Chun-Yu Lin1, Chi-Hua Ko2, Chung-Yuan Hsu3, Hung-An Chen4. 1. Division of Rheumatology, Department of Internal Medicine, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan; Division of General Medicine, Department of Internal Medicine, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan. 2. Department of Life Sciences, National Chung Hsing University, Taichung, Taiwan; Department of Rheumatology, Allergy and Immunology, Chang Gung Memorial Hospital, Yunlin, Taiwan. 3. Division of Rheumatology, Allergy, and Immunology, Department of Internal Medicine, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan. 4. Division of Allergy-Immunology-Rheumatology, Department of Internal Medicine, Chi Mei Medical Center, Tainan, Taiwan. No.901, Zhonghua Rd., Yongkang Dist., Tainan 710, Taiwan. Electronic address: hachen801039@yahoo.com.tw.
Abstract
OBJECTIVE: To estimate and compare the incidence and survival impact of pulmonary arterial hypertension (PAH) among patients with various connective tissue diseases (CTDs). METHODS: We used a national health insurance database in Taiwan and enrolled patients with incident systemic sclerosis (SSc), systemic lupus erythematosus (SLE), primary Sjögren's syndrome (pSS), polymyositis/dermatomyositis (PM/DM) and rheumatoid arthritis (RA) between 2002 and 2013. We calculated the cumulative incidence of PAH and the probability of survival after PAH diagnosis by the Kaplan-Meier method. RESULTS: Of 1653 SSc patients, 127 (7.68%) developed PAH. Of 11,735 SLE patients, 242 (2.06%) developed PAH. Of 17,316 pSS patients, 1811 PM/DM patients, and 32,296 RA patients, 38 (0.22%), 6 (0.33%) and 15 (0.04%) patients developed PAH, respectively. The most common CTD among all CTD-PAH patients was SLE (57%), followed by SSc (30%), pSS (9%), RA (3%) and PM/DM (1%). The 1-, 3- and 5-year survival rates for SSc-PAH were 88.3%, 72.1% and 61.9%, respectively. The 1-, 3- and 5-year survival rates for SLE-PAH were 87.6%, 75.8% and 69.4%, respectively. The 1-, 3- and 5-year survival rates for pSS-PAH were 90.8%, 86.8% and 80.6%, respectively. CONCLUSIONS: SLE was the most common underlying CTD, followed by SSc, in the total CTD-PAH population. However, the highest risk of developing PAH was observed in the SSc groups. PAH is a very rare complication in pSS, PM/DM and RA. Patients with SSc-PAH have the worst survival rate compared to those with SLE-PAH or pSS-PAH.
OBJECTIVE: To estimate and compare the incidence and survival impact of pulmonary arterial hypertension (PAH) among patients with various connective tissue diseases (CTDs). METHODS: We used a national health insurance database in Taiwan and enrolled patients with incident systemic sclerosis (SSc), systemic lupus erythematosus (SLE), primary Sjögren's syndrome (pSS), polymyositis/dermatomyositis (PM/DM) and rheumatoid arthritis (RA) between 2002 and 2013. We calculated the cumulative incidence of PAH and the probability of survival after PAH diagnosis by the Kaplan-Meier method. RESULTS: Of 1653 SSc patients, 127 (7.68%) developed PAH. Of 11,735 SLEpatients, 242 (2.06%) developed PAH. Of 17,316 pSSpatients, 1811 PM/DMpatients, and 32,296 RApatients, 38 (0.22%), 6 (0.33%) and 15 (0.04%) patients developed PAH, respectively. The most common CTD among all CTD-PAH patients was SLE (57%), followed by SSc (30%), pSS (9%), RA (3%) and PM/DM (1%). The 1-, 3- and 5-year survival rates for SSc-PAH were 88.3%, 72.1% and 61.9%, respectively. The 1-, 3- and 5-year survival rates for SLE-PAH were 87.6%, 75.8% and 69.4%, respectively. The 1-, 3- and 5-year survival rates for pSS-PAH were 90.8%, 86.8% and 80.6%, respectively. CONCLUSIONS:SLE was the most common underlying CTD, followed by SSc, in the total CTD-PAH population. However, the highest risk of developing PAH was observed in the SSc groups. PAH is a very rare complication in pSS, PM/DM and RA. Patients with SSc-PAH have the worst survival rate compared to those with SLE-PAH or pSS-PAH.