Journal List > J Korean Rheum Assoc > v.15(4) > 1003638

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

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.

References

1. DeVere R, Bradley WG. Polymyositis: its presentation, morbidity and mortality. Brain. 1975; 98:637–66.
crossref
2. Arahata K, Engel AG. Monoclonal antibody analysis of mononuclear cells in myopathies. V: identification and quantitation of T8+ cytotoxic and T8+ suppressor cells. Ann Neurol. 1988; 23:493–9.
crossref
3. Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet. 2003; 362:971–82.
crossref
4. Hill CL, Zhang Y, Sigurgeirsson B, Pukkala E, Mellemkjaer L, Airio A, et al. Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet. 2001; 357:96–100.
crossref
5. Buchbinder R, Forbes A, Hall S, Dennett X, Giles G. Incidence of malignant disease in biopsy-proven inflammatory myopathy. A population-based cohort study. Ann Intern Med. 2001; 134:1087–95.
crossref
6. Dalakas MC. Polymyositis, dermatomyositis and inclusion-body myositis. N Engl J Med. 1991; 325:1487–98.
crossref
7. Troyanov Y, Targoff IN, Tremblay JL, Goulet JR, Raymond Y, Senecal JL. Novel classification of idiopathic inflammatory myopathies based on overlap syndrome features and autoantibodies: analysis of 100 French Canadian patients. Medicine (Baltimore). 2005; 84:231–49.
8. van der Meulen MF, Bronner IM, Hoogendijk JE, Burger H, van Venrooij WJ, Voskuyl AE, et al. Polymyositis: an overdiagnosed entity. Neurology. 2003; 61:316–21.
crossref
9. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975; 292:344–7.
10. Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med. 1975; 292:403–7.
11. Amato AA, Griggs RC. Unicorns, dragons, polymyositis, and other mythological beasts. Neurology. 2003; 61:288–9.
crossref
12. Chahin N, Engel AG. Correlation of muscle biopsy, clinical course, and outcome in PM and sporadic IBM. Neurology. 2008; 70:418–24.
crossref
13. Hoogendijk JE, Amato AA, Lecky BR, Choy EH, Lundberg IE, Rose MR, et al. 119th ENMC international workshop: trial design in adult idiopathic inflammatory myopathies, with the exception of inclusion body myositis, 10–12 October 2003, Naarden, The Netherlands. Neuromuscul Disord. 2004; 14:337–45.
crossref
14. Griggs RC, Askanas V, DiMauro S, Engel A, Karpati G, Mendell JR, et al. Inclusion body myositis and myopathies. Ann Neurol. 1995; 38:705–13.
crossref
15. Arnett FC, Edworthy SM, Bloch DA, McShane DJ, Fries JF, Cooper NS, et al. The American Rheumatism Association 1987 revised criteria for the classification of rheumatoid arthritis. Arthritis Rheum. 1988; 31:315–24.
crossref
16. Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982; 25:1271–7.
crossref
17. Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum. 1980; 23:581–90.
18. Vitali C, Bombardieri S, Moutsopoulos HM, Balestrieri G, Bencivelli W, Bernstein RM, et al. Preliminary criteria for the classification of Sjogren's syndrome. Results of a prospective concerted action supported by the European Community. Arthritis Rheum. 1993; 36:340–7.
19. Nimelstein SH, Brody S, McShane D, Holman HR. Mixed connective tissue disease: a subsequent evaluation of the original 25 patients. Medicine (Baltimore). 1980; 59:239–48.
20. Bradley WG. Polymyositis: an overdiagnosed entity. Neurology. 2004; 63:402.
crossref
21. Hengstman GJ, van Engelen BG. Polymyositis: an overdiagnosed entity. Neurology. 2004; 63:402–3.
