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Recent advances in polymyositis

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Abstract

The most typical clinical features of polymyositis (PM), the criteria of diagnosis and principles of treatment are outlined. An inflammatory disease of muscle, PM also frequently affects other organs such as the skin and hence the name dermatomyositis. The principal cardiac symptom is a peculiar disturbance of atrioventricular conduction, correlated with a specific anti-Ro autoantibody, present in 25% of patients. The etiology of PM is as yet unknown, although there is evidence for an autoimmune pathogenesis. It is frequently found in association with other immune-mediated diseases such as myasthenia gravis, pemphigus, immune-complex vasculitis and Sjogren syndrome. Laboratory investigations show hypergammaglobulinemia, a decrease of complement factors C3 and C4 and the presence of circulating immune complexes in 70% of patients. Very frequent, especially in cases of dermatomyositis, is a histologically detectable accumulation of IgG and complement in the walls of the intramuscular venous vessels. Cell-mediated hypersensitivity, emphasised formerly as highly significant in PM, has not been confirmed. The presence of specific antimyoglobin lymphocyto-toxicity, once considered to be the hallmark of muscle degeneration in PM, has been excluded by a number of laboratories.

In a personal series of patients with various clinical forms of PM a severe loss of suppressor/cytotoxic lymphocytes was found in the peripheral blood and a relative increase in the first subset. These results support the hypothesis that a serious disturbance of immunoregulation is present in PM and is the cause of a multitude of immunological anomalies, the characterisation of which is under study.

Sommario

Della polimiosite, (PM) malattia infiammatoria dei muscoli, vengono tratteggiati gli aspetti clinici più tipici, i criteri diagnostici e le linee terapeutiche. Frequente è la compromissione di altri organi come la cute da cui la definizione di dermatomiosite. Le alterazioni cardiache si caratterizzano per un peculiare disturbo di conduzione atrio-ventricolare, correlato ad un autoanticorpo specifico anti-Ro presente nel 25% dei pazienti.

L'eziologia della PM non è ancora nota anche se vi sono una serie di evidenze in favore di una patogenesi autoimmune.

Frequente è l'associazione con altre malattie immunomediate quali la miastenia grave, il pemfigo, la vasculite da immunocomplessi e la sindrome di Sjogren. Le indagini di laboratorio rivelano una ipergammablobulinemia, una diminuzione dei fattori del complemento C3 e C4 e la presenza di immunocomplessi circolanti nel 70% dei pazienti. Molto frequente, specie nei casi di dermatomiosite è l'accumulo istologicamente rilevabile di IgG e complemento nelle pareti dei vasi venosi intramuscola-ri.

L'ipersensibilità cell-mediated enfatizzata in passato come molto significativa nella PM non è stata confermata. È stata esclusa da una serie di indagini di laboratorio la presenza di linfocitossicità specifica antifibra muscolare che era considerata l'Hallmark patogenetico della degenerazione muscolare nella P.M.

In una serie di pazienti con diverse forme cliniche di PM gli Autori hanno riscontrato una severa diminuzione nel numero di linfociti soppressori/citotossici nel sangue periferico e un relativo aumento del primo subset. Questi risultati supportano l'ipotesi che un severo disturbo del controllo immunitario è presente nella PM ed è causa di una moltitudine di anomalie immunologiche la cui caratterizzazione è ancora oggetto di studio.

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This work was supported by the Muscular Dystrophy Group of Great Britain

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Behan, W.M.H., Behan, P.O. Recent advances in polymyositis. Ital J Neuro Sci 5, 23–31 (1984). https://doi.org/10.1007/BF02043966

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