There are more than 200 different types of connective tissue diseases (CTD). Rare diseases such as Marfan and Ehler-Danlos are inherited. The more ‘common’ autoimmune CTD are rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), Sjogren Syndrome, mixed connective tissue disease (MCTD), idiopathic inflammatory myositis (IIM) and systemic vasculitis. Although these CTDs have diverse clinical symptoms, courses, and prognosis, they have overlapping features of development of specific autoantibodies, sustained inflammation and systemic phenotype involving several organs.
Pulmonary arterial hypertension (PAH) is a progressive disease characterised by an elevation of pulmonary arterial pressure (PAP) and pulmonary vascular resistance, leading to right ventricular and death. There was little interest in PAH until the epidemic of aminorex-induced PAH in the late 1960’s. Since then, advances in the epidemiology, mechanisms, and genetics of PAH have led to a surge of clinical trials since the turn of the new millennium, leading to development of various therapeutic options.
Now it is known that although PAH is rare is in its idiopathic form, it is not uncommonly associated with CTD. Real-life cases of CTD-PAH will be discussed in this talk. At the end of this talk, the participants should be knowledgeable in these aspects of CTD-PAH