Objective. We have tried to clarify the clinical importance of the measurement of serum type-I interferon (IFN) in patients with anti-melanoma differentiation-associated gene 5 Ab (MDA5 Ab)-positive dermatomyositis (DM). Methods. We studied 30 patients with DM: 10 were anti-MDA5 Ab-positive and 20 were anti-MDA5 Ab-negative. At each patient's initial visit, serum IFN-α, IFN-β, interleukin 18 (IL-18), ferritin, and the titer of anti-MDA5 Ab were measured using enzyme-linked immunosorbent assays (ELISAs). The associations between the IFNs and with the other variables were examined. Results. Rapidly progressive interstitial lung disease (RPILD) was confirmed in 10 patients, most of whom were complicated in the anti-MDA5 Ab-positive DM patients. The presence of clinically amyopathic dermatomyositis (CADM) as well as the serum concentrations of IFN-α and ferritin was significantly higher in the anti-MDA5 Ab-positive DM patients. Serum concentration of IL-18 did not differ between anti-MDA5 Ab-positive and anti-MDA5 Ab-negative groups; however, a positive correlation was found between IFN-α and IL-18 in the anti-MDA5 Ab-positive DM patients (r = 0.8139, p = 0.0146). Conclusion. Serum IFN-α can be used as a useful biomarker in patients with anti-MDA5 Ab-positive DM, which may reflect the presence of RPILD.

What is known and Objective: Tumour necrosis factor‐α (TNF‐α)‐blocking agents are increasingly used in the management of refractory rheumatoid arthritis (RA). Although effective, they are associated with rare but potentially fatal adverse effects, including interstitial lung disease (ILD). In patients with pre‐existing ILD, eternacept (ETN) monotherapy is often regarded as a suitable choice. Other anti‐TNF‐α blockers such as infliximab and adalimumab, are used in combination therapy with methotrexate (MTX) in most of the cases. We report on a case of fatal exacerbation of ILD in a patient given ETN monotherapy and review the literature on ETN‐associated ILD. Methods: We report on a case of a 75‐year‐old male with RA who developed severe ILD after the introduction of ETN, and we undertook a literature search to identify other reports of similar cases. We then critically assessed those reports. Results and Discussion: In addition to our case, 11 other patients have been reported to have developed ILD in association with the use of ETN. Six patients had pre‐existing ILD. Although four patients received MTX, eight patients developed severe ILD without MTX. Ten patients recovered after termination of ETN, although two patients died. What is new and Conclusion: Although ETN is often regarded as safe for patients with ILD, our case and the literature reports suggest that caution is still required.

The aim of the study was to reassess the prevalence and characteristics of human T lymphotropic virus type I (HTLV-I)-associated Sjögren’s syndrome (SS) and SS in HTLV-I-associated myelopathy (HAM) based on the American European Consensus Group (AECG) criteria in HTLV-I endemic area, Nagasaki prefecture. The 349 patients who underwent a minor salivary gland biopsy (MSGB) for suspected SS were retrospectively classified by AECG classification criteria and divided with or without anti-HTLV-I antibody. The HTLV-I data-available 294 patients were investigated. One hundred-seventy patients were classified as SS and 26.5 % were HTLV-I-seropositive. We have included 26 patients with HTLV-I-associated myelopathy (HAM) and 38.5 % were classified as having SS. The prevalences of ANA and anti-SS-A/Ro antibody of HAM + SS were significantly low compared to the HTLV-I asymptomatic carriers (AC) with SS and the HTLV-I-seronegative SS patients, although lacrimal dysfunction tended to be high in HAM + SS and significantly high in AC + SS patients compared with the patients with HTLV-I-seronegative SS. The focus scores of MSGB in the HAM + SS patients were similar to those of the AC + SS patients and the HTLV-I-seronegative patients with SS. Among the MSGB-positive patients, there was a low prevalence of ANA in the HAM + SS patients. Similar results were obtained in case of anti-SS-A/Ro or SS-B/La antibody. In HTLV-I endemic area, high prevalence of anti-HTLV-I antibody among SS as well as the characteristics of HAM + SS and AC + SS was still determined by AECG classification criteria.

A 46-year-old female with a past history of ulcerative colitis (UC) was diagnosed with subacute thyroiditis (SAT), which improved with prednisolone (PSL) treatment (60 mg/day). The dose of PSL was gradually decreased, however upper back and neck pain and chest discomfort developed. The patient was diagnosed with Takayasu's arteritis (TA) based on wall thickening and luminal narrowing of the left common carotid artery and the left subclavian artery. The result of human leukocyte antigen typing analysis was A24 and B52 positive. These findings suggested that common genetic factors may be important for the etiology of TA, UC and SAT. This is the first report of TA that developed following UC and SAT.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a disease entity characterized by systemic vasculitis positive for ANCAs, which often leads to severe organ damage such as diffuse bronchoalveolar hemorrhage and rapidly progressive glomerulonephritis. It is known that the incidence and characteristics of AAV vary depending on region, and differences in the peak age of onset, the ratio of positive rates of MPO-ANCA to PR3-ANCA, and occurrence rates of GPA and MPA may have resulted in different approaches to clinical practice. It may also be necessary to modify therapeutic strategies according to ethnic factors. Avacopan is a therapeutic option recently recommended for the management of AAV; however, the rate of severe liver injuries associated with avacopan was reported to be relatively high in the Japanese population. In this review, we introduce current globally recognized knowledge on the diagnosis and treatment of AAV, including a comparison of patient characteristics and clinical practice between Europe and Japan obtained from the recent literature.

A 63-year-old man with enlargement of the bilateral submandibular glands visited with elevated serum IgG4. A biopsy specimen showed plasma cell infiltration with more than 50% IgG4/IgG staining, suggesting the existence of IgG4-related disease (IgG4-RD). Positive anti-SS-A/Ro antibody and the labial salivary gland's biopsy suggested existence of primary Sjögren's syndrome (pSS). Administration of glucocorticoid improved the serum IgG4 level while reducing the submandibular gland lesions. Positive TUNEL staining suggested the coexistence of IgG4-RD and pSS.