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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. [ABSTRACT FROM AUTHOR]

Objective: To explore the influence of chemokine, CXCL16, on the expression of the receptor activator nuclear factor κB ligand (RANKL) in rheumatoid arthritis (RA) fibroblast-like synoviocytes (RA-FLS). Methods: The expression of CXCL16/CXCR6 and RANKL in RA or osteoarthritis (OA) patient synovia was examined by Western blot and immunohistochemistry. The serum concentration of CXCL16 and RANKL was measured by enzyme-linked immunosorbent assay (ELISA). RA-FLS were treated with recombinant CXCL16, and RANKL mRNA and protein were measured using PCR, Western blot and ELISA. Results: The synovial expression of CXCL16, CXCR6, and RANKL was higher in RA patients than in patients with OA. The serum CXCL16 and RANKL levels were higher in RA patients compared with OA patients and healthy controls. CXCL16 correlated with erythrocyte sedimentation rate, C reactive protein, disease activity, serum rheumatoid factor, and RANKL. RA-FLS treated with CXCL16 showed markedly increased expression of RANKL. When STAT3 or p38 activation was blocked by an inhibitor, CXCL16 failed to upregulate RANKL expression. In contrast, inhibiting the Akt or Erk pathway did not achieve the same effect. Conclusions: CXCL16 upregulates RANKL expression in RA-FLS and these effects are mainly mediated by the JAK2/STAT3 and p38/MAPK signaling pathways. [ABSTRACT FROM AUTHOR]

To identify the magnetic resonance imaging (MRI) features of hands and wrists in early rheumatoid arthritis (RA). A total of 129 early arthritis patients (≤1 year) were enrolled in the study. At presentation, MRI of the hands was performed, with clinical and laboratory analyses. After a 1-year follow-up, clinical diagnosis of early RA or non-RA was confirmed by two rheumatologists. The characteristics of MRI variables at baseline in RA patients not fulfilling ACR 1987 criteria [RA-87(−)] were compared with those fulfilling ACR1987 criteria [RA-87(+)] and non-RA. In the 129 early arthritis patients, 90 were diagnosed with RA in a 1-year follow-up. There were 47.8 % (43/90) of the RA patients not fulfilling ACR 1987 criteria [RA-87(−)]. The scores of synovitis in RA-87(−) patients were similar with those in RA-87(+) [Synovitis score, 14.0 (IQR, 4.0-25.0) vs. 14.0 (IQR, 10.0-25.0), p > 0.05]. Compared with those in non-RA, RA-87(−) patients had higher synovitis scores and occurrence of synovitis in proximal interphalangeal (PIP) joints [synovitis score, 14.0 (IQR, 4.0-25.0) vs. 6.0 (IQR, 2.0-14.5), p = 0.046; occurrence of PIP synovitis: 53.5 vs. 27.3 %, p = 0.02]. There was no significant difference of bone marrow edema, bone erosion, and tenosynovitis between RA-87(−) and non-RA. Synovitis in PIP joints was independent predictor for RA-87(−) [OR, 3.1 (95 %CI 1.2-8.1)]. High synovitis scores and synovitis in PIP joints on MRI were important in early RA, especially those not fulfilling ACR 1987 criteria. [ABSTRACT FROM AUTHOR]

Endogenous nitric oxide synthase (eNOS) inhibitor asymmetric dimethylarginine (ADMA) is a cardiovascular risk factor. We tested the hypothesis that L-citrulline may ameliorate the endothelial function altered by ADMA in porcine coronary artery (PCA). Myograph study for vasorelaxation, electrochemical measurement for NO, RT-PCR, and Western blot analysis for expression of eNOS, argininosuccinate synthetase (ASS), and p-eNOSser1177 were performed. cGMP was determined by enzyme immunoassay. Superoxide anion (O2.−) production was detected by the lucigenin-enhanced chemiluminescence method. Compare with controls (96.03% ± 6.2%), the maximal relaxation induced by bradykinin was significantly attenuated (61.55% ± 4.8%, p < 0.01), and significantly restored by L-citrulline (82.67 ± 6.4%, p < 0.05) after 24 hours of ADMA exposure. Expression of eNOS, p-eNOSser1177, and ASS in PCA significantly increased after L-citrulline incubation. L-citrulline also markedly restored the NO production, and cGMP level which was reduced by ADMA. The increased O2.− production by ADMA was also inhibited by L-citrulline. L-citrulline restores the endothelial function in preparations treated with ADMA by preservation of NO production and suppression of O2.− generation. Preservation of NO is attributed to the upregulation of eNOS expression along with activation of p-eNOSser1177. L-citrulline improves endothelium-dependent vasodilation through NO/ cGMP pathway. [ABSTRACT FROM AUTHOR]