This study demonstrates whether serum β2-microglobulin (β2-MG) level can be an indicator of the status of systemic lupus erythematosus (SLE) and adult-onset Still's disease (AOSD), and development of hemophagocytic syndrome (HPS) complication. Serum β2-MG level was compared between the active and inactive statuses of SLE and AOSD in hospitalized patients. Active status was defined as a state for which a therapy was introduced. Serum β2-MG level was also compared between patients with and without HPS complication. HPS was diagnosed on the basis of clinical and pathological findings. Laboratory markers of HPS including peripheral blood cell counts and levels of serum lactate dehydrogenase (LDH), serum ferritin, plasma fibrin/fibrinogen degradation product (FDP), and plasma D-dimer were examined to determine their correlations with serum β2-MG level. Sixteen SLE and seven AOSD patients (all females, aged 39.0 ± 16.4) were included. The serum β2-MG level was high in the active status of underlying diseases and decreased significantly after the therapy (3.5 ± 1.4 vs. 2.1 ± 0.8 mg/L, p < 0.001). Among patients with active status, the β2-MG level was higher in patients with HPS (two with SLE and three with AOSD) than in patients without HPS (4.9 ± 1.8 vs. 3.3 ± 1.4 mg/L, p < 0.05). Serum β2-MG level significantly correlated with the levels of serum LDH (r(s) = 0.42, p < 0.05), plasma FDP (r(s) = 0.58, p < 0.05), and plasma D-dimer (r(s) = 0.77, p < 0.01). Serum β2-MG level would be a useful indicator of disease activity and development of HPS complication in patients with SLE and AOSD.

IgG4-related pericardial involvement has rarely been reported and its clinical features remain unknown. We herein report a case of a 50-year-old woman with pericarditis who presented with a fever, elevated C-reactive protein levels, elevated serum IgG4 concentrations, and thickened pericardium with a patchy 18 Ffluorodeoxyglucose (FDG) uptake. A biopsy specimen of 18 F-FDG accumulated in the mediastinal lymph nodes revealed an abundant infiltration of IgG4-bearing plasma cells without fibrosis. Moderate-dose glucocorticoids promptly resolved the physical, serological, and imaging abnormalities, thus indicating a relatively acute and reversible nature of IgG4-related pericardial involvement.

This trial is the first-in-man clinical trial to demonstrate that BCAA supplementation might be safe and, at least in part, improve skeletal muscle mass, strength, and function in patients with rheumatic disorders treated with GC.

This paper aims to study the background and clinical characteristics of tacrolimus (TAC)-induced lung disease. A case of a rheumatoid arthritis (RA) patient who developed TAC-induced interstitial lung disease (TAC-ILD) is reported. The Japanese Pharmaceuticals and Medical Devices Agency (PMDA) website was searched for cases of TAC-ILD and its prevalence among all cases of TAC-related adverse events. As for cases of TAC-ILD, its underlying disease, preexisting lung diseases, and fatal outcome were also searched. Literature review of TAC-ILD cases was added. A 65-year-old female RA patient with preexisting bronchiectasis developed near-fatal TAC-ILD. Amelioration of RA, ground-glass opacities in the upper, anterior, and central lung fields, and decrease in peripheral blood lymphocyte count were the major findings in this patient. A search of the PMDA website revealed the following: the prevalence of TAC-ILD was 3 % of all cases of TAC-related adverse events, 56 out of 85 RA cases (66 %), and one out of 15 other cases had a preexisting lung disease; the prevalences of fatal outcome in RA and other cases were 24 and 38 %, respectively. A few cases in the literature had preexisting ILD and developed diffuse alveolar damage. In our case, preexisting bronchiectasis, arthritis remission, newly developed ground-glass opacities (GGOs) in the upper, anterior, and central lung fields, and decrease in peripheral blood lymphocyte count were the major findings. From the search of the PMDA website, about one fourth of the cases with TAC-related lung injury had a fatal outcome, and among RA patients, two thirds had preexisting lung diseases.

