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OBJECTIVE@#Adult-onset Still's disease (AOSD) is a rare but clinically well-known polygenic systemic autoinflammatory disease. In this review, we aim to present frontiers in the pathogenesis, clinical features, diagnosis, biomarkers, disease course, prognosis, and treatment in AOSD.@*DATA SOURCES@#We retrieved information from the PubMed database up to July 2019, using various search terms and relevant words, including AOSD and Still's disease.@*STUDY SELECTION@#We included data from peer-reviewed journals. Both basic and clinical studies were selected.@*RESULTS@#Pathogenesis of AOSD involves genetic background, infectious triggers, and immunopathogenesis, mainly the activation of macrophages and neutrophils followed by a cytokine storm. Diagnosis and prognosis evaluation of AOSD is still challenging; therefore, there is an urgent need to identify better biomarkers. Biologic agents, including interleukin (IL)-1β, IL-6, and tumor necrosis factor-α antagonists in the treatment of AOSD, have good prospect.@*CONCLUSION@#This review highlights the advances in pathogenesis, potential biomarkers, disease course, and treatment in AOSD.
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OBJECTIVE@#Catastrophic antiphospholipid syndrome (CAPS), also known as Asherson's syndrome, is a special subtype of antiphospholipid syndrome (APS) characterized by multiple intravascular thrombosis involving multiple organs systems or tissues simultaneously or continuously, high titer antiphospholipid antibodies and high mortality rate. This article's aims was to analyze the clinical manifestation, laboratory examination and treatment therapy of CAPS for the purpose of improving the understanding, diagnosis and treatment of the disease in clinical practice.@*METHODS@#Retrospective analysis and descriptive statistics were applied to the clinical manifestations and laboratory findings of 14 CAPS cases from APS Shanghai Database (APS-SH) with catastrophic antiphospholipid.@*RESULTS@#Of the 14 CAPS patients, 12 cases satisfied the 2003 CAPS Classification Criteria accepted in the 10th International Congress on Antiphospholipid Antibody, and were diagnosed as definite APS and 2 cases were diagnosed as probable CAPS. Three cases were categorized as primary APS and 11 as APS secondary to systemic lupus erythematosus (SLE). Infection was mostly commonly seen before the onset of CAPS, followed by SLE activity and surgery. Among the involved organs, systems and tissues, brain and lung were most commonly affected sites of arterial thrombosis while peripheral vein was most commonly affected in venous thrombosis events among the clinical events. Triple positivity of anticardiolipin antibody (aCL), anti-β2 glyeoprotein I antibody (aβ2GPI), lupus anticoagulant (LA) were detected in 54.55% of the patients. Thrombocytopenia and decreased hemoglobin were frequently seen in the CAPS patients, and the majority proved to be hemolytic anemia. Of all the cases, 6 ended with death. The triple therapy strategy (anticoagulants, glucocorticoid, intravenous immunoglobulin and/or plasma exchange) could help to improve prognosis, cyclophosphamide and rituximab might benefit the patients with other comorbidities such as SLE and micro-angiopathic hemolytic anemia (MHA).@*CONCLUSION@#CAPS patients suffer from life-threatening acute multiple small vessel thrombosis with high titer of antiphospholipid antibody, potentially leading to multiple organ failure and a poor prognosis, thus early diagnosis and sufficient treatment are critical to prevent the progression of disease and improve the prognosis.