Advances in Pulmonary Hypertension

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Guest Editor's Memo

Targeting the Connection Between PAH and Connecive Tissue Disease

Pulmonary hypertension (PH) is associated with all of the connective tissue diseases (CTDs), but it is most commonly seen in systemic sclerosis (scleroderma), followed by systemic lupus erythematosus, and the antiphospholipid antibody syndrome. Although the prognosis for CTD-associated PH has improved with the availability of numerous pulmonary arterial hypertension therapies, it still carries a substantially poorer prognosis than idiopathic pulmonary arterial hypertension, and necessitates additional research into its treatment and prevention.

Further complicating treatment, CTD-associated PH is often multifactorial and can be caused by any of the World Health Organization categories of PH. In this issue of Advances in Pulmonary Hypertension, the contributors review what is known regarding the epidemiology, pathobiology, and treatment of PH, with a focus on pulmonary arterial hypertension in systemic sclerosis, systemic lupus erythematosus, and antiphospholipid syndrome. — Kristin B. Highland, MD, MSCR

Editor's Memo

Highlighting Current and Future Perspectives in the Journal

The recent increase in awareness and recognition of pulmonary hypertension is in part related to the efforts of Rheumatologists who are now screening many of their patients for this deadly disease. This issue, guest edited by Dr Kristin Highland, focuses on the spectrum of connective tissue diseases and their relation to pulmonary hypertension. While there is still much to learn about how and why patients with connective tissue diseases develop pulmonary hypertension, the benefits of treatment for many of these patients are apparent. This issue also spotlights rheumatologist, Dr Virginia Steen, whose efforts in the world of pulmonary hypertension, particularly on screening and early diagnosis, are close to my heart. For our new International Corner, Dr Nick Morrell provides commentary on combination therapy issues covered in the previous issue of Advances, with an international perspective.

I am pleased to preview important content to be published in Advances in Pulmonary Hypertension in the next 2 issues. The journal will be focusing on 2 meetings held earlier this year that provided relevant and timely updates on pulmonary hypertension. First, physicians who were unable to attend the World Health Organization 4th World Symposium on Pulmonary Hypertension will be pleased to see the next issue and its comprehensive discussion of key findings from this meeting. Among the topics to be covered are: current perspectives on pathophysiology, diagnosis and assessment of pulmonary arterial hypertension (PAH), interventional and surgical modalities, endpoints and clinical trial design in PAH, as well as inflammation, growth factors, and pulmonary vascular remodeling.

In the following issue, the journal will feature content from another important meeting, this year's 8th International Conference and Scientific Sessions of the Pulmonary Hypertension Association. The information from this meeting will be gleaned from physicians who led the sessions and who are among the leading investigators in the field. We look forward to providing articles on these important issues and welcome your feedback. Please send your comments and suggestions to our executive editor at Stulink@aol.com. Ronald J. Oudiz, MD Editor-in-Chief