Connective tissue associated pulmonary arterial hypertension

The number of publications in the field of pulmonary hypertension has increased exponentially over the past 10 years. As a consequence our understanding of the condition has advanced enormously. Pulmonary hypertension is now recognised as an uncommon and treatable disease, rather than a rare lethal disease. Progress in the field of connective tissue disease associated pulmonary hypertension has been less consistent. This paper covers the most influential published data on connective tissue associated pulmonary hypertension, and adds some insights from data available on file with pharmaceutical companies and from our database at the Royal Free Hospital. The following conclusions may be drawn. First, the prevalence of pulmonary hypertension associated with connective tissue diseases is lower than generally accepted. Second, the natural history and prognosis, at least in the setting of systemic sclerosis, is worse than seen in patients with idiopathic pulmonary hypertension. Finally, widespread and timely availability of disease modifying therapies substantially improves the prognosis for patients with systemic sclerosis associated pulmonary hypertension, in the treatment population and consequentially in the population as a whole. Identifying patients with very early disease and analysing the drivers for disease progression, is the key to further progress. Early diagnosis is most readily achieved in patients with connective tissue disease associated pulmonary hypertension. This represents an opportunity for those with an interest in academic rheumatology.