Yoshiya Tanaka1, Masataka Kuwana2, Takao Fujii3, Hideto Kameda4, Yoshinao Muro5, Keishi Fujio6, Yasuhiko Itoh7, Hidekata Yasuoka8, Shusaku Fukaya8, Konomi Ashihara8, Daisuke Hirano8, Koichiro Ohmura9, Yuya Tabuchi9, Hisanori Hasegawa10, Ryo Matsumiya3, Yuichiro Shirai2, Takehisa Ogura4, Yumi Tsuchida6, Mariko Ogawa-Momohara5, Hidehiko Narazaki7, Yoshino Inoue1, Ippei Miyagawa1, Kazuhisa Nakano1, Shintaro Hirata11, Masaaki Mori12. 1. The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. 2. Department of Allergy and Rheumatology, Nippon Medical School, Graduate School of Medicine, Tokyo, Japan. 3. Department of Rheumatology and Clinical Immunology, Wakayama Medical University, Wakayama, Japan. 4. Division of Rheumatology, Toho University, Tokyo, Japan. 5. Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya, Japan. 6. Department of Allergy and Rheumatology, Graduation School of Medicine, The University of Tokyo, Tokyo, Japan. 7. Department of Pediatrics, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan. 8. Division of Rheumatology, Department of Internal Medicine, Fujita Health University School of Medicine, Aichi, Japan. 9. Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan. 10. Department of Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan. 11. Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan. 12. Department of Lifetime Clinical Immunology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.
Abstract
OBJECTIVE: To update and revise the diagnostic criteria for mixed connective tissue disease (MCTD) issued by the Japan Research Committee of the Ministry of Health, Labor, and Welfare (MHLW), a round table discussion by experts from rheumatology, dermatology, and pediatric medicine was conducted in multiple occasions. METHODS: The definition of MCTD, and items included in the diagnostic criteria were generated by consensus method and evaluation using clinical data of typical and borderline cases of MCTD, by applying to the diagnostic criteria for MCTD proposed in 1996 and 2004 by the Research Committee of MHLW. RESULTS: To the end, all committee members reached consensus. Then, the criteria were assessed in an independent validation cohort and tested against preexisting criteria. The revised criteria facilitate an understanding of the overall picture of this disease by describing the concept of MCTD, common manifestations, immunological manifestation and characteristic organ involvement. Conditions with characteristic organ involvement include pulmonary arterial hypertension, aseptic meningitis and trigeminal neuropathy. Even if the overlapping manifestations are absent, MCTD can be diagnosed based on the presence of the characteristic organ involvement. Furthermore, the criteria were validated for applicability in actual clinical cases, and public comments were solicited from the Japan College of Rheumatology and other associated societies. CONCLUSION: After being reviewed through public comments, the revised diagnostic criteria have been finalized.
OBJECTIVE: To update and revise the diagnostic criteria for mixed connective tissue disease (MCTD) issued by the Japan Research Committee of the Ministry of Health, Labor, and Welfare (MHLW), a round table discussion by experts from rheumatology, dermatology, and pediatric medicine was conducted in multiple occasions. METHODS: The definition of MCTD, and items included in the diagnostic criteria were generated by consensus method and evaluation using clinical data of typical and borderline cases of MCTD, by applying to the diagnostic criteria for MCTD proposed in 1996 and 2004 by the Research Committee of MHLW. RESULTS: To the end, all committee members reached consensus. Then, the criteria were assessed in an independent validation cohort and tested against preexisting criteria. The revised criteria facilitate an understanding of the overall picture of this disease by describing the concept of MCTD, common manifestations, immunological manifestation and characteristic organ involvement. Conditions with characteristic organ involvement include pulmonary arterial hypertension, aseptic meningitis and trigeminal neuropathy. Even if the overlapping manifestations are absent, MCTD can be diagnosed based on the presence of the characteristic organ involvement. Furthermore, the criteria were validated for applicability in actual clinical cases, and public comments were solicited from the Japan College of Rheumatology and other associated societies. CONCLUSION: After being reviewed through public comments, the revised diagnostic criteria have been finalized.
Authors: Henry S Sacks; Margaret Smirnoff; Deborah Carson; Michael L Cooney; Moshe Z Shapiro; Christopher J Hahn; Christopher R Dasaro; Cynthia Crowson; Ioannis Tassiulas; Robert P Hirten; Benjamin L Cohen; Richard S Haber; Terry F Davies; David M Simpson; Michael A Crane; Denise J Harrison; Benjamin J Luft; Jacqueline M Moline; Iris G Udasin; Andrew C Todd; Nancy L Sloan; Susan L Teitelbaum Journal: Am J Ind Med Date: 2021-11-25 Impact factor: 3.079