Myocardial dysfunction in polymyositis

Abstract

Myocardial involvement in polymyositis is occasionally suspected, but symptomatic cardiac dysfunction is rarely reported. Described in the present report is a 48-year-old woman with a two-year history of polymyositis who suddenly developed near fatal ventricular arrhythmia, and a 56-year-old man with a relapsing polymyositis who developed severe systolic dysfunction. These two cases emphasize the importance of systematic cardiac evaluation when the diagnosis of polymyositis is initially made and the necessity of re-evaluating cardiac function, even in the presence of clinical remission and normalization of creatine phosphokinase with treatment.

Clinical cardiac involvement in idiopathic inflammatory myopathies: A systematic review

“…Twelve of these were case reports contributing 14 patients [12][13][14][15][16][17][18][19][20][21][22][23]. The other 21 articles were either cohort studies or case series.…”

“…There were 11 prospective series, 8 retrospective chart reviews and 2 retrospective autopsy reviews. Table 2 documents the characteristics of patients in case reports [12,23] and Table 3 depicts the same in the cohort studies [8,[24][25][26][27][28][29][30][31][32][33][34][35][36].…”

“…Two prospective studies [25,36], three retrospective studies [26,27,31] and eight case reports included pathology data [12,13,16,[18][19][20][21]23]. One patient underwent heart transplantation and the pathology of the diseased heart was reported.…”

Treatment consensus for management of polymyositis and dermatomyositis among rheumatologists, neurologists and dermatologists

“…Methylprednisolone pulse therapy and high‐dose GC: Detailed therapeutic courses and the outcomes of 10 PM and DM patients with cardiac involvement were reported previously . While eight patients responded well to immunosuppressive agents, the other two patients developed new cardiac symptoms, which required pacemaker implantation even after treatment with immunosuppressants.…”

“…Immunosuppressants and other agents: Immunosuppressants were used from the early stage of treatment in all of the above 10 cases (CPA, four cases; MTX, five cases; AZA, four cases; CsA, one case; hydroxychloroquine, one case; rituximab, one case; in total number). Nonetheless, four cases were resistant and two cases improved only after two or more trials of immunosuppressive agents .…”