The Clinical and Pathological Characteristics of Polymyositis Using ENMC Diagnostic Criteria

Seung Hun Oh; Seung Min Kim; Il Nam Sunwoo; Dong Hyun Lee; Tae Seung Kim; Se Hun Kim; Young Chul Choi

doi:10.4078/jkra.2008.15.2.296

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Oh, Kim, Sunwoo, Lee, Kim, Kim, and Choi: The Clinical and Pathological Characteristics of Polymyositis Using ENMC Diagnostic Criteria

Original Article

J Korean Rheum Assoc 2008;15(4):296-305.

Published online: 20 January 2008

DOI: https://doi.org/10.4078/jkra.2008.15.2.296

The Clinical and Pathological Characteristics of Polymyositis Using ENMC Diagnostic Criteria

Seung Hun Oh¹, Seung Min Kim², Il Nam Sunwoo², Dong Hyun Lee², Tae Seung Kim³, Se Hun Kim³, Young Chul Choi²

¹Department of Neurology, Pochon CHA University College of Medicine, Pocheon, Korea

²Department of Neurology, Yonsei University College of Medicine, Seoul, Korea

³Department Pathology, Yonsei University College of Medicine, Seoul, Korea

Received 2 June 2008 Accepted 9 September 2008

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.

Keywords: Polymyositis, Inflammatory myopathy, Malignancy, Connective tissue disorders, Diagnostic criteria

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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.

Table 1.

Pathologic findings in 23 patients with polymyositis on muscle biopsy

Pathologic findings	No./tot al No. (%)
Endomysial mononuclear cell inflammation	23/23 (100%)
Infiltration into non-necrotic tissue	5/23 (22%)
(Definite PM)
Only surrounding inflammation	18/23 (78%)
(Probable PM) CD8^＋ immunoreactivity	5/5 (100%)
Perivascular inflammation	7/23 (30%)
Fiber type I predominance	1/23 (4%)
Perifascicular atrophy	0/23 (0%)
Dilated vessel or fibrin clot	0/23 (0%)
Rimmed vacuole or amyloid deposit	0/23 (0%)

Table 2.

Initial symptoms in 23 patients with polymyositis

Symptom	No	(%)
Muscle weakness of limbs	17	(74%)
Muscle weakness of limbs＋myalgia	2	(9%)
Muscle weakness of limbs＋dyspnea	1	(4%)
Muscle weakness of head and neck	1	(4%)
Myalgia only	1	(4%)
Asymptomatic elevation of liver enzyme	s 1	(4%)

Table 3.

Extramuscular symptoms in 23 patients with polymyositis

Marker	No.	(%)
Myalgia	10	(43%)
Fever	2	(9%)
Weight loss	6	(26%)
Arthralgia	2	(9%)^∗
Raynaud's phenomenon	1	(4%)^∗
Joint contracture	1	(4%)
Dyspnea	6	(26%)^†
Dysphagia	6	(26%)^‡

^∗ one patient was overlapped with rheumatoid arthritis,

^† one patient had interstitial pneumonia,

^‡ two patients were overlapped with systemic sclerosis

Table 4.

Positive rate of autoimmune markers in patients with polymyositis

Marker	No./total No.	(%)
RF^∗,†	4/16	(25%)
ANA	11/20	(55%)
Anti-dsDNA ab	1/15	(7%)
c-ANCA	0/6	(0%)
p-ANCA Anti-Ro/La^† ab	1/6 8/17	(17%) (47%)
Anti-RNP ab	2/10	(20%)
Anti-smooth muscle ab	2/7	(29%)
Anti-Jo1 ab	0/20	(0%)

^∗ one patient had rheumatoid arthritis,

^† two patients had systemic sclerosis

Table 5.

Response to treatment after 3 months in patients with polymyositis

Treatment	CR	PR	REF	REC	Death
Steroid	5 (22%)	9 (39%)	0 (0.0%)	2 (9%)	1 (4%)
Steroid＋immunosupressants^∗	0 (0%)	3 (13%)	0 (0%)	0 (0%)	0 (0%)
Steroird＋immunosupressants＋IVIg	0 (0%)	0 (0%)	2 (9%)	0 (0%)	1 (4%)

^∗ azathioprine or methotrexate, Abbreviations: CR=complete recovery, PR=partial recovery, REF=refractory, REC=recurred

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