Juvenile dermatomyositis (JDM) is a collagen vascular disease characterized by typical rashes and proximal muscle weakness. Although JDM shares several similarities with adult DM in both pathophysiology and clinical features, complication of ulcerative lesions as a result of severe vasculopathy and calcification are more common in JDM than DM. Other features of JDM include a low frequency of anti-ARS antibodies, a lack of relationship between malignancy and anti-TIF1γ antibodies. Because rapidly progressive-interstitial lung disease (RP-ILD) is a major cause of death in JDM, we carried out a nation-wide collaborative study on this complication. We found that, similar to adult cases, anti-MDA5 antibodies are associated with RP-ILD. RP-ILD was associated with high serum levels of IL-18 and ferritin. Furthermore, we found a correlation between IL-18 and KL-6 levels, suggesting involvement of alveolar macrophages in the development of RP-ILD associated with JDM. On the other hand, there was no relationship between anti-MDA5 antibodies and RP-ILD in Caucasian, suggesting that the antibodies play different pathological roles between races.

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[Introduction] With the development of biologics (BIO) for rheumatoid arthritis (RA), clinical remission has become a treatment goal and functional remission, the most important aim. Our hospital uses the Health Assessment Questionnaire-Disability Index (HAQ-DI) for functional assessment; however, the HAQ-DI does not allow the complete assessment of housework in female RA patients.
[Objectives] To assess housework among female RA patients receiving BIO who are in functional remission.
[Subjects] Thirty-eight female RA patients receiving BIO whose HAQ-DI scores were ≤0.5 and disease activity scores (DAS28-ESR) indicated remission or low disease activity.
[Methods] A proprietary questionnaire on housework (cooking, washing, and cleaning) was administered. The HAQ-DI was used as a reference. Satisfaction regarding housework was assessed using the visual analog scale (VAS).
[Results] The mean age of the patients was 57.7 years and mean HAQ-DI score was 0.18. Of the 38 patients, 30 were in clinical remission and 8 had low disease activity. The lowest scores were for “lifting pots and frying pans with food” in cooking, “putting clothespins” in washing, and “airing out the futon” in cleaning. The mean VAS score for cleaning satisfaction was 2.84, and this was lower compared to those for the other forms of housework.
[Discussion] We found that female RA patients in functional remission feel that housework is inconvenient and are dissatisfied. A better understanding of inconveniences in housework and collaboration with therapists to create approaches for improving the quality of housework, such as functional recovery through rehabilitation and use of support tools, are needed.

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    Objective: The purpose of this study was to investigate the efficacy of switching to CZP for patients with remaining multiple swollen joints despite serologic inflammation being improved after treatment with biologic DMARDs.
    Methods: We studied 11 patients with multiple swollen joints (number of swollen joints ≧2) and whose serum CRP level < 1 mg/dl after treatment with other biologic DMARDs. Assessment of disease activity were evaluated using DAS28 (ESR), DAS28 (CRP), SDAI, and the number of swollen and tender joints.
    Results: Moderate or better improvement by EULAR response criteria were observed in 73% of cases. Although there was trend toward lower scores of DAS28 (ESR), DAS28 (CRP), and SDAI at 12 weeks after switching compared with those at baseline, these differences did not reach statistical significance. The number of swollen and tender joints decreased in 80% and 64% of all the patients. However, there was a statistically significant difference only in the numbers of swollen joints.
    Conclusion: Switching to CZP may be effective in patients with multiple swollen joint even though serological inflammation was low after treatment with biologic DMARDs.

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Objective: Recently we presented the induction failure of Eternercept 50mg solution for injection in pre-filled pens in about half of the patients (8/17). Then we investigated the reasons for induction-failure. Hand grip weakness (≦130mm Hg in mercurial grip dynamometer), pressure force weakness (≦100mg) and severe finger deformities were found to be the reasons.
    In turn, we evaluated the availability of TCZAI which used the same injection type. Method. We started TCZAI treatment for 8 RA patients and investigated their availability.
Results: All the patients could be induced and continued self-injection despite having hand grip weakness (130mg≦, n=4), pressure force weakness (100mg≦, n=4), and severe fingers deformity (n=5).
Conclusion: TCZAI has excellent usability due to the large area of skin contact and thick injection button by comparison with other autoinjectors of this type.

