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Review
. 2009 Nov;21(6):642-8.
doi: 10.1097/BOR.0b013e3283307dc8.

Therapy for pulmonary arterial hypertension associated with systemic sclerosis

Stephen C Mathai  1 , Paul M Hassoun
Affiliations
Review

Therapy for pulmonary arterial hypertension associated with systemic sclerosis

Stephen C Mathai et al. Curr Opin Rheumatol. 2009 Nov.

Abstract

Purpose of review: Systemic sclerosis is commonly complicated by pulmonary arterial hypertension (PAH-SSc) and is a leading cause of death in this population. We will review existing challenges and recent advances in the treatment of this disease.

Recent findings: Traditionally employed outcome measures in pulmonary arterial hypertension research may not be applicable in PAH-SSc. Importantly, new therapies that target abnormal cellular proliferation in the pulmonary vasculature are currently under investigation and may be particularly relevant to PAH-SSc.

Summary: Pulmonary arterial hypertension complicating systemic sclerosis occurs commonly and portends a poor prognosis. However, recent advances in our understanding of the disease in the context of systemic sclerosis may lead to novel diagnostic and therapeutic strategies that will ultimately improve quality of life and survival in this population.

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Figures

Figure 1
Figure 1. Proposed Treatment Algorithm for PAH-SSc
Treatment algorithm utilized by the Johns Hopkins Pulmonary Hypertension Program. This algorithm is based on a combination of available evidence from clinical trials and clinical experience. Clinical treatment algorithm for PAH-SSc employed by Johns Hopkins Pulmonary Hypertension Program. Note that this algorithm reflects our current practice patterns and includes aspects that have not been studied in clinical trials.
See this image and copyright information in PMC

References

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