pulmonary arterial hypertension in connective tissue diseases

Pulmonary arterial hypertension (PAH) is an entity that is known to complicate connective tissue diseases (CTD). PAH in CTD is a very important diagnosis which greatly affects treatment and prognosis.

The most commonly affected CTD is scleroderma, although lupus, inflammatory myopathies such as poly and dermatomyositis, and mixed CTD are also associated with PAH.

The manifestations of PAH have both similarities and differences when occurring in the setting of CTD as compared with idiopathic PAH. These differences are most notable in scleroderma.

See also

 lupus-associated pulmonary hypertension

References

 Frequency of Pleural Effusions in Patients with Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases. Luo YF, Robbins IM, Karatas M, Brixey AG, Rice TW, Light RW. Chest. 2011 Jan 6. PMID: 21212140

 Pulmonary arterial hypertension in connective tissue diseases. Goldberg A. Cardiol Rev. 2010 Mar-Apr;18(2):85-8. PMID: 20160534

 Fibrous remodeling of the pulmonary venous system in pulmonary arterial hypertension associated with connective tissue diseases. Dorfmüller P, Humbert M, Perros F, Sanchez O, Simonneau G, Müller KM, Capron F. Hum Pathol. 2007 Jun;38(6):893-902. PMID: 17376507