A clinical analysis of 13 patients with scleroderma renal crisis and pulmonary arterial hypertension / 中华风湿病学杂志

Objective:To describe a series of systemic sclerosis (SSc) patients with the combination of scleroderma renal crisis (SRC) and pulmonary arterial hypertension (PAH).Methods:The medical records of 472 SSc patients in Peking Union Medical College Hospital between January 2012 and October 2020 were reviewed and a retrospective analysis of the characteristics of patients with SRC and PAH among SSc patients was conducted.Results:Thirteen patients suffered from SRC and PAH in the SSc patients, 1 case was limited cutaneous SSc, and 12 cases were diffuse cutaneous SSc. Five patients had renal crisis before pulmonary arterial hypertension, 4 patients had pulmonary arterial hypertension before the occurrence of renal crisis, and the remaining 4 patients were found at the same time. Among them, 11 patients had Raynaud's phenomenon, 7 had gastrointestinal bleeding, 6 had pulmonary edema and 3 had telangiectasias. Twelve cases were positive for anti-nuclear antibodies and 4 cases were positive for anti-Scl-70 antibodies. N-terminal pro-brain natriuretic peptide (NT-proBNP)>1 400 ng/L in 11 patients. Two patients had thrombotic microangiopathy (TMA). Among the 13 patients, 3 patients died during hospitalization, 2 patients were lost to follow-up, and 2 patients died within 5 years of follow-up. Six patients survived, and 1 of the 4 patients with regular dialysis were discharged from dialysis.Conclusion:In patients with scleroderma, SRC can occur earlier, later than, or at the same time with SSc-PAH. Patients may have a higher incidence of gastrointestinal bleeding and higher level of NT-proBNP. PDE5i or ERAs may be beneficial.

Progress on pathogenesis and treatment of adult-onset Still disease / 中华全科医师杂志

Adult-onset Still disease (AOSD) is a polygenic autoinflammatory disease with uncertain etiology.The clinical manifestations are complex,including high-spiking fever,rash,arthritis,arthralgia,lymphadenopathy,and leukocytosis.The pathogenesis of AOSD is still unclear,whereas it has been confirmed that many inflammatory cells and cytokines take part in the development of AOSD.The treatment of AOSD is mainly based on corticosteroids and immunosuppressive agents.Recently,with the research progress of autoinflammatory diseases and the emerging biological agents,we have new knowledge on the pathogenesis and therapeutic strategies of AOSD.This article reviews the current progress in pathogenesis and therapy of AOSD.