crossref
22. Hengstman GJ, van Engelen BG. Polymyositis, invasion of non-necrotic muscle fibres, and the art of repetition. BMJ. 2004; 329:1464–7.
crossref
23. Scola RH, Werneck LC, Prevedello DM, Toderke EL, Iwamoto FM. Diagnosis of dermatomyositis and polymyositis: a study of 102 cases. Arq Neuropsiquiatr. 2000; 58:789–99.
crossref
24. Ramirez G, Asherson RA, Khamashta MA, Cervera R, D'Cruz D, Hughes GR. Adult-onset polymyositis-dermatomyositis: description of 25 patients with emphasis on treatment. Semin Arthritis Rheum. 1990; 20:114–20.
25. Hochberg MC, Feldman D, Stevens MB. Adult onset polymyositis/dermatomyositis: an analysis of clinical and laboratory features and survival in 76 patients with a review of the literature. Semin Arthritis Rheum. 1986; 15:168–78.
crossref
26. Douglas WW, Tazelaar HD, Hartman TE, Hartman RP, Decker PA, Schroeder DR, et al. Polymyositis-dermatomyositis-associated interstitial lung disease. Am J Respir Crit Care Med. 2001; 164:1182–5.
crossref
27. Marie I, Hachulla E, Cherin P, Dominique S, Hatron PY, Hellot MF, et al. Interstitial lung disease in polymyositis and dermatomyositis. Arthritis Rheum. 2002; 47:614–22.
crossref
28. Hirakata M, Nagai S. Interstitial lung disease in polymyositis and dermatomyositis. Curr Opin Rheumatol. 2000; 12:501–8.
crossref
29. Hochberg MC, Feldman D, Stevens MB, Arnett FC, Reichlin M. Antibody to Jo-1 in polymyositis/dermatomyositis: association with interstitial pulmonary disease. J Rheumatol. 1984; 11:663–5.
30. Spath M, Schroder M, Schlotter-Weigel B, Walter MC, Hautmann H, Leinsinger G, et al. The longterm outcome of anti-Jo-1-positive inflammatory myopathies. J Neurol. 2004; 251:859–64.
crossref
31. Sigurgeirsson B, Lindelof B, Edhag O, Allander E. Risk of cancer in patients with dermatomyositis or polymyositis. A population-based study. N Engl J Med. 1992; 326:363–7.
32. Medsger TA Jr, Dawson WN Jr, Masi AT. The epidemiology of polymyositis. Am J Med. 1970; 48:715–23.
crossref
33. Reichlin M, Maddison PJ, Targoff I, Bunch T, Arnett F, Sharp G, et al. Antibodies to a nuclear/nucleolar antigen in patients with polymyositis overlap syndromes. J Clin Immunol. 1984; 4:40–4.
crossref
34. Oddis CV, Okano Y, Rudert WA, Trucco M, Duquesnoy RJ, Medsger TA Jr. Serum autoantibody to the nucleolar antigen PM-Scl. Clinical and immunogenetic associations. Arthritis Rheum. 1992; 35:1211–7.
crossref
35. Maugars YM, Berthelot JM, Abbas AA, Mussini JM, Nguyen JM, Prost AM. Long-term prognosis of 69 patients with dermatomyositis or polymyositis. Clin Exp Rheumatol. 1996; 14:263–74.
36. Sultan SM, Ioannou Y, Moss K, Isenberg DA. Outcome in patients with idiopathic inflammatory myositis: morbidity and mortality. Rheumatology (Oxford). 2002; 41:22–6.
crossref
37. Marie I, Hachulla E, Hatron PY, Hellot MF, Levesque H, Devulder B, et al. Polymyositis and dermatomyositis: short term and longterm outcome, and predictive factors of prognosis. J Rheumatol. 2001; 28:2230–7.
38. Miller FW, Leitman SF, Cronin ME, Hicks JE, Leff RL, Wesley R, et al. Controlled trial of plasma exchange and leukapheresis in polymyositis and dermatomyositis. N Engl J Med. 1992; 326:1380–4.
crossref

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.
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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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