RHEUMATOLOGY INTERNATIONAL 33, 4, 1059 - 1064 (2010).
Clinical, serologic and magnetic resonance imaging of 3 cases of inflammatory myopathy with abundant macrophages in the Japanese population


Shintaro Hara , Tomoko Henmi , Atsushi Kawakami , Keita Fujikawa , Hiroshi Mukae , Yuji Ishimatsu , Noriho Sakamoto , Tomoyuki Kakugawa , Kenzou Kaji , Manabu Fujimoto , Masataka Kuwana , Toshiaki Tsukada , Katsuya Satoh , Masakatsu Motomura , Mami Tamai , Hideki Nakamura , Hiroaki Ida , Tomayoshi Hayashi , Tomoki Origuchi , Katsumi Eguchi , Shigeru Kohno




We report the first 3 cases of inflammatory myopathy with abundant macrophages (IMAM) to be found in an Asian country. Diagnosis of IMAM was based on the infiltration of CD68+ macrophages into biopsied specimens, particularly the fascia. Proximal skeletal muscle symptoms and signs, elevation of creatine kinase, and myogenic changes in electromyography were found in all of the cases, and magnetic resonance imaging clearly revealed thickening of the fascia. Since dermatomyositis (DM)-specific skin alterations were not found, none of the patients in this study fulfilled Bohan and Peter's criteria for DM; however, anti-PL-7 antibody was detected in case number 1. In addition, CD20+ B-cell infiltration into the fascia was also detected in all of the cases, indicating further transition to DM. Severe illness, namely macrophage activation syndrome and acute respiratory distress syndrome, occurred in case 1 but was resolved with intensive combination therapy. The other 2 cases also required glucocorticoids to achieve remission.