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Objectives The incidence of femoral localized periosteal thickening (LPT), which can precede atypical femoral fracture (AFF), is not low (1–10%) in Japanese patients with autoimmune inflammatory rheumatic diseases (AIRDs). We explored the associations between underlying AIRDs and the prevalence of LPT. Methods We conducted post hoc analyses of two cohorts that included a total of 280 Japanese women, 105 of whom had AIRDs and had been taking bisphosphonate (BP) and prednisolone (PSL) and 175 of whom had rheumatoid arthritis (RA). Results LPT was detected in a total of 18 patients (6.4%) and 3 (1.1%) developed AFFs. RA was negatively correlated with LPT. A disease other than RA requiring glucocorticoid treatment, BP use ≥5 years, PSL use ≥7 years, and a PSL dose ≥5.5 mg/day were positively correlated with LPT. After adjusting for age, diabetes mellitus, and BP duration or daily PSL dose, RA was no longer associated with LPT. Conclusions LPT in Japanese patients with AIRDs was associated with BP and glucocorticoid treatment rather than underlying AIRDs. When PSL dose ≥5.5 mg/day is required long-term [typically combined with long-term BP treatment (≥5 years)], clinicians need to pay particular attention in cases LPT and AFF as well as glucocorticoid-induced osteoporosis.

Although biological disease-modifying antirheumatic disease drugs (bDMARDs) have represented a paradigm shift in the treatment of rheumatoid arthritis (RA), their high cost is a burden for patients...

Objectives: The aim of this study was to identify the risk factors associated with severe infection in RA patients, with a particular focus on the association of the nutritional status.Methods: We ...

We describe a 53-year-old woman with primary Sjögren's syndrome and tubulointerstitial nephritis showing distal renal tubular acidosis and Fanconi syndrome. The patient showed high serum IgM levels and positivity for antimitochondrial antibodies, although her liver function was in normal range. According to our literature review, 75% of patients with tubulointerstitial nephritis who were positive for antimitochondrial antibodies showed Fanconi syndrome, suggesting that these antibodies may directly be associated with the pathophysiology of Fanconi syndrome.

The intensification of infliximab (IFX) treatment, involving escalation of the dose and shortening of interval, was approved in Japan in July 2009. We consider IFX intensification therapy to be preferable for patients with treatment-resistant active rheumatoid arthritis (RA). We retrospectively compared the efficacy of IFX with that of other bDMARDs in methotrexate (MTX)-resistant patients.Patients who satisfied the following criteria were enrolled: (i) those who started bDMARDs between February 2011 and December 2016, and (ii) those who required bDMARDs after 180 d of MTX treatment. We compared 33 patients who had been treated with IFX (IFX group) and 146 who had received other bDMARDs treatment (non-IFX group).IFX was administered at a dose of 6.98 mg/kg/8-week equivalent at 52 weeks. Clinical disease activity index clinical remission (CDAI-CR) was achieved in 49 of the 179 patients at 52 weeks and 13 of these 49 patients received IFX. Logistic regression analysis showed that treatment with IFX was an important variable for the achievement of CDAI-CR at 52 weeks (odds ratio 2.69, 95% confidence interval 1.13-6.42). The severity and frequency of adverse events did not differ.Intensification of IFX was effective and well tolerated for MTX resistant patients.

We describe a 20-year-old female with systemic lupus erythematosus (SLE) who developed renal failure during continuous ambulatory peritoneal dialysis (CAPD). The patient was treated with mizoribine (MZR). MZR peak concentration was 1.8 mug/ml and was sufficient for clinical efficacy as measured by serological data. Treatment with MZR was safe and useful even while undergoing CAPD. Achieving optimal MZR blood concentration was important for treatment of SLE, even though the patient was in end-stage renal failure.

To investigate the precise modality of association between SAA1 gene polymorphisms and the development of AA amyloidosis in patients with rheumatoid arthritis (RA), Japanese patients with RA (n = 153), among whom 29 were histologically diagnosed as having amyloidosis, were genotyped for three single nucleotide polymorphisms (SNPs), C-13T, C2995T, and C3010T, in the SAA gene. Pairwise linkage disequilibrium coefficients between each pair of SNPs were calculated and estimated haplotype frequencies were compared between patients with and without amyloidosis. Possible associations between these SNPs and amyloidosis were analyzed by a case–control study and by the Kaplan–Meier method, in which the endpoint was defined as the time of diagnosis of AA amyloidosis. The -13T and 2995C alleles, which were in a tight linkage disequilibrium, were more frequent in the patients with amyloidosis, and the groups with the -13TT and 2995CC genotype had worse survival curves than patients without these genotypes, whereas C3010T was not associated with amyloidosis. Moreover, the haplotype containing −13C and 2995T was found to be protective. Both C-13T and C2995T were associated with the development of amyloidosis. Examining both polymorphisms may be more useful than examining only one of them for estimating the risk of the development of amyloidosis.

