Mehmet Soy1, Mustafa Yıldız2, Aslı Özmaden Hantal Özmaden Hantal3, Tuba Çiftçi Küsbeci4, İffet Doğan5

1Department of Internal Medicine-Rheumatology, Altınbaş University Faculty of Medicine, Bahçelievler Medicalpark Hospital, İstanbul, Türkiye
2Department of Cardiology, İstanbul University-Cerrahpaşa, Institute of Cardiology, İstanbul, Türkiye
3Department of Physical Therapy and Rehabilitation, Altınbaş University Faculty of Medicine, Bahçelievler Medicalpark Hospital, İstanbul, Türkiye
4Department of Chest Diseases, Altınbaş University Faculty of Medicine, Bahçelievler Medicalpark Hospital, İstanbul, Türkiye
5Department of Radiology, Istanbul Health Science University Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, İstanbul, Türkiye

Keywords: Connective tissue diseases; endothelin receptor antagonists; phosphodiesterase 5 inhibitors; prostacyclin analogs; pulmonary arterial hypertension; pulmonary hypertension; scleroderma.


Pulmonary hypertension (PH) is defined as mean pulmonary artery pressure >20 mmHg at rest, confirmed by right heart catheterization (RHC). The European Society of Cardiology and the European Respiratory Society (ESC/ ERS) published a new guideline in 2022 with recommendations for the classification, diagnosis, and treatment of PH. Pulmonary arterial hypertension (PAH) is a subgroup of PH and is most commonly seen together with connective tissue diseases after the idiopathic form. PAH may develop in connective tissue patients, most commonly in cases of systemic scleroderma. The presence of PAH significantly affects the quality of life and survival in connective tissue patients, especially in scleroderma. In PAH cases, early diagnosis and treatment before organ damage develops is the golden rule in treatment. Diagnosis should first be triggered by complaints such as unexplained dyspnea and syncope that develop in the presence of an underlying connective tissue disease such as scleroderma, which increases the risk of PAH and is made by RHC in the light of data obtained from examinations such as electrocardiogram, echocardiography, pulmonary function tests, and diffusing capacity for carbon monoxide. When planning treatment, pharmacological treatments are used in addition to non-pharmacological measures. Drug selection should be made by taking into account the patient’s other characteristics. Combination oral therapy with an endothelin receptor antagonist and a phosphodiesterase 5 inhibitor is often the first-line treatment in scleroderma-PAH. During follow-ups, treatment may be changed according to the patient’s clinical and laboratory data and risk analysis. For patients with functional class IV (the most severely ill patients), additional triple combination therapy consisting of a prostaglandin analog may be considered. Treatment is determined and followed according to the ERS/ESC 2022 guideline and other guidelines mostly developed in light of this guideline. Lung transplantation should not be ignored in cases resistant to these treatments. Despite current developments, the prognosis in PH cases is still poor and patients should be followed and treated in experienced centers specialized for PH.

Cite This Article: Soy M, Yıldız M, Özmaden Hantal A, Çiftçi Küsbeci T, Doğan İ. Pulmonary Hypertension in Connective Tissue Diseases. Koşuyolu Heart J 2024;27(1):40–52.

Peer Review

Externally peer-reviewed.

Author Contributions

Concept – M.S.; Design – M.S.; Supervision – M.Y.; Funding – A.Ö.H.; Materials – İ.D.; Data collection and/or processing – T.Ç.K.; Data analysis and/or interpretation – M.Y.; Literature search – A.Ö.H.; Writing – M.S.; Critical review – T.Ç.K.

Conflict of Interest

All authors declared no conflict of interest.

Use for AI for Writing Assistance

Not declared.

Financial Disclosure

The authors declared that this study received no financial support.