Mediterr J Rheumatol 2019;30(2):90-3
Pulmonary Arterial Hypertension in Connective Tissue Disorders: The emerging role of screening and early diagnosis. A position paper for Greek Rheumatologists
Authors Information

1Onassis Cardiac Surgery Center, Pulmonary Hypertension Unit, Athens, Greece

2Attikon University Hospital, Pulmonary Hypertension Clinic, Athens, Greece

34th Department of Internal Medicine, Hippokration Hospital, Thessaloniki, Greece

4AHEPA University General Hospital, Pulmonary Hypertension Unit, Thessaloniki, Greece

5Rheumatology and Clinical Immunology Unit, "Attikon" University Hospital, Athens, Greece

6Department of Thoracic Medicine, Faculty of Medicine, University of Crete, Crete, Greece

7Department of Cardiology, Larissa University Hospital, Larissa, Greece

8Rheumatology Clinic, Department of Internal Medicine, Medical School, University of Ioannina, Ioannina, Greece

9First Department of Propaedeutic Internal Medicine and Rheumatology Unit, Medical School, National and Kapodistrian University of Athens, Athens, Greece


Pulmonary Arterial Hypertension is regarded as a devastating disease, complicating Connective Tissue Diseases. Although much progress has been achieved in the last 20 years, several unfulfilled needs in diagnosis and management of PAH in these patients may still be identified.

After a systematic review of the literature and integrating results from the latest research articles, key clinical issues for the screening and diagnosis of Pulmonary Arterial Hypertension in Connective Tissue Disorder Patients and specifically Scleroderma patients are described in this article, allowing physicians to contribute to early diagnosis of patients with Scleroderma-associated Pulmonary Arterial Hypertension.