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Multifactorial study of inflammatory myopathies. Report of 29 cases

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Abstract

We made a comparative clinical, immunopathological and therapeutic evaluation in 17 patients with polymyositis (PM) and 12 patients with dermatomyositis (DM), followed up at our Neuromuscular Center. DM can be distinguished by its clinical appearance and pathological changes. Current evidence suggests that it results from vasculopathy.

For studying these inflammatory myopathies we used multifactorial diagnostic criteria, evaluating the therapeutic response by means of a composite clinical and functional score in a longitudinal study. In muscle biopsy specimens we characterized with monoclonal antibodies T lymphocyte subpopulations (CD4, CD8), macrophages, IgG, IgM, C1q, C3, C4 complement fractions, MHC-I, MHC-II. In PM the cell-mediated immunity was more pronounced and in sowithin muscle fibers. Our patients were treated with steroids; in resistant cases azathioprine, cyclophosphamide, plasmapheresis, high-dose intravenous immunoglobulins (ivIgG) and total body irradiation were added to the therapeutic schedule.

Sommario

È stata condotta una valutazione comparativa di tipo clinico, immunopatologico e terapeutico di 17 pazienti affetti da Polimiosite (PM) e 12 affetti da Dermatomiosite (DM), seguiti presso il nostro Centro.

Nello studio di queste miopatie infiammatorie è stato utilizzato un criterio diagnostico multifattoriale clinico-bioumorale con diversi parametri immunologici ed immunoistopatologico sulla biopsia, valutando la risposta terapeutica durante un follow-up longitudinale con uno score clinico-funzionale composito.

La DM può essere distinta dai segni clinici e dalle anomalie patologiche; si ritiene attualmente che essa sia il risultato di una vasculopatia.

Sulle biopsie muscolari sono stati caratterizzati con anticorpi monoclonali, le sottopopolazioni di linfociti T (CD4, CD8), i macrofagi, IgG, IgM, le frazioni C1q, C3, C4 del Complemento, gli antigeni di istocompatibilità MHC-I ed MHC-II.

Nelle PM l'immunità cellulo mediata era più pronunciata ed in alcuni casi, l'espressione di MHC-I ed MHC-II era osservabile anche sulla superficie ed all'interno di fibre muscolari.

I pazienti sono stati trattati, con steroidi e nei casi non responsivi, con aggiunta di azatioprina, ciclofosfamide, plasmaferesi, alte dosi di immunoglobuline endovena ed irradiazione “total body”.

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Authors and Affiliations

  1. Centro di Epidemiologia e Prevenzione delle Malattie Neuromuscolari, Università di Padova, Via Giustiniani 5, 35128, Padova

    Angelini C., Menegazzo E. & Fanin M.

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  1. Angelini C.
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  2. Menegazzo E.
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  3. Fanin M.
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Angelini, C., Menegazzo, E. & Fanin, M. Multifactorial study of inflammatory myopathies. Report of 29 cases. Ital J Neuro Sci 14, 69–76 (1993). https://doi.org/10.1007/BF02339045

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