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Review
. 2023 Jan 26:13:974078.
doi: 10.3389/fimmu.2022.974078. eCollection 2022.

Scleromyositis: A distinct novel entity within the systemic sclerosis and autoimmune myositis spectrum. Implications for care and pathogenesis

Margherita Giannini  1   2   3 , Benjamin Ellezam  4 , Valérie Leclair  5 , Frédéric Lefebvre  6   7 , Yves Troyanov  8 , Marie Hudson  5 , Jean-Luc Senécal  6   7 , Bernard Geny  1   3 , Océane Landon-Cardinal  6   7 , Alain Meyer  1   3   9
Affiliations
Review

Scleromyositis: A distinct novel entity within the systemic sclerosis and autoimmune myositis spectrum. Implications for care and pathogenesis

Margherita Giannini et al. Front Immunol. .

Abstract

Systemic sclerosis and autoimmune myositis are both associated with decreased quality of life and increased mortality. Their prognosis and management largely depend on the disease subgroups. Indeed, systemic sclerosis is a heterogeneous disease, the two predominant forms of the disease being limited and diffuse scleroderma. Autoimmune myositis is also a heterogeneous group of myopathies that classically