Abstract
BACKGROUND: Although the prognosis in systemic lupus erythematosus (SLE) has dramatically improved, pulmonary artery hypertension (PAH) is one of the life-threatening comorbidities associated with SLE. The management of the comorbidity is occasionally challenging due to the lack of consensus regarding treatment options including immunosuppressive agents, selective pulmonary vasodilators, and cardiac rehabilitation. CASE SUMMARY: A 28-year-old female who terminated prednisolone after remission of SLE by her own discretion 3 years ago developed dyspnoea on effort. Transthoracic echocardiography showed dilatation of the right atrium (RA) and ventricle (RV), as well as the RV dysfunction. The findings of right heart catheterization and pulmonary perfusion scintigraphy confirmed PAH associated with connective tissue disease. According to the SLE Disease Activity Index (SLEDAI) and the REVEAL Registry Risk Score, she was at high risk of PAH despite mild SLE activity. Upfront combination therapy including macitentan and tadalafil for PAH and steroid semi-pulse and cyclophosphamide pulse therapy for SLE alongside supervised cardiac rehabilitation were initiated simultaneously. The RA and RV sizes were normalized after the aforementioned therapy. The RV-pulmonary artery (PA) coupling improved from 0.15 to 0.77 mm/mmHg, and the mean PA pressure decreased from 55 to 29 mmHg. DISCUSSION: The case presentation highlighted the potential benefits of comprehensive treatment strategy including immunosuppressive treatment, upfront combination therapy, and supervised exercise training. Notably, the initiation of cardiac rehabilitation at the early phase did not exacerbate her condition and might have contributed to remission of symptoms and improvement in exercise tolerance. This multidisciplinary approach achieved long-term good quality of life.