Is early diagnosis of pulmonary arterial hypertension possible in inflammatory rheumatic diseases? Experience from a single center in Turkey

Objective: Pulmonary arterial hypertension (PAH) is a devastating complication of inflammatory rheumatic diseases. The aim of this study was to determine the role of screening for the early diagnosis of pulmonary hypertension (PH) in inflammatory rheumatic diseases. Material and Methods: Data of patients with inflammatory rheumatic diseases and PH who had no obvious cause of PH and who were evaluated by Working Group for Pulmonary Hypertension in Hacettepe University were investigated retrospectively. All patients with inflammatory disease were evaluated by right heart catheterization (RHC) to check if they had systolic pulmonary arterial pressure (sPAP) >= 40 mmHg and/or symptoms related to PH unless explained by other causes. Results: RHC was performed in 47 patients with inflammatory rheumatic diseases and PH out of 50 patients who were to be evaluated by RHC based on clinical and Doppler echocardiographic findings. There was a positive correlation between sPAP estimated by Doppler echocardiography and sPAP determined by RHC in patients with inflammatory rheumatic diseases (r=0.66; p<0.001). The mean pulmonary arterial pressure (mPAP) was found to be <25 mmHg in 27.7% of the patients. New York Heart Association functional capacity (NYHA FC) was class III or IV in 79.0% of the patients with PAH. PAH was more frequent in patients with NYHA FC III-IV compared with patients with NYHA FC I-II [58.7% (15) patients vs. 19.0% (4) patients; p=0.009]. Conclusion: In this study, approximately 80% of the patients with inflammatory disease-associated PAH were diagnosed late in NYHA FC III or IV. There are still unresolved issues in the diagnosis and treatment of PH in inflammatory diseases. Collaboration and multidisciplinary approach are the key points to overcome the challenges in this field.