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Objectives: Using an expert- and data-driven methodology, we have constructed the first clinical practice guidelines (CPGs) for adult Still's disease (ASD) after complete systematic review (SR) of the literature based upon the Medical Information Network Distribution Service (Minds) procedure. Methods: The CPG committee for ASD organized by the Research Team for Autoimmune Diseases, the Research Program for Intractable Disease of the Japanese Ministry of Health, Labour, and Welfare has developed CPG for ASD 2017, according to the procedure proposed by Minds. The CPG development process includes (1) clarification of the purpose of CPG, (2) organization of the steering committee, (3) organization of the CPG committee and secretariat, (4) defining the scope (setting of clinical questions (CQs)), (5) SR, (6) development of recommendations, (7) drafting the CPG, (8) external evaluation and public comments, and (9) release. Because we wanted to construct CPG for ASD to encompass both adult-onset Still's disease (AOSD) and adult patients with systemic juvenile idiopathic arthritis (sJIA), we also included SR data from sJIA in this study. Results: Twenty-six CQs were selected and roughly divided into the following items: (1) clinical findings (CQs 1-4), (2) laboratory findings (CQs 5-8), (3) complications (CQs 9-13), (4) treatment with oral medicine (CQs 14-19), (5) treatment with biological reagents (CQs 20-23), and (6) treatments for sJIA (CQs 25-26). Recommendations and the strength of the recommendations for these CQs were decided by a modified Delphi method. Conclusion: We have developed the first published CPG for ASD including AOSD and sJIA, which includes 26 CQs and recommendations. This guideline will help rheumatologists, non-specialized physicians, other healthcare providers, medical and health-related students, and patients and their family members to understand and treat ASD. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the performance of the 2012 Systemic Lupus International Collaborating Clinics criteria (SLICC-12) on classifying systemic lupus erythematosus (SLE) in an uncontrolled multi-centered study with real-life scenario of the patients in Japan. Methods: This study comprised 495 patients with SLE or non-SLE rheumatic diseases and allied conditions from 12 institutes in Japan. Chart review of each patient was performed by the 27 expert rheumatologists and diagnosis of 487 cases reached to the consensus. Value of the SLICC-12 on SLE classification was analyzed comparing with the 1997 revised American College of Rheumatology SLE classification criteria (ACR-97) employing the expert-consented diagnoses. Results: Compared to the ACR-97, the SLICC-12 had a higher sensitivity (ACR-97 vs. SLICC-12: 0.88 vs. 0.99, p<.01) and comparable specificity (0.85 vs. 0.80). The rate of misclassification (0.14 vs. 0.11) or the area under the receiver operating characteristic curves (0.863 vs. 0.894) was not statistically different. In the cases that diagnoses corresponded in high rates among experts, both criteria showed high accordance of SLE classification over 85% with the expert diagnoses. Conclusion: Although employment of SLICC-12 for the classification for SLE should be carefully considered, the SLICC-12 showed the higher sensitivity on classifying SLE in Japanese population. [ABSTRACT FROM AUTHOR]

We report a case of interstitial cystitis (IC) associated with primary Sj ö gren's syndrome (SS) successfully controlled with combination therapy of tacrolimus and a corticosteroid. In 2011, a 69-year-old female, who had been diagnosed with primary SS 23 years ago, developed IC and was successfully treated with tacrolimus and prednisolone combination therapy. The mechanism of IC, including the involved autoimmunity, has not been elucidated. Clinical observation studies suggest a potential association between SS and IC. However, IC is currently thought to be underdiagnosed in patients with SS as well as in the general population. Based on our case and others reported previously, IC associated with SS responds well to immunosuppressive therapy. In particular, a combination of a calcineurin inhibitor (tacrolimus or cyclosporine) with a corticosteroid seems to be highly effective. The possibility of IC in patients with SS complaining of lower urinary tract symptoms without features of infection or other identifiable causes should be given attention. [ABSTRACT FROM AUTHOR]

