Clinical, serologic and magnetic resonance imaging of 3 cases of inflammatory myopathy with abundant macrophages in the Japanese population.
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.
Second Department of Internal Medicine, Nagasaki University School of Medicine, Nagasaki, Japan.
Shintaro
S
Hara
Tomoko
T
Henmi
Atsushi
A
Kawakami
Keita
K
Fujikawa
Hiroshi
H
Mukae
Yuji
Y
Ishimatsu
Noriho
N
Sakamoto
Tomoyuki
T
Kakugawa
Kenzou
K
Kaji
Manabu
M
Fujimoto
Masataka
M
Kuwana
Toshiaki
T
Tsukada
Katsuya
K
Satoh
Masakatsu
M
Motomura
Mami
M
Tamai
Hideki
H
Nakamura
Hiroaki
H
Ida
Tomayoshi
T
Hayashi
Tomoki
T
Origuchi
Katsumi
K
Eguchi
Shigeru
S
Kohno
D044466:Asian People; D001402:B-Lymphocytes / Q000473:pathology; D003402:Creatine Kinase / Q000097:blood; D005205:Fascia / Q000473:pathology; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007564:Japan; D008264:Macrophages / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000473:pathology; D009220:Myositis / Q000097:blood / Q000188:drug therapy / Q000473:pathology*; D012867:Skin / Q000473:pathology; D016896:Treatment Outcome
D005938:Glucocorticoids; D003402:Creatine Kinase