Yoshitaka Yamanouchi1, Michiru Sawahata2, Noritaka Sakamoto3, Shu Hisata4, Noriharu Shijubo5, Satoshi Konno6, Tetsuo Yamaguchi7, Meri Watanabe8, Hidetoshi Kawashima9, Takuji Suzuki10, Masashi Bando11, Koichi Hagiwara12. 1. Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, Shimotsuke, Japan. Electronic address: m05101yy@jichi.ac.jp. 2. Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, Shimotsuke, Japan. Electronic address: sawahata@jichi.ac.jp. 3. Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, Shimotsuke, Japan. Electronic address: nori.smoto@gmail.com. 4. Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, Shimotsuke, Japan. Electronic address: hisata@jichi.ac.jp. 5. Department of Respiratory Medicine, JR Sapporo Hospital, Sapporo, Japan. Electronic address: n-sijubo@jrhokkaido.co.jp. 6. Department of Respiratory Medicine, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan. Electronic address: satkonno@pop.med.hokudai.ac.jp. 7. Shinjuku Tsurukame Clinic, Tokyo, Japan. Electronic address: yamatet@icloud.com. 8. Department of Ophthalmology, Jichi Medical University, Shimotsuke, Japan. Electronic address: r1015ms@jichi.ac.jp. 9. Department of Ophthalmology, Jichi Medical University, Shimotsuke, Japan. Electronic address: hidemeak@khaki.plala.or.jp. 10. Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, Shimotsuke, Japan. Electronic address: suzutaku@jichi.ac.jp. 11. Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, Shimotsuke, Japan. Electronic address: bando034@jichi.ac.jp. 12. Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, Shimotsuke, Japan. Electronic address: hagiwark@me.com.
Abstract
BACKGROUND: The presence of histologically evident epithelioid granuloma is required for the diagnosis of sarcoidosis worldwide. The Japan Society of Sarcoidosis and Other Granulomatous Disorders 2015 diagnostic criteria (JSSOG 2015 criteria) includes "clinically proven diagnosis" (involvement of at least 2 of 3 systems confirmed solely by clinical assessment) because of the frequency of sarcoidosis with ocular, cardiac, and respiratory involvement in Japan and the difficulty of obtaining specimens. Here, we describe in detail the clinical presentation of clinically diagnosed sarcoidosis. METHODS: We enrolled 68 consecutive patients with clinically diagnosed sarcoidosis (12 men, 56 women) based on the JSSOG 2015 criteria who were treated at Jichi Medical University between December 2018 and January 2000. We analyzed age at diagnosis, organ involvement, and laboratory findings. RESULTS: Age at diagnosis was unimodal in women. Ocular, splenic, cardiac, and skin involvement, and hypercalcemia were observed in 95.6%, 8.8%, 7.4%, 5.9%, and 35.0% of patients, respectively. High serum lysozyme and soluble interleukin-2 receptor (sIL-2R) levels, bilateral hilar lymphadenopathy on chest radiography, high-grade atrioventricular block or fatal ventricular arrhythmia, and bundle branch block were found in 18.8%, 48.3%, 95.6%, 5.0%, and 10.0% of patients, respectively. CONCLUSIONS: The age-specific distribution of clinically diagnosed sarcoidosis was similar to histologically diagnosed sarcoidosis in women, as previously reported. Rates of elevated serum lysozyme and sIL-2R levels were lower in this study than previously reported in histologically diagnosed patients in Japan.
BACKGROUND: The presence of histologically evident epithelioid granuloma is required for the diagnosis of sarcoidosis worldwide. The Japan Society of Sarcoidosis and Other Granulomatous Disorders 2015 diagnostic criteria (JSSOG 2015 criteria) includes "clinically proven diagnosis" (involvement of at least 2 of 3 systems confirmed solely by clinical assessment) because of the frequency of sarcoidosis with ocular, cardiac, and respiratory involvement in Japan and the difficulty of obtaining specimens. Here, we describe in detail the clinical presentation of clinically diagnosed sarcoidosis. METHODS: We enrolled 68 consecutive patients with clinically diagnosed sarcoidosis (12 men, 56 women) based on the JSSOG 2015 criteria who were treated at Jichi Medical University between December 2018 and January 2000. We analyzed age at diagnosis, organ involvement, and laboratory findings. RESULTS: Age at diagnosis was unimodal in women. Ocular, splenic, cardiac, and skin involvement, and hypercalcemia were observed in 95.6%, 8.8%, 7.4%, 5.9%, and 35.0% of patients, respectively. High serum lysozyme and soluble interleukin-2 receptor (sIL-2R) levels, bilateral hilar lymphadenopathy on chest radiography, high-grade atrioventricular block or fatal ventricular arrhythmia, and bundle branch block were found in 18.8%, 48.3%, 95.6%, 5.0%, and 10.0% of patients, respectively. CONCLUSIONS: The age-specific distribution of clinically diagnosed sarcoidosis was similar to histologically diagnosed sarcoidosis in women, as previously reported. Rates of elevated serum lysozyme and sIL-2R levels were lower in this study than previously reported in histologically diagnosed patients in Japan.