J Rheum Dis.  2015 Oct;22(5):308-312. 10.4078/jrd.2015.22.5.308.

A Case of Eosinophilic Polymyositis Treated with Immunosuppressants

• Choi JH
• Kang JH
• Wen L
• Lee JW
• Lee KE
• Park DJ
• Park YW
• Kim TJ
• Lee SS

• KMID: 2222844
• DOI: http://doi.org/10.4078/jrd.2015.22.5.308

Abstract

Eosinophilic polymyositis is a rare form of inflammatory muscle disease associated with peripheral blood and/or tissue eosinophilia. Most patients respond to glucocorticoids, however some show poor prognosis, leading to mortality. A 28-year-old female was admitted to our hospital because of myalgia and motor weakness of the upper and lower extremities. Laboratory findings showed significantly elevated levels of muscle enzymes and inflammatory lesions in both thigh muscles were demonstrated on magnetic resonance imaging. A diagnosis of eosinophilic polymyositis was based on histological findings, which showed diffuse eosinophilic infiltration into perivascular spaces in the endomysium and perimysium, and necrosis of myofibers. High-dose methylprednisolone treatment improved the clinical symptoms and muscle enzymes. However, on tapering the glucocorticoid dose, clinical and laboratory findings were exacerbated. After the addition of methotrexate and azathioprine, the symptoms and muscle enzymes recovered without relapse. Here, we report on a case of eosinophilic polymyositis, which responded to immunosuppressants.

Keyword

Myositis; Eosinophilic myositis; Immunosuppressants

MeSH Terms

Adult
Azathioprine
Diagnosis
Eosinophilia
Eosinophils*
Female
Glucocorticoids
Humans
Immunosuppressive Agents*
Lower Extremity
Magnetic Resonance Imaging
Methotrexate
Methylprednisolone
Mortality
Muscles
Myalgia
Myositis
Necrosis
Polymyositis*
Prognosis
Recurrence
Thigh
Azathioprine
Glucocorticoids
Immunosuppressive Agents
Methotrexate
Methylprednisolone

Figure

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  Figure 1. Electromyography (EMG) of right bicpes muscle (A) and right anterior tibialis muscle (B) show short duration and small polyphasic motor unit action potentials.

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  Figure 2. (A, B) T2 weighted magnetic resonance imaging of right thigh shows heterogeneous hypersignal intensity of thigh muscles and subcutaneous edema (arrows). (C) Fat suppression gadolinium enhanced axial T1 weight imaging shows heterogeneous enhancement of right thigh muscles (arrowheads).

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  Figure 3. A hematoxylin and eosin-stained paraffin section from the right thigh muscle biopsy specimen reveals infiltration of eosinophils (arrowhead) and inflammatory cells on perivascular spaces in the endomysium and perimysium and necrosis of myofibers (A: ×40, B: ×400).

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