A 36-year-old woman was diagnosed with systemic lupus erythematosus (SLE). Seven days after beginning glucocorticoid treatment, she developed reduced visual acuity, and atypical severe central serous chorioretinopathy (CSC) was confirmed. Since glucocorticoid use is an important risk factor for CSC, the PSL was reduced, tacrolimus was added, and the visual acuity improved rapidly. Reduction in glucocorticoid combined with the use of immunosuppressive agents is one option for preventing a deterioration in atypical severe CSC while still controlling SLE.

We report an intriguing case of Epstein- Barr virus (EBV)-related multiple lymphadenopathy that clinically mimics immunoglobulin G4-related disease (IgG4-RD). A 72-year-old woman presented with a history of asthma attacks, systemic lymphadenopathy, hypergam- maglobulinemia, proteinuria, and an elevated level of serum IgG4, leading to a possible diagnosis of IgG4-RD based on current comprehensive diagnostic criteria. How- ever, a percutaneous kidney biopsy specimen showed mild mesangial proliferative glomerulonephritis with focal membranous transformation, and there was no interstitial lesion or lymphocyte infiltration. Cervical lymph node biopsy demonstrated follicular hyperplasia associated with prominent lymphoplasmacytic infiltration in the interfol- licular area. However, only a few IgG4-positive plasma cells were present. An in situ hybridization study demon- strated many EBV-infected lymphocytes in the germinal center as well as in the interfollicular area. This case illustrates the diversity of conditions associated with ele- vated levels of serum IgG4 and the necessity for tissue biopsy when diagnosing IgG4-RD.

We report a case of orbital tumor and hypertrophic cranial pachymeningitis in a 64-year-old woman, who was initially suspected to have IgG4-related disease because of an elevated level of serum IgG4 at onset. However, her condition was resistant to glucocorticoid therapy, and additional cyclophosphamide was necessary to control the disease activity. Additional features included elevated levels of serum myeloperoxidase (MPO) and proteinase-3-anti-neutrophil cytoplasmic antibody (PR3-ANCA), and biopsy specimens from the orbital mass revealed very few infiltrating IgG4-positive cells. Instead, rupture of the elastic layer of the arterial walls with neovascularization and a small number of giant cells were observed. Considering these findings and the clinical course, the disease was considered more likely to be ANCA-associated pachymeningitis with elevation of the serum IgG4 level.

Anti-neutrophil cytoplasmic autoantibodies against bactericidal/permeability-increasing protein (BPI-ANCA) are known to be present in patients with cystic fibrosis, diffuse panbronchiolitis, and inflammatory bowel disease, especially in relation to chronic Gram-negative bacterial infection. To investigate the possible role of BPI-ANCA in rheumatoid arthritis (RA), we measured the serum titer of BPI-ANCA and examined clinical manifestations, including pulmonary complications, in patients with RA. Seventy-four RA patients were recruited to our study. The titer of BPI-ANCA was measured by enzyme-linked immunosorbent assay (ELISA). Pulmonary complications were evaluated using high-resolution computed tomography (HRCT), which revealed 26 patients with bronchial diseases (BD group), 25 with interstitial pneumonia (IP group), and 23 without any particular lung lesion (normal group). The correlations between the titer of BPI-ANCA and patients’ clinical and laboratory findings were then analyzed. The numbers of tender joints, swollen joints, and the Disease Activity Score including 28 joint count were significantly higher in the BD group. The titer of BPI-ANCA was positively correlated with age, erythrocyte sedimentation rate (ESR), and bronchial involvement in all subjects. Stepwise multiple regression analysis of factors affecting the titer of BPI-ANCA selected ESR and bronchial involvement as independent variables. Our results show that BPI-ANCA was positively correlated with chronic inflammatory status in RA patients, and we is believe it is positively linked with bronchial diseases.