Assessment of 2012 EULAR/ACR new classification criteria for polymyalgia rheumatica in Japanese patients diagnosed using Bird's criteria.

AIM: The 2012 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria for polymyalgia rheumatica (PMR) were published in 2012. The present study aimed to assess the 2012 EULAR/ACR classification criteria for PMR in Japanese patients diagnosed with PMR using Bird's criteria. METHODS: The study included 75 patients diagnosed using Bird's criteria. The patients were divided into fulfilled and not-fulfilled groups according to whether they met the 2012 EULAR/ACR classification criteria for PMR. The factors in the new criteria were morning stiffness duration > 45 min, hip pain or limited range of motion, absence of rheumatoid factor or anti-citrullinated protein antibody, and absence of other joint involvement. RESULTS: Thirty-two of the patients diagnosed with PMR using Bird's criteria met the new EULAR/ACR classification criteria, while the remaining 43 patients did not meet the new criteria. Among the factors, only morning stiffness duration > 45 min was an independent predictive factor. CONCLUSIONS: A morning stiffness duration > 45 min is the only independent predictive factor for differentiating patients diagnosed according to the new 2012 EULAR/ACR classification criteria for PMR.

Reliability and clinical utility of Enzyme-linked immunosorbent assay for detection of anti-aminoacyl-tRNA synthetase antibody.

Anti-aminoacyl-tRNA synthetase (ARS) antibody is one of the myositis-specific autoantibodies to make a diagnosis of polymyositis (PM) and dermatomyositis (DM). Recently a new enzyme-linked immunosorbent assay (ELISA) kit of concurrently detected anti-ARS antibodies (anti-Jo-1, anti-PL-7, anti-PL-12, anti-EJ and anti-KS) have become to measure in the clinical setting. To evaluate the reliability of this ELISA kit, we measured anti-ARS antibodies in 75 PM and DM patients using by this ELISA assay and compared them with the results by RNA immunoprecipitation assay. Between the measurements of anti-PL-7, anti-PL-12, anti-EJ and anti-KS autoantibodies by ELISA assay and RNA-IP assay, the concordance rate of reproducibility is 95.1% and the positive agreement rate is 90.9% and negative agreement rate is 96.0% and kappa statistic is 0.841. Between the measurements of existing anti-Jo-1 antibody ELISA kit and anti-ARS antibody ELISA kit, the concordance rate of reproducibility is 96.9%, the positive agreement rate is 100%, negative agreement rate is 96.1% and kappa statistic is 0.909. The lung involvement in patients with PM and DM patients are positive of anti-ARS antibodies and anti-melanoma differentiation associated gene5 (MDA5) antibody at a rate around 70%. Then most life-threatening ILD with anti-MDA5 positive clinically amyopathic dermatomyositis patients could be highly guessed when anti-ARS antibodies are negative.

A case of familial Mediterranean fever who complained of periodic fever and abdominal pain diagnosed by MEFV gene analysis.

Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease caused by Mediterranean FeVergene (MEFV) mutations on Chromosome 16, and characterized by periodic fever of and serositis. FMF is the result of gain-of-function mutations in pyrin that lead to interleukin-1ß activation. FMF can be classified as "typical" and "atypical" types based on clinical finding and genetic screening. Although MEFV genotyping has enabled FMF to be confirmed in some cases, the diagnosis remains predominantly clinical since genotyping has shown that the disease is characterized by variable manifestations in Japanese. In 1976, the first report performed on the case of Japanese FMF with periodic fever of and serositis. Since 2002, genetic analyses are performed on Japanese FMF patients by K. Shiozaki et al. and N. Tomiyama et al. In our case, she was a 25-year-old Japanese woman with at periodic fever and abdominal pain. MEFV gene analysis demonstrated a heterozygous mutation of variant M694I, leading to a diagnosis of FMF. After the increase dose (up to 3 mg/day) of colchicine, periodic fever and abdominal pain disappeared. It is the important candidate of FMF for differential diagnosis with unexplained periodic fever and serositis, such as our case.

[A case of HELLP syndrome at 34w-pregnancy with systemic lupus erythematosus and antiphospholipid antibody syndrome;Importance of measurement of VW factor].

SUMMARY: A 39-year-old woman was diagnosed with Systemic lupus erthymatosus (SLE) in 1993, and initially received 30 mg of prednisolone (PSL) daily as treatment. In 2012, the patient was diagnosed with pregnancy-induced hypertension (PIH) complecated with proteinurea, hypertension and pretibial edema at 24 weeks of gestation. At onset, protein urea was 1.6 g/day and she was given bed rest in the hospital with a protein-restricted and low salt diet, which led to a decrease in protein urea to approximately 1 g/day. At 34 weeks of gestation epigastric pain developed, and laboratory examinations showed liver dysfunction and low platelets. We made a diagnosis of hemolysis, elevated liver enzymes, low platelet (HELLP) syndrome and performed an emergency cesarean procedure. Thereafter blood pressure was elevated, protein urea was 3.2 g/daily, anti-ds-DNA antibody level was elevated and serum C3/C4/CH50 was reduced, thus we gave. plasma exchange therapy, along with immunoadsorption and steroid pulse therapy (methyl-prednisolone 500 mg/daily for 3 days), as well as PSL at 30 mg/day. Overtime clinical symptoms and laboratory data gradually improved. CONCLUSION: Some reports suggest that SLE during pregnancy is a risk factor for hypertension, nephritis, SLE relapse and HELLP syndrome. In the patient, ADAMTS13 activity did not decrease, while there was an increase in VW factor level. We assessed this case was as atypical thrombotic microangiopathy. And herein report HELLP syndrome during pregnancy associated with SLE in our patient.