title: Could pulmonary arterial hypertension be an active index of systemic lupus erythematosus? A successful case of SLE-PAH cured by methylprednisolone pulse therapy.
abstract: A 35-year-old female patient was diagnosed with pulmonary arterial hypertension (PAH) by right heart catheterization (RHC) in the 3rd month of systemic lupus erythematosus (SLE) duration with high disease activity. Through methylprednisolone pulse therapy, she got total recovery from PAH proven by repeat catheterization and follow-ups. This is the first simultaneously diagnosed, totally recovered, gold standard-proved SLE-PAH case ever reported in the literature. We would like to share this successful case, and at the same time, we want to highlight the relationship between presentation of PAH and active disease manifestations of SLE patients, and to discuss how immune suppressive treatment could benefit this subset of patients.
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.
authors.forename: C
authors.initials: C
authors.lastname: Huang
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.
authors.forename: S
authors.initials: S
authors.lastname: Zhang
authors.affiliation: Department of Cardiology, Peking Union Medical College Hospital, Beijing China.
authors.forename: Z
authors.initials: Z
authors.lastname: Tian
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China mengtao.li@cstar.org.cn.
authors.forename: M
authors.initials: M
authors.lastname: Li
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.
authors.forename: X
authors.initials: X
authors.lastname: Zeng
mesh_terms: D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D062186:Arterial Pressure; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology*; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000188:drug therapy*; D008775:Methylprednisolone / Q000627:therapeutic use; D012720:Severity of Illness Index
chemical_list: D000893:Anti-Inflammatory Agents; D008775:Methylprednisolone
keywords: Pulmonary arterial hypertension; corticosteroid treatment; methylprednisolone pulse therapy; systemic lupus erythematosus