J Korean Rheum Assoc.  2008 Dec;15(4):296-305. 10.4078/jkra.2008.15.4.296.

The Clinical and Pathological Characteristics of Polymyositis Using ENMC Diagnostic Criteria

• Oh SH
• Kim SM
• Sunwoo IN
• Lee DH
• Kim TS
• Kim SH
• Choi YC

• KMID: 1270160
• DOI: http://doi.org/10.4078/jkra.2008.15.4.296

Abstract

OBJECTIVE: Polymyositis (PM) has known to be the most common type of idiopathic inflammatory myopathy (IIM). However, recent immunopathological studies demonstrated that PM was overdiagnosed previously due to suboptimal classification system. Using newly proposed classification system, we investigated the frequency, clinical and pathological characteristics of PM. METHODS: Among the patients diagnosed as IIM during past 6 years, we classified a 'definite' or 'probable PM' using the European Neuromuscular Center (ENMC) diagnostic criteria. The findings of clinical, laboratory and pathological findings were analyzed. Response to treatment was assessed at 6 months after treatment. RESULTS: Of total 97 cases with IIM, twenty-three cases (24%) were satisfactory to the diagnostic criteria for PM (definite=5 and probable=18). Most cases were young adults, and female predominance was found. All cases showed proximal muscle weakness, and about two-thirds of patients showed extramuscular manifestation. One (4%) had breast cancer, and accompanying connective tissue disorders (CTDs) were found in 3 cases (13%), two of which had systemic sclerosis. Interstitial pneumonia was found in one case (4%). All cases showed marked elevation of serum creatine kinase level. On muscle biopsy, there were endomysial mononuclear cell infiltrations in all cases. Three-fourths of patients responded to immunosuppressant therapy (74%). CONCLUSION: Using ENMC criteria, the frequency of PM was lower than that had been reported previously. The results of clinical characteristics, response to therapy and clinical outcome were similar to the previous reports. However, association of malignancy or CTDs was low in PM.

Keyword

Polymyositis; Inflammatory myopathy; Malignancy; Connective tissue disorders; Diagnostic criteria

MeSH Terms

Adult
Male
Female
Humans
Biopsy
Breast Neoplasms

Figure

• 

  Fig. 1. Examples of muscle pathology in patients with PM. There were widespread mononuclear inflammatory cells infiltrations on endomysium. (A) Inflammatory cells invaded into the non-necrotic tissues (white arrows), which fulfilled the ‘definite' PM according to the ENMC diagnostic criteria. (B) Inflammatory cells only surround the non-necrotic tissues without invading the tissues, which fulfilled the ‘probable' PM. (C) There were numerous CD8＋ T lymphocytes (brown color) on endomysium.

Reference

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