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Clinical, serologic and magnetic resonance imaging of 3 cases of inflammatory myopathy with abundant macrophages in the Japanese population

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Abstract

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.

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Fig. 1
Fig. 2

Abbreviations

anti-ARS Abs:

Anti-aminoacyl tRNA synthetase antibodies

anti-CADM-140-kD Abs:

Anti-clinically amyopathic dermatomyositis-140-kD polypeptide antibodies

anti-155/140-kD Abs:

Anti-155/140-kD polypeptide antibodies

ARDS:

Acute respiratory distress syndrome

CHP:

Cytophagic histiocytic panniculitis

CK:

Creatine kinase

CT:

Computed tomography

DM:

Dermatomyositis

EMG:

Electromyography

IMAM:

Inflammatory myopathy with abundant macrophages

MAS:

Macrophage activation syndrome

MRI:

Magnetic resonance imaging

STIR:

Short-time inversion recovery

TNF α:

Tumor necrosis factor α

sTNFR:

Soluble TNR receptor

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Conflict of interest

The authors declare that they have no conflict of interest.

Author information

Authors and Affiliations

  1. Second Department of Internal Medicine, Nagasaki University School of Medicine, Nagasaki, Japan

    Shintaro Hara, Yuji Ishimatsu, Noriho Sakamoto, Tomoyuki Kakugawa & Shigeru Kohno

  2. Unit of Translational Medicine, Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan

    Tomoko Henmi, Atsushi Kawakami, Keita Fujikawa, Katsuya Satoh, Masakatsu Motomura, Mami Tamai, Hideki Nakamura, Hiroaki Ida & Katsumi Eguchi

  3. Department of Respiratory Medicine, University of Occupational and Environmental Health, Fukuoka, Japan

    Hiroshi Mukae

  4. Department of Dermatology, Kanazawa University Graduate School of Medical Science, Ishikawa, Japan

    Kenzou Kaji & Manabu Fujimoto

  5. Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan

    Masataka Kuwana

  6. Department of Internal Medicine, Isahaya Health Insurance General Hospital, Nagasaki, Japan

    Toshiaki Tsukada

  7. Department of Pathology, Nagasaki University Hospital, Nagasaki, Japan

    Tomayoshi Hayashi

  8. Nagasaki University School of Health Sciences, Nagasaki University, Nagasaki, Japan

    Tomoki Origuchi

Authors
  1. Shintaro Hara
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  2. Tomoko Henmi
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  3. Atsushi Kawakami
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  4. Keita Fujikawa
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  5. Hiroshi Mukae
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  7. Noriho Sakamoto
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  8. Tomoyuki Kakugawa
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  9. Kenzou Kaji
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  10. Manabu Fujimoto
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  14. Masakatsu Motomura
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  15. Mami Tamai
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  16. Hideki Nakamura
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  17. Hiroaki Ida
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  18. Tomayoshi Hayashi
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  19. Tomoki Origuchi
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  20. Katsumi Eguchi
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  21. Shigeru Kohno
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Corresponding author

Correspondence to Atsushi Kawakami.

Additional information

S. Hara and T. Henmi contributed equal to this work.

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Hara, S., Henmi, T., Kawakami, A. et al. Clinical, serologic and magnetic resonance imaging of 3 cases of inflammatory myopathy with abundant macrophages in the Japanese population. Rheumatol Int 33, 1059–1064 (2013). https://doi.org/10.1007/s00296-010-1662-4

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  • DOI: https://doi.org/10.1007/s00296-010-1662-4

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