To evaluate the eff ects of tocilizumab (TCZ) on adult-onset Still's disease (AOSD), we reviewed medical records of seven patients with refractory AOSD treated with TCZ at our institution. TCZ therapy might allow rapid corticosteroid tapering and help maintain remission status, that is, resolution of clinical symptoms and normalization of biomarkers such as CRP and ferritin. Patients, however, should be monitored for the development of macrophage activation syndrome when TCZ is administered for active AOSD. [ABSTRACT FROM AUTHOR]

Résumé Objectifs Cette étude avait pour but d’évaluer les techniques d’imagerie, incluant la 18F-fluorodeoxyglucose – tomographie par émission de positons/scan (FDG-TEP/scan), dans les maladies à immunoglobuline IgG4 (AL-IgG4). Méthodes Nous avons revu huit patients atteints d’AL-IgG4, qui ont été orientés vers notre hôpital de 2006 à 2012. Tous les patients avaient bénéficié d’un FDG-TEP/scan, d’une IRM ainsi que d’une échographie endobronchique, réalisés respectivement dans cinq cas et un cas. Résultats Bien que presque tous les patients atteints d’AL-IgG4 avaient une CRP négative (moyenne 0,22 mg/dL), plusieurs atteintes organiques ont été détectées via le FDG-TEP/scan. Dans la phase active d’une pancréatite auto-immune dans deux cas, le FDG-TEP/scan avait montré une accumulation hétérogène et longitudinale de FDG au niveau du pancréas associée à une accumulation au niveau hilaire et médiastinal. Le suivi par FDG-TEP/scan après traitement dans un cas avait montré que l’accumulation de FDG avait complètement disparu. Dans deux cas, l’IRM cérébrale avait montré une pachyméningite hypertrophique asymptomatique. Dans un cas, l’échographie endobronchique des ganglions médiastinaux avait montré un gonflement en rapport avec des vaisseaux tortueux, associé à un signal Doppler intense et des cordons hyperéchogènes entre les ganglions lymphatiques. Conclusions Lorsque le FDG-TEP/scan montre une accumulation de FDG, caractéristique de l’AL-IgG4 au niveau des organes, sans signes inflammatoires associés sur le plan biologique, le diagnostic d’AL-IgG4 peut être posé. L’efficacité thérapeutique peut être évaluée à travers la disparition de la fixation du FDG. Une IRM cérébrale est utile afin de détecter la pachyméningite hypertrophique asymptomatique. L’échographie endobronchique pourrait être utile dans la différenciation entre les étiologies des adénopathies avec des caractéristiques échographiques typiques. [ABSTRACT FROM AUTHOR]

Objectives: This study aimed to evaluate the utility of imaging techniques, including 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT), in immunoglobulin (IgG)4-related disease (IgG4-RD). Methods: We reviewed eight IgG4-RD patients who were referred to our hospital between August 2006 and April 2012. All cases underwent FDG-PET/CT and brain magnetic resonance imaging (MRI) and endobronchial ultrasonography (EBUS) were also performed in five cases and one case, respectively. Results: Although nearly all patients with IgG4-RD in this study were negative for CRP (mean 0.22mg/dL), various organ involvement sites were detected by FDG-PET/CT. In the active phase in two autoimmune pancreatitis (AIP) cases, FDG-PET/CT showed longitudinal and heterogeneous FDG accumulation in the pancreas with FDG uptake in the hilar or mediastinal lymph nodes. Follow-up FDG-PET/CT after therapy in one case revealed that the abnormal FDG uptake in all affected lesions had completely disappeared. In two cases, brain MRI revealed asymptomatic hypertrophic pachymeningitis. In one case, EBUS imaging of mediastinal lymph node swelling was consistent with tortuous vessels with high Doppler signals and hyperechoic strands between lymph nodes. Conclusions: When FDG-PET/CT shows FDG accumulation, characteristic of IgG4-RD in organs, without evidence of an associated inflammatory reaction, a diagnosis of IgG4-RD can be made. Treatment effects can be assessed by the disappearance of FDG uptake. A routine brain MRI is useful for detecting asymptomatic hypertrophic pachymeningitis. EBUS may also be useful for differentiating among the etiologies of lymphadenopathy with characteristic sonographic imaging findings. [ABSTRACT FROM AUTHOR]