AB002. Rheumatoid arthritis associated pulmonary hypertension: three cases with different pathophysiology

Abstract: Pulmonary hypertension (PH) occurs as a complication of rheumatoid arthritis (RA) increasing morbidity and mortality of the primary disease. The aim of this presentation is to elucidate the diversity of pathophysiological mechanisms that underlie pulmonary hypertension through three cases that were challenging. Three patients with diagnosed RA under immunosuppressing treatment appeared with dyspnea on exertion, respiratory failure and severe functional impairment. The first patient suffered from moderate restriction due to fibrosis and was diagnosed with pulmonary hypertension during an episode of life threatening hypoxia. Early upfront combination therapy prevented intubation and reversed hypoxia to adequate levels. The second presented patient was a case of isolated pulmonary hypertension attributable to vasculopathy. The patient maintained normal lung volumes but low diffusion capacity and echocardiography dictated the need for right heart catheterization. Finally, the third patient presented severe functional limitation due to several manifestations of rheumatoid arthritis, but a past episode of acute pulmonary embolism was also reported although it had never been evaluated. Chronic thromboembolic disease was eventually proved to be one major cause of the patient’s pulmonary hypertension. The condition’s three major causes are: interstitial lung disease, vasculitis, and chronic thromboembolic disease, but it should be noted that the multiple pulmonary manifestations of rheumatoid arthritis, can all contribute to chronic lung disease or hypoxia. The importance of early identification of pulmonary hypertension in patients with rheumatoid arthritis is therefore emphasized, especially since multiple treatment options are available, symptoms can be treated, and right heart failure can be avoided.

Keywords: Pulmonary hypertension (PH); vasculitis; rheumatoid arthritis (RA)