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The objective of this study was to describe the clinical and laboratory characteristics, precipitating factors, treatment, and outcome of macrophage activation syndrome (MAS) complicating systemic lupus erythematosus (SLE). A multicenter case-control study was performed across six tertiary hospitals from 1997 to 2014. A total of 32 patients with SLE-associated MAS were enrolled. Sixty-four age- and sex-matched SLE patients diagnosed in the same period without MAS episodes were selected as controls. The most frequent clinical feature was fever, followed by splenomegaly. Hyperferritinemia, hypoalbuminemia, and hyper-lactate dehydrogenase (LDH)-nemia were among the most common laboratory abnormalities. Compared with pre-MAS visit, patients at the onset of MAS had greater frequencies of renal involvement, liver dysfunction, and cytopenia. Receiver operating characteristic (ROC) analysis identified optimal cutoff values of ferritin (>662.5 ng/mL) and LDH (>359 U/mL) to predict the occurrence of MAS in SLE. SLE flare and infection were the common triggers of MAS in SLE. Abortion and parturition were recorded as well. The overall mortality rate was 12.5%. All patients received corticosteroids. Cyclosporine A, cyclophosphamide, and etoposide were the three most commonly used immunosuppressants. Rituximab was given to one patient. Intravenous immunoglobulin (IVIG) was added for 46.9% patients. MAS is a potentially fatal complication of SLE. Its occurrence is most frequently associated with active SLE disease or infection. The presentation of unexplained fever, cytopenia, or liver dysfunction, with high levels of ferritin and LDH, in patients with SLE should raise the suspicion of MAS. Corticosteroids with immunosuppressants and IVIG may be an appropriate treatment.

Objective: To analyze the clinical features and prognosis of cardiovascular complications of diffuse connective tissue disease(dCTD)., Methods: Clinical data of cardiovascular complications of 181 cases of dCTD were retrospectively analyzed. Clinical data of two subsets [rheumatoid arthritis (RA, 81) and systemic lupus erythematosus (SLE, 42)] were also analyzed. Follow-up was carried out for all the patients., Results: RA and SLE were the most common disease complicated by cardiovascular disease needing hospitalization. The most frequent cardiovascular complications in dCTD patients were hypertension, hyperlipidemia and coronary artery disease. The most common echocardiographic abnormalities were valvular regurgitation, left ventricular diastolic dysfunction, enlargement of left atrium, pulmonary hypertension and pericardial effusion (57.8%, 50.6%, 33.7%, 21.7% and 19.3%, respectively). Compared with the subset of RA, patients were younger at onset of hypertension, coronary artery disease and hyperlipidemia in the subset of SLE [(40+/-11) vs (56+/-15), P<0.001; (53+/-12) vs (64+/-10), P=0.011; (44+/-16) vs (58+/-12), P=0.012, respectively]. Both pericardial effusion (P<0.001) and enlargement of left ventricle (P=0.03) were more frequent, and left ventricular diastolic dysfunction was less common (P<0.001). Median survival time of these dCTD patients was 9.8 years., Conclusion: RA and SLE are the most common diffuse connective tissue diseases complicated by cardiovascular disease needing hospitalization. The most frequent cardiovascular complications in dCTD patients are hypertension, hyperlipidemia and coronary artery disease. The prognosis of dCTD patients complicated with cardiovascular diseases is poor. SLE patients are younger at onset of cardiovascular diseases.