Abstract
Pulmonary hypertension (PH) is common in most forms of connective tissue disease (CTD);
the prevalent type of PH depends on the particular CTD. Thus, pulmonary arterial hypertension
(PAH) is dominantly associated with scleroderma, while postcapillary PH is most common
in rheumatoid arthritis and lung disease-associated PH is typically found in myositis
and sarcoidosis.
Considerable expertise is required to identify, diagnose, and manage CTD-PH, as the
primary physicians providing the majority of care for this population, rheumatologists,
need a good working knowledge of CTD-PH, its rather subtle presentation, and how to
access the necessary investigations to screen for and identify patients with PH. The
role of the rheumatologist does not stop at diagnosis; in some conditions such as
lupus, optimizing immunosuppression is key to the management of PH, and unlike simple
idiopathic PAH, the natural history of CTD-PH is often punctuated by complications
of the CTD rather than just events due to progression of PH or therapy-related adverse
events.
The aim of this article is to provide an overview of all forms of CTD-PH, and to provide
an easy reference source on current best practice.
Keywords
pulmonary hypertension - pulmonary arterial hypertension - screening - WHO group -
risk assessment tools - NTproBNP - SSc PAH
