OBJECTIVE: To clarify the clinical features of systemic lupus erythematosus (SLE) complicated with pulmonary hypertension (PH) and to evaluate the efficacy of immunosuppressive treatment. METHODS: Case records were reviewed for 194 patients with SLE who were admitted to Aoyama Hospital of Tokyo Women's Medical University between 1992 and 1999. There were 12 patients with PH [8 SLE and 4 SLE + systemic sclerosis (SSc) overlap syndrome]. These patients were compared with 59 age and sex matched patients with SLE for clinical characteristics and laboratory findings. The efficacy of treatments for PH was also evaluated. RESULTS: In our cohort of 194 patients with SLE, 6.2% had PH. The plasma thrombin-antithrombin III complex and plasma D-dimer levels were significantly higher in patients with PH compared with those without PH. Eight patients with PH (4 SLE and 4 SLE + SSc) were treated with corticosteroids (CS) +/- cyclophosphamide (CYC). Right ventricular systolic pressure (RVSP) was improved in 7 of 8 patients. In 6 of 7 responders to the therapy, the treatment was started as soon as they were diagnosed with PH. PH relapsed in 2 patients treated with oral CS +/- CS pulse therapy, but their RVSP was decreased again by immunosuppressive treatment. CONCLUSION: CS +/- CYC was effective for PH associated with SLE. Immunosuppressive treatment should be performed during the early stage of PH to improve prognosis.
OBJECTIVE: To clarify the clinical features of systemic lupus erythematosus (SLE) complicated with pulmonary hypertension (PH) and to evaluate the efficacy of immunosuppressive treatment. METHODS: Case records were reviewed for 194 patients with SLE who were admitted to Aoyama Hospital of Tokyo Women's Medical University between 1992 and 1999. There were 12 patients with PH [8 SLE and 4 SLE + systemic sclerosis (SSc) overlap syndrome]. These patients were compared with 59 age and sex matched patients with SLE for clinical characteristics and laboratory findings. The efficacy of treatments for PH was also evaluated. RESULTS: In our cohort of 194 patients with SLE, 6.2% had PH. The plasma thrombin-antithrombin III complex and plasma D-dimer levels were significantly higher in patients with PH compared with those without PH. Eight patients with PH (4 SLE and 4 SLE + SSc) were treated with corticosteroids (CS) +/- cyclophosphamide (CYC). Right ventricular systolic pressure (RVSP) was improved in 7 of 8 patients. In 6 of 7 responders to the therapy, the treatment was started as soon as they were diagnosed with PH. PH relapsed in 2 patients treated with oral CS +/- CS pulse therapy, but their RVSP was decreased again by immunosuppressive treatment. CONCLUSION: CS +/- CYC was effective for PH associated with SLE. Immunosuppressive treatment should be performed during the early stage of PH to improve prognosis.
Authors: Elisabetta Zanatta; Pamela Polito; Giulia Famoso; Maddalena Larosa; Elena De Zorzi; Elena Scarpieri; Franco Cozzi; Andrea Doria Journal: Exp Biol Med (Maywood) Date: 2019-01-22
Authors: Reda E Girgis; Adaani E Frost; Nicholas S Hill; Evelyn M Horn; David Langleben; Vallerie V McLaughlin; Ronald J Oudiz; Ivan M Robbins; James R Seibold; Shelley Shapiro; Victor F Tapson; Robyn J Barst Journal: Ann Rheum Dis Date: 2007-05-01 Impact factor: 19.103
Authors: Ayse Cefle; Murat Inanc; Mehmet Sayarlioglu; Sevil Kamali; Ahmet Gul; Lale Ocal; Orhan Aral; Meral Konice Journal: Rheumatol Int Date: 2009-12-11 Impact factor: 2.631