Both serum ferritin and anti-MDA5 antibody are powerful indicators for the early diagnosis of A/SIP with DM. Ferritin also predicts disease severity and prognosis for patients with anti-MDA5 antibody. Intensive treatment should be administered to cases that have anti-MDA5 antibody-associated ILD with DM showing hyperferritinaemia, especially if the ferritin level is>or=1600 ng/ml.

Anti-MDA5ab titre and ferritin and IL-18 concentrations are useful for the evaluation of the response to treatment and the status of ILD in patients with anti-MAD5ab-positive DM.

IL-6, IL-8, TNF-α and IP-10 are associated with global disease activity in PM/DM. These cytokine levels were high, especially in the ILD subset. Serum IL-8 levels and the balance between IL-4 and IFN-γ may contribute to the differences in pathophysiology between anti-ARS-ILD and anti-MDA5-ILD.

Objective. Previous case series have examined the relationship between anti-Jo-1 antibody levels and myositis disease activity, demonstrating equivocal results. Using enzyme-linked immunosorbent assays (ELISAs) and novel measures of myositis disease activity, the current study was undertaken to systematically reexamine the association between anti-Jo-1 antibody levels and various disease manifestations of myositis.Methods. Serum anti-Jo-1 antibody levels were quantified using 2 independent ELISA methods, while disease activity was retrospectively graded using the Myositis Disease Activity Assessment Tool, which measures disease activity in 7 different organ systems via the Myositis Disease Activity Assessment Visual Analog Scale (VAS) and the Myositis Intention-to-Treat Index (MITAX) components. Spearman's rank correlation coefficients and mixed linear regression analysis were used to identify associations between anti-Jo-1 antibody levels and organ-specific disease activity in crosssectional and longitudinal analyses, respectively.Results. Cross-sectional assessment of 81 patients with anti-Jo-1 antibody revealed a modest correlation between the anti-Jo-1 antibody level and the serum creatine kinase (CK) level, as well as muscle and joint disease activity. Correlation coefficients were similar for CK levels (r s ‫؍‬ 0.38, P ‫؍‬ 0.002), myositis VAS (r s ‫؍‬ 0.36, P ‫؍‬ 0.002), and arthritis VAS (r s ‫؍‬ 0.40, P ‫؍‬ 0.001). In multiple regression analyses of 11 patients with serial samples, anti-Jo-1 antibody levels correlated significantly with CK levels (R 2 ‫؍‬ 0.65, P ‫؍‬ 0.0002), myositis VAS (R 2 ‫؍‬ 0.53, P ‫؍‬ 0.0008), arthritis VAS (R 2 ‫؍‬ 0.53, P ‫؍‬ 0.006), pulmonary VAS (R 2 ‫؍‬ 0.69, P ‫؍‬ 0.005), global VAS (R 2 ‫؍‬ 0.63, P ‫؍‬ 0.002), and global MITAX (R 2 ‫؍‬ 0.64, P ‫؍‬ 0.0003).Conclusion. In this large series of patients with idiopathic inflammatory myopathy, anti-Jo-1 antibody levels correlated modestly with muscle and joint disease, an association confirmed by a custom ELISA using recombinant human Jo-1. More striking associations emerged in a smaller longitudinal subset of patients that link anti-Jo-1 antibody levels to muscle, joint, lung, and global disease activity.

Serum ferritin can be useful as a predictor of the occurrence of A/SIP and correlates with the prognosis of A/SIP in DM. The intensive treatment using combination therapy with various immunosuppressant agents should be chosen for patients with ILD with DM showing hyperferritinaemia, especially levels >1500 ng/ml.

The aim of this study was to evaluate neurological manifestations of primary Sjögren’s syndrome (pSS) and investigate the etiology and pathogenesis of peripheral and central nervous complications in pSS. Thirty-two patients with pSS were enrolled in the present study, 20 of whom had neurological involvement plus sicca symptoms. The clinical features were evaluated by neurological examinations including nerve conduction study, magnetic resonance imaging, cerebrospinal fluid, and electroencephalogram. The frequency of fever was significantly higher (P = 0.006) in pSS with neurological involvement than in pSS without neurological involvement. There was no statistical significance in other factors between the two groups. Peripheral nervous system (PNS), central nervous system (CNS), and both PNS and CNS involvements were revealed in 14, 3, and 3 patients, respectively. Optic neuritis and trigeminal neuralgia were revealed frequently in cranial neuropathy. Anti-aquaporin 4 antibody was detected in one patient with optic neuritis. Of the nine patients with polyneuropathy, eight patients presented pure sensory neuropathy including small fiber neuropathy (SFN). pSS with SFN appeared to have no clinically abnormal features, including muscle weakness and decreasing deep tendon reflex. Skin biopsy revealed epidermal nerve fiber degenerated in one pSS patient with pure sensory neuropathy who was diagnosed as having SFN. Our observations suggest that a number of mechanisms can be attributed to neurological involvements in pSS rather than just the mechanisms previously described (i.e., vasculitis and ganglioneuronitis). Presumably, specific autoantibodies may directly induce injury of the nervous system.

Objective Develop response criteria for juvenile dermatomyositis (JDM). Methods We analyzed the performance of 312 definitions that used core set measures (CSM) from either the International Myositis Assessment and Clinical Studies Group (IMACS) or the Pediatric Rheumatology International Trials Organization (PRINTO) and were derived from natural history data and a conjoint-analysis survey. They were further validated in the PRINTO trial of prednisone alone compared to prednisone with methotrexate or cyclosporine and the Rituximab in Myositis trial. Experts considered 14 top-performing candidate criteria based on their performance characteristics and clinical face validity using nominal group technique at a consensus conference. Results Consensus was reached for a conjoint analysis–based continuous model with a Total Improvement Score of 0-100, using absolute percent change in CSM with thresholds for minimal (≥30 points), moderate (≥45), and major improvement (≥70). The same criteria were chosen for adult dermatomyositis/polymyositis with differing thresholds for improvement. The sensitivity and specificity were 89% and 91-98% for minimal, 92-94% and 94-99% for moderate, and 91-98% and 85-85% for major improvement, respectively, in JDM patient cohorts using the IMACS and PRINTO CSM. These criteria were validated in the PRINTO trial for differentiating between treatment arms for minimal and moderate improvement (P=0.009–0.057) and in the Rituximab trial for significantly differentiating the physician rating of improvement (P<0.006). Conclusion The response criteria for JDM was a conjoint analysis–based model using a continuous improvement score based on absolute percent change in CSM, with thresholds for minimal, moderate, and major improvement.

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