A case of SLE-PAH（systemic lupus erythematosus associated pulmonary arterial hypertension）which accepted improvement by combination of the pulmonary vasodilator without succeeding for existing immunosuppressive treatment

Abstract

  A 49-year-old woman was diagnosed with Systemic lupus erthymatosus（SLE）at 27 years old. She was diagnosed with type V lupus nephritis by kidney biopsy at 39 years old, and received 7.5mg of prednisolone（PSL）and 50mg of cyclosporine as treatment. She was hospitalized due to effort dyspnea in May, 2016. Her mean pulmonary arterial pressure was 51.3mmHg, NYHA/WHO pulmonary hypertension functional classification was class Ⅲ. It was found to be highly unlikely that she had chronic thromboembolic pulmonary hypertension by testing of lung perfusion scintigraphy, so we diagnosed her clinical condition was pulmonary artery hypertension caused by SLE. First, we prescribed her a steroid pulse, a cyclophosphamide pulse, prednisolone 1mg/kg, and 50mg of cyclosporine, then started to use pulmonary vasodilator together. Her mean pulmonary arterial pressure significantly was improved after this treatment. It was reported that collagenosis PAH had good steroid and immunosuppressant reactiveness, but this case wasn’t responsive to the treatment, and was responsive to use with pulmonary vasodilator. By case report, most of the SLE-PAH cases were responsive to precociously use pulmonary vasodilator together have low SLEDAI. I thought that using pulmonary vasodilator together is necessary for SLE-PAH cases which have low SLEDAI.