, Duygu Temiz Karadağ 2
, Mustafa Erdoğan 3
, Müçteba Enes Yayla 4
, Büşra Fırlatan Yazgan 5
, Betül Dikkanoğlu Demirok 2
, Shirkhan Amikishiyev 6
, Yasemin Yalçınkaya 6
, Murat İnanç 6
, Oğuz Karcıoğlu 7
, Uğur Nadir Karakulak 8
, Zeynep Pınar Önen 9
, Bahri Akdeniz 10
, Gülfer Okumuş 11
, Bülent Mutlu 12
, Serdar Küçükoğlu 13
, Ali Akdoğan 5
, Reviewer 1: Meral Kayıkçıoğlu 14
, Reviewer 2: Gülen Hatemi 15
2Department of Rheumatology, Kocaeli University Faculty of Medicine, Kocaeli, Türkiye
3Department of Rheumatology, Marmara University Pendik Training and Research Hospital, İstanbul, Türkiye
4Department of Rheumatology, Ankara University Faculty of Medicine, Ankara, Türkiye
5Department of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Türkiye
6Department of Rheumatology, İstanbul University Faculty of Medicine, İstanbul, Türkiye
7Department of Chest Diseases, Hacettepe University Faculty of Medicine, Ankara, Türkiye
8Department of Cardiology, Hacettepe University Faculty of Medicine, Ankara, Türkiye
9Department of Chest Diseases, Ankara University Faculty of Medicine, Ankara, Türkiye
10Department of Cardiology, Dokuz Eylül University Faculty of Medicine, İzmir, Türkiye
11Department of Chest Diseases, İstanbul University Faculty of Medicine, İstanbul, Türkiye
12Department of Cardiology, Marmara University Faculty of Medicine, İstanbul, Türkiye
13Department of Cardiology, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Türkiye
14Department of Cardiology, Ege University Faculty of Medicine, İzmir, Türkiye
15Department of Internal Medicine, Division of Rheumatology and Behçet Disease Research Center, İstanbul University-Cerrahpaşa, Cerrahpaşa Medical Faculty, İstanbul, Türkiye
Abstract
Patients with connective tissue diseases (CTDs) are at increased risk for the development of pulmonary hypertension (PH) and pulmonary arterial hypertension (PAH), which is a specific subtype of PH characterized by progressive remodeling of precapillary pulmonary arterioles. Evidence suggests that early detection of PH through screening in this patient group may be associated with better outcomes. Various methods, including cardiac and thoracic imaging, blood biomarkers, pulmonary function tests, and composite algorithms, have been employed for screening of PH. Here, a multidisciplinary consensus group composed of 10 rheumatologists, 4 cardiologists, and 3 pulmonologists was formed with the objective of developing recommendations and a screening algorithm for PH in patients with CTD. A systematic literature review in the PubMed database focusing on the studies evaluating the performance of different screening methods on detecting PH in CTDs was conducted. The literature review identified 33 relevant articles after title, abstract, and full-text evaluation. The included studies had considerable heterogeneity regarding hemodynamic definitions of PH and PAH, the diagnostic cutoff values of screening methods utilized, and the symptom status of the patients. With the exception of 2 studies, the populations in included studies consisted of only patients with systemic sclerosis (SSc). Consensus-based recommendations and an algorithm prioritizing echocardiography for screening and early detection of PH in patients with SSc and patients with CTD exhibiting overlap features of SSc were developed based on literature data and incorporating the perspectives of group members. No recommendations could be made for asymptomatic patients with CTDs without overlap features of SSc due to limited data.