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Case Reports
. 2023 Aug 28;15(8):e44292.
doi: 10.7759/cureus.44292. eCollection 2023 Aug.

Scleromyositis: A Novel Entity Beyond Systemic Sclerosis and Autoimmune Myositis

Affiliations
Case Reports

Scleromyositis: A Novel Entity Beyond Systemic Sclerosis and Autoimmune Myositis

Osvaldo Alexis Marche Fernandez et al. Cureus. .

Abstract

Scleromyositis is a new clinical entity, which not only has clinical and histopathological components of systemic sclerosis and inflammatory myopathy but is also characterized by presenting unique characteristics, which may not be in the previously mentioned diseases. Up until now, there are no specific classification criteria proposed by the American College of Rheumatology or the European League Against Rheumatism (ACR/EULAR). This paper presents a case report of a female patient in her 60s who was admitted to our institution due to muscle weakness in her legs and dysphagia. Within her diagnosis approach, clinical characteristics compatible with autoimmune myopathy were found; however, she presented with anti-PM/Scl75 antibody-positive results. In this paper, we emphasized the clinical characteristics and forms of presentation of scleromyositis, additionally discussing the available treatment for this entity.

Keywords: clinical rheumatology; diffuse systemic sclerosis; inflammatory myositis; limited systemic sclerosis; scleromyositis.

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. Flow-volume curve showing a decrease in FVC (blue lines showing the area under the curve) by 59% and FEV1 (red arrow) by 53%; a moderate restrictive pattern is then established
FVC: forced vital capacity, FEV1: forced expiratory volume in one second

References

    1. Systemic sclerosis. Hughes M, Herrick AL. Br J Hosp Med (Lond) 2019;80:530–536. - PubMed
    1. The 15% rule in scleroderma: the frequency of severe organ complications in systemic sclerosis. A systematic review. Muangchan C, Baron M, Pope J. J Rheumatol. 2013;40:1545–1556. - PubMed
    1. Idiopathic inflammatory myopathies. Lundberg IE, Fujimoto M, Vencovsky J, et al. Nat Rev Dis Primers. 2021;7:86. - PMC - PubMed
    1. Scleromyositis: a distinct novel entity within the systemic sclerosis and autoimmune myositis spectrum. Implications for care and pathogenesis. Giannini M, Ellezam B, Leclair V, et al. Front Immunol. 2022;13:974078. - PMC - PubMed
    1. Histopathological features of systemic sclerosis-associated myopathy: a scoping review. Lefebvre F, Giannini M, Ellezam B, et al. Autoimmun Rev. 2021;20:102851. - PubMed

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