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Biological disease-modifying antirheumatic drugs (bDMARDs) are highly effective agents for the treatment of inflammatory arthritis; however, they also possess a potential risk for serious infection. Recently, with the rapid expansion of the bDMARDs market in Korea, reports of serious adverse events related to the agents have also increased, necessitating guidance for the use of bDMARDs. Current work entitled, “Expert Consensus for the Use of bDMARDs Drugs for Inflammatory Arthritis in Korea,” is the first to describe the appropriate use of bDMARDs in the management of inflammatory arthritis in Korea, with an aim to provide guidance for the local medical community to improve the quality of clinical care. Twelve consensus statements regarding the use of bDMARDs for the management of rheumatoid arthritis and ankylosing spondylitis were generated. In this review, we provide detailed guidance on bDMARDs use based on expert consensus, including who should prescribe, the role of education, indications for use, and monitoring strategies for safety. (J Rheum Dis 2020;27:4-21)

Background/Aims: This study was undertaken to perform a cross-cultural adaptation of the Behcet’s Disease Current Activity Form (BDCAF, version 2006) questionnaire to the Korean language and to evaluate its reliability and validity in a population of Korean patients with Behcet’s disease (BD). Methods: A cross-cultural study was conducted among patients with BD who attended our rheumatology clinic between November 2012 and March 2013. There were 11 males and 35 females in the group. The mean age of the participants was 48.5 years and the mean disease duration was 6.4 years. The first BDCAF ques tionnaire was completed on arrival and the second assessment was performed 20 minutes later by a different physician. The test-retest reliability was analyzed by computing κ statistics. Kappa scores of > 0.6 indicated a good agreement. To assess the validity, we compared the total BDCAF score with the patient’s/clinician’s perception of disease activity and the Korean version of the Behcet’s Disease Quality of Life (BDQOL). Results: For the test-retest reliability, good agreements were achieved on items such as headache, oral/genital ulceration, erythema, skin pustules, arthralgia, nausea/vomiting/abdominal pain, and diarrhea with altered/frank blood per rectum. Moderate agreement was observed for eye and nervous system involvement. We achieved a fair agreement for arthritis and major vessel involvement. Signifi cant correlations were obtained between the total BDCAF score with the BDQOL and the patient’s/clinician’s perception of disease activity (p < 0.05). Conclusions: The Korean version of the BDCAF is a reliable and valid instrument for measuring current disease activity in Korean BD patients.

Background/Aims The clinical manifestations of Behcet disease (BD) have been reported to differ according to country, region, and race. Gender, onset age, and human leukocyte antigen (HLA)-B51 have also been known as the factors that influence the clinical features of BD. The aim of this study is to investigate the clinical phenotypes of Korean patients who visited the rheumatology clinic with BD with respect to gender, onset age, and HLA-B51. Methods Total 193 Korean patients (129 females and 64 males) fulfilling the international criteria for BD were retrospectively assessed. Results The mean age at disease onset and disease duration of the BD patients were 32.2 ± 11.1 and 14.2 ± 9.3 years, retrospectively. Females suffered more frequently from genital ulcers (90.7% vs. 75.0%, p < 0.01), peripheral arthritis (67.4% vs. 43.8%, p < 0.01), and inf lammatory low back pain (38.8% vs. 23.4%, p = 0.03) than males, while skin involvement was more frequent in males than in females (90.6% vs. 75.2%, p = 0.01). The patients with late onset of BD (> 40 years) suffered from neurologic involvement (15.9% vs. 4.2%, p = 0.007) more frequently than those with early onset of BD. The patients with HLA-B51 showed earlier onset of disease than without HLA-B51 (28.3 ± 11.4 years vs. 33.8±11.6 years, p = 0.02) and the neurologic and gastrointestinal involvements were more frequent in the patients without HLA-B51 than with HLA-B51 (17.2% vs. 2.5%, p = 0.02 and 20.7% vs. 2.5%, p = 0.01, respectively). Conclusions The clinical phenotypes in Korean patients with BD may be influenced by gender, onset age and HLA-B51.

Background/Aims: We prepared a cross-cultural adaptation of the Behcet’s Syndrome Activity Scale (BSAS) and evaluated its reliability and validity in Korea. Methods: Fifty patients with Behcet’s disease (BD) who attended the Rheumatology Clinic of Gachon University Gil Medical Center were included in this study. The first BSAS questionnaire was administered at each clinic visit, and the sec ond questionnaire was completed at home within 24 hours of the visit. A Behcet’s Disease Current Activity Form (BDCAF) and a Behcet’s Disease Quality of Life (BDQOL) form were also given to patients. The test-retest reliability was analyzed by intraclass correlation coefficients (ICC). To assess the validity, the total BSAS score was compared with the BDCAF score, the patient/physician global assessment, and the BDQOL by Spearman rank correlation. Results: Twelve males and 38 females were enrolled. The mean age was 48.5 years and the mean disease duration was 6.7 years. Thirty-eight patients (76.0%) returned the questionnaire by mail. For the test-retest reliability, the two assessments were significantly correlated on all 10 items of the BSAS questionnaire (p < 0.05) and the total BSAS score (ICC, 0.925; p < 0.001). The total BSAS score was statistically correlated with the BDQOL, BDCAF, and patient/physician global assessment (p < 0.01). Conclusions: The Korean version of BSAS is a reliable and valid instrument to measure BD activity.

Muscle involvement in acute febrile neutrophilic dermatosis is uncommon. Herein, we report a case of acute febrile neutrophilic myositis, without cutaneous involvement, as the first manifestation of acute myeloid leukemia. The patient was a 35-year-old male, referred due to painful swelling of the left upper arm and fever. The overlying skin looked normal, and a muscle biopsy revealed dense infiltrates, predominantly composed of mature neutrophils, edema and tissue necrosis. All culture reports were negative, and he was finally diagnosed as having acute febrile neutrophilic myositis, associated with acute myeloid leukemia. Corticosteroid treatment resulted in the progressive regression of the fever, myalgia and swelling.