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[Objectives] In the treatment of RA with biologics, patientʼs care, prevention of infection are very important. This study aimed to clarify the role of pharmacist in patient care with RA. [Methods] We executed the research for outpatient care RA 64 person in our hospital from March to May in 2013. We used questionnaire sheet about daily activity, medication of RA, and psychological state. [Results] 64 patients (12 males and 52 females, age average: 60.3, duration: 9 years, DAS28: 2.64, CRP: 0.54mg/dl) were enrolled and 30% was influence of daily activity after onset of RA. About MTX, 86% of patients could understand the effect of MTX, and answered inconvenience of medication for once of a week. About biologics, over 67% of the patients felt non-stress and over 62% of patients felt the biologics effect. The some patients were anxious about side effect and inconvenience of biologics. [Conclusion] This finding suggest the importance of Pharmacist full understanding the psychological and financial of patients in order to keep the motivation of medication.

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    A 70-year-old woman had high fever for 21 days accompanied by erythema concentrated on extremity, polyarthralgia with bone erosion and serum ferritin was 4450 ng/ml, whereas rheumatoid factor and anti-cyclic citrullinated peptide antibody were negative. We diagnosed as AOSD, initially she was treated with high dose corticosteroids (1 mg/kg/day, prednisone 40 mg) and her symptom were improve. We thought AOSD flare with decreasing the dose of prednisone to 25 mg, when her symptom got worse as pyrexia, arthralgia, and elevation of inflammatory reaction. She was treated to add on tacrolimus (TAC), then it got improved for one week. Finally, we discontinued steroid without relapse AOSD, adding TAC made it possible for steroid tapering. We need to think that TAC is regarded as one of the choice of treatment for AOSD in case of difficulty toward steroid sparing.

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    In recent years, it has been reported that tocilizumab is effective in adult onset Still’s disease (AOSD). We report a case of severe AOSD where tocilizumab was effective and contributed to cessation of steroid treatment. A 43 year-old female patient suffering from fever, skin rash and arthralgia was diagnosed as having AOSD. Despite treatment with high-dose prednisolone, its response was poor and the patient suffered the complications of disseminated intravascular coagulation (DIC) and acute kidney injury (AKI). After disease activity improved by plasma exchange, cyclosporine and cyclophosphamide, tocilizumab was introduced with the aim of maintaining remission. Thereafter, disease activity stayed low even after cessation of prednisolone and cyclosporine. The significance of tocilizumab as the maintenance therapy is discussed along with previous reports.

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    Selective IgM deficiency (SIgMD) is a rare immunodeficiency disease with a reported prevalence of 0.03%. Most SIgMD in adult people is found in patients with malignant disorders or autoimmune diseases. We report a case of selective IgM deficiency associated with systemic lupus erythematosus (SLE). A 61-year-old female was admitted with severe cough and fever. There was no history of recurrent infection, and there was no family history of immunodeficiency or SLE. Computed tomography scan of the chest revealed interstitial infiltrates. Laboratory findings revealed pancytopenia, proteinuria and tests for anti-nuclear antibody and anti-DNA antibody were positive. Her serum IgM level was very low, lower than 7 mg/ml, while her IgA and IgG level were rather high. Renal biopsy showed ISN class V lupus nephritis, so she was diagnosed as SLE associated with SIgMD. She was treated with a pulse therapy of 500mg metylpredonisolone, followed by daily prednisolone (40 mg/day) and tacrolimus (2 mg/day), which was proved to be effective.

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    A 22-year-old female was hospitalized with pyrexia and multiple arthralgia as main complaints and was then diagnosed systemic erythematosus. This case was lupus nephritis ISN/PRS classification ⅢA/C type with a high level of SLEDAI; 29 points. We considered that the disease activity was high and thus conducted steroid half-pulse therapy, 3 days of mPSL 500 mg/day intravenous infusion. Although the symptoms were alleviated temporarily, because it recurred during post-therapy with PSL 40 mg, multi-target therapy combined with tacrolimus and mizoribine was initiated. Because the effective blood concentration of tacrolimus didn’t increase, the drug was changed to cyclosporine, resulting in rapid increase in the effective blood concentration and notably alleviated symptoms. The gene polymorphisms of drug-metabolic enzymes were thus measured and it was then found that it was CYP3A5*1/*1 whose blood concentration didn’t increase easily. Thus, On Day 64 of the hospitalization, the patient was discharged to home and has had favorable course since then. This is a case that the measurement of gene polymorphisms of drug metabolic enzymes in selection of immunosuppressant was effective. As gene polymorphisms may be measured conveniently and rapidly in recent years, it may be a useful method to predeict the efficacy of a drug and may lead to an efficacious decision of immunosuppressant agent.

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