Yasuhiro Katsumata

We aimed to validate the reliability of the Medical Outcomes Study Short Form-36 (SF-36) among Japanese patients with systemic lupus erythematosus (SLE). Japanese patients with SLE (n = 233) completed the SF-36 and other related demographic questionnaires, and physicians simultaneously completed the SLE Disease Activity Index 2000 (SLEDAI-2K) and the Systemic Lupus International Collaborating Clinics Damage Index (SDI). Patients were prospectively followed for a repeat assessment the following year. The SF-36 subscales demonstrated acceptable internal consistency (Cronbach’s α of 0.85–0.89), and an overall good test–retest reliability (intraclass correlation coefficient >0.70). The average baseline SF-36 subscale/summary scores except for “bodily pain” were significantly lower than those of the Japanese general population (p < 0.05). The SDI showed an inverse correlation with the SF-36 subscale/summary scores except for “vitality” and “mental component summary” at baseline, whereas the SLEDAI-2K did not. In the second year, “social functioning” and “mental component summary” of the SF-36 deteriorated among patients whose SDI or SLEDAI-2K score increased (effect sizes < −0.20). In conclusion, the SF-36 demonstrated acceptable reliability among Japanese patients with SLE. Health-related quality of life measured by the SF-36 was reduced in Japanese patients with SLE and associated with disease damage, rather than disease activity.

The Systemic Lupus Activity Questionnaire (SLAQ) is a patient-reported outcome for systemic lupus erythematosus (SLE). We aimed to translate it into Japanese and further investigate its validity and reliability. The English version of the SLAQ was translated into Japanese and administered to Japanese SLE patients at our university clinic. Physicians assessed disease activity using the SLE Disease Activity Index 2000 (SLEDAI-2K). The patients were prospectively followed for repeat assessment a year later. Ultimately, 255 patients participated. The patients’ 10-point ratings of disease activity and SLAQ scores were significantly correlated (Spearman’s ρ = 0.53). The SLAQ score was weakly correlated with the SLE Disease Activity Index 2000 (SLEDAI-2K)-nolab (omitting laboratory items; ρ = 0.18) but not with the SLEDAI-2K (ρ = 0.02). These results suggested its convergent and discriminant validity. The SLAQ demonstrated acceptable internal consistency (Cronbach’s α = 0.80), and good test–retest reliability (intraclass correlation coefficient = 0.85). The effect sizes and the standardized response means of the SLAQ were as follows: clinical worsening, 0.26 and 0.31, and improvement, −0.39 and −0.41, respectively, which indicated a small but significant responsiveness. The Japanese version of the SLAQ demonstrated acceptable reliability and validity; its performance was comparable to that of the original version.

The association between interstitial lung disease (ILD) and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), particularly microscopic polyangiitis (MPA), has been described in a number of case reports and case series reports in the last 2 decades. In addition, patients with pulmonary fibrosis and ANCA positivity but without other manifestations of systemic vasculitis have also been reported. Pulmonary fibrosis was clinically manifested at the time of diagnosis in the majority of AAV patients that developed this condition. Moreover, ANCA-positive conversion occurs in patients initially diagnosed with idiopathic pulmonary fibrosis, and as a result, other manifestations of systemic vasculitis develop in some of these patients. There is significant predominance of myeloperoxidase (MPO)-ANCA and MPA in patients with AAV and ILD. Radiological and pathological findings generally demonstrate usual interstitial pneumonia (pattern) in the lungs of these patients. In most studies, AAV patients with ILD have a worse prognosis than those without it.

The diagnosis of macrophage activation syndrome (MAS) in patients with systemic lupus erythematosus (SLE) may be challenging as it can mimic the clinical features of the underlying disease or be confused with an infectious complication. In this report, a Japanese woman in her forties had diverse clinical features of MAS at initial presentation of SLE, where erythematosus plaques with histiocytic infiltrates focally surrounding degenerated collagen might be the earliest indicator of MAS.

Idiopathic inflammatory myopathies (IIMs) are a group of inflammatory muscle disorders of unknown etiology; these include polymyositis (PM), dermatomyositis (DM), and inclusion body myositis. Extra-muscular manifestations such as dermatitis, arthritis, interstitial lung disease (ILD), cardiomyopathy, and enteropathy are occasional complications in patients with PM/DM. Several myositis-specific autoantibodies (MSAs) have been discovered in IIMs; these can help predict clinical characteristics, response to treatment, and prognosis. For example, anti-aminoacyl-tRNA synthetase (ARS) antibodies, including Jo-1 antibody (Ab) and anti-melanoma differentiation-associated gene 5 (MDA-5) Ab, have been associated with the manifestation of ILD in PM and DM. Anti-MDA5 Ab-associated ILD has a 1-year survival rate of 50-60%; however, short-term prognosis is relatively good in anti-ARS Ab-associated ILD. Fatal outcome occurs remarkably often within the first 6 months of anti-MDA5 Ab-associated ILD. Therefore, intensive treatment should be administered to patients harboring anti-MDA-5 Ab or showing hyperferritinemia in ILD with DM. In addition, corticosteroid occasionally induces myopathy, which is an issue arising in PM/DM treatment. Some experts recommend combination therapy of corticosteroid and an immunosuppressive agent as a first-line treatment for myositis in PM/DM. Methotrexate and azathioprine are commonly used immunosuppressive agents for myositis in western countries. Immunosuppressive agents are steroid-sparing, serving to mitigate corticosteroid-related side effects, thus making combination therapy an effective treatment option. Preventing the progression of physical dysfunction is of prime importance to patients with PM/DM. Dermatologists, neurologists, and rheumatologists should therefore work together to care for these patients before muscular and extra-muscular involvement develop progressively and irreversibly.

Objectives: We assessed the association between serum autoantibodies against the 70-kDa polypeptide of the U1-ribonucleoprotein (RNP) complex (U1-70k) and the central nervous system (CNS) syndromes in systemic lupus erythematosus (SLE) patients. Methods: We studied 106 hospitalized patients with active SLE, comparing those with (n = 32) and without (n = 74) CNS syndromes. CNS syndromes were further classified into neurologic (n = 21) and psychiatric (n = 15) disorders. Immunoglobulin G (IgG) anti-U1-70k antibodies were measured by enzyme-linked immunosorbent assay (ELISA) using recombinant antigens. IgG antibodies against whole U1-RNP were measured using commercial ELISA kits. Results: Although there was no significant difference in the levels of serum anti-U1-70k antibodies in SLE patients with or without CNS syndromes (p = 0.83), the levels were significantly elevated in SLE patients compared with patients without psychiatric syndromes (p = 0.030). In contrast, no significant difference was observed in the levels of serum anti-U1-RNP antibodies in SLE patients with or without psychiatric syndromes (p = 0.555). Conclusions: These results indicate that serum anti-U1-70k antibodies are associated with psychiatric syndromes in SLE but that they are not associated with CNS syndromes as a whole or with neurologic syndromes. The anti-U1-70k antibodies might be involved in the pathological mechanisms of psychiatric syndromes in SLE.

Objectives To assess the association between serum aquaporin-4 (AQP4) autoantibodies and neuromyelitis optica spectrum disorders (NMOSDs) associated with systemic autoimmune diseases.Methods We ret...

Background: The risk for amenorrhea following treatment of systemic lupus erythematosus (SLE) patients with low-dose intravenous cyclophosphamide (IVCY) has not been fully explored. Our objective was to ascertain the incidence of amenorrhea following treatment with low-dose IVCY and the association between amenorrhea and the clinical parameters of SLE. Methods: A case-control retrospective study of premenopausal women ≤ 45 years old who had been treated for SLE with low-dose IVCY (500 mg/body/pulse) plus high-dose glucocorticoids (0.8-1.0 mg/kg/day of prednisolone; IVCY group) or glucocorticoids alone (0.8-1.0 mg/kg/day of prednisolone; steroid group) in our hospital from 2000 through 2009 was conducted using a questionnaire survey and medical record review. Results: Twenty-nine subjects in the IVCY group and 33 subjects in the steroid group returned the questionnaire. A multivariate analysis revealed that age at initiation of treatment ≥ 40 years old was significantly associated with amenorrhea [p = 0.009; odds ratio (OR) 10.2; 95% confidence interval (CI) 1.8-58.7]. IVCY treatment may display a trend for association with amenorrhea (p = 0.07; OR 2.9; 95% CI 0.9-9.4). Sustained amenorrhea developed in 4 subjects in the IVCY group and 1 subject in the steroid group; all of these patients were ≥ 40 years old. Menses resumed in all subjects < 40 years old, irrespective of treatment. Conclusions: Although low-dose IVCY may increase the risk for amenorrhea, our data suggest that patients < 40 years old have a minimum risk for sustained amenorrhea with low-dose IVCY treatment. A higher risk for sustained amenorrhea following treatment with IVCY is a consideration for patients ≥ 40 years old.