No abstract

A 65-year-old Japanese woman, who was diagnosed with rheumatoid arthritis and Sjögren's syndrome with various autoantibodies including anti-DNA antibody, developed bullous pemphigoid (BP) and hematological abnormalities like lupus erythematosus after adalimumab therapy. The discontinuation of adalimumab resolved those disorders but polyarthritis thereafter relapsed. The introduction of abatacept was not effective, but tocilizumab was found to be effective for polyarthritis, however, thereafter both bullous disease and severe pancytopenia developed. Discontinuation of tocilizumab was effective, but polyarthritis again developed, and baricitinib resolved it. There is an increasing number of reports of drug-induced BP and lupus erythematosus, and biologics might trigger an alteration in the pathophysiological/clinical course of rheumatic disorder.

Catastrophic antiphospholipid syndrome (CAPS) survivors rarely relapse. We herein report a case of a second CAPS episode with an unusual subacute course and no microangiopathic hemolytic anemia (MAHA), a common CAPS symptom. During the first episode, the 69-year-old woman responded well to high-dose glucocorticoids and plasma exchange. On relapse, these treatments plus rituximab were ineffective and she died of multi-organ failure and bacterial cholangitis. The absence of MAHA and a subacute course do not exclude a CAPS recurrence.

Background Peripheral circulation is often disturbed in patients with connective tissue diseases (CTDs). Raynaud phenomenon (RP) is prevalent in and considerably characteristic of CTDs and we have long noticed colour unevenness among fingers in patients during RP attacks. We hypothesized that temperature unevenness among fingers detected by infrared thermography would be a useful parameter to evaluate peripheral circulation. Objectives To evaluate temperature unevenness among fingers as a thermographic parameter by comparing it with other parameters validated in previous studies [1-3]. Methods Patients with RP who underwent thermographic examinations at our hospital were included and compared with healthy volunteers. Skin temperatures of dorsum of hands at 10 fingers' nail folds and MCP joints were measured at baseline. Then hands were immersed in 10°C water for 10 seconds. Skin temperatures were measured at 0, 3, 5, 10, 15, 20, and 30 min after immersion. Mean temperature, recovery rate (temperature recovery from immersion/decrease by immersion), and coefficient of variation (standard deviation/mean temperature) of nail fold temperature were calculated. Higher coefficient of variation means temperature among fingers is more uneven. Distal-dorsal difference (DDD: measured by subtracting mean temperature of MCP from that of nail fold) was also calculated and these parameters were compared between the two groups. Receiver operating characteristic (ROC) curve was generated to compare these parameters in terms of their capability of differentiating patients with RP from HCs. Results Thirty-one patients with RP (10 with primary Raynaud, 11 with systemic sclerosis (SSc), 11 with other CTDs) were included and compared with 25 healthy controls (HCs). Baseline nail fold temperature was significantly lower in patients of RP than in HC (30.8±3.1°C vs. 33.2±1.8°C, p=0.0002). Cold-water immersion of hands revealed patients with RP had lower recovery rate, lower DDD, and higher coefficient of variation than did HCs. The differences in these parameters were the most evident at 5 min after immersion (patients with RP vs HCs: recovery rate; 49.6±27.7 vs 71.5±26.8, p=0.004 DDD; -1.4±2.8 vs 0.85±2.7, p=0.0008 coefficient of variation; 0.053±0.024 vs 0.021±0.015, p=1.2x10–6). On the basis of ROC curve analyses for these parameters, coefficient of variation of nail fold temperature most effectively differentiated patients with RP from HCs (Area under the curve; recovery rate: 0.64 DDD: 0.79 coefficient of variation: 0.88). Conclusions Unevenness of temperature among fingers was the most useful thermographic parameter to evaluate disturbed peripheral circulation. References O'Reilly D, et al. (1992) Measurement of cold challenge responses in primary Raynaud's phenomenon and Raynaud's phenomenon associated with systemic sclerosis. Ann Rheum Dis 51: 1193-1196. Anderson ME, et al. (2007) The 'distal-dorsal difference': a thermographic parameter by which to differentiate between primary and secondary Raynaud's phenomenon. Rhe...