), especially systemic sclerosis (SSc), increasing mortality. Scleroderma-associated PH has a poorer prognosis, and its treatment remains controversial.
Area of Science:
- Rheumatology
- Cardiology
- Pulmonology
Background:
- Pulmonary hypertension (PH) is a severe complication of connective tissue disease (CTD).
- Systemic sclerosis (SSc, scleroderma) accounts for over 70% of CTD-associated PH cases.
- Pulmonary complications, including PH and interstitial lung disease (ILD), are leading causes of death in SSc.
Purpose of the Study:
- To review the clinical presentation and management of PH in CTD, with a focus on SSc.
- To discuss diagnostic markers and autoantibody profiles associated with SSc-PH.
- To evaluate current therapeutic strategies and the role of lung transplantation for CTD-associated PH.
Main Methods:
- Literature review of studies on CTD-associated PH, particularly SSc-PH.
- Analysis of clinical markers and autoantibody associations with PH in SSc.
- Discussion of medical therapies and lung transplantation outcomes.
Main Results:
- PH complicates SSc in 7.5-20% of cases, with or without co-existing ILD.
- Prognosis and treatment response in SSc-PH are generally worse than in idiopathic pulmonary arterial hypertension.
- Specific clinical markers and autoantibody profiles are linked to PH in SSc.
Conclusions:
- SSc-associated PH significantly impacts mortality and presents unique therapeutic challenges.
- The efficacy of current PH-specific therapies in SSc remains debated.
- Lung transplantation is a consideration for refractory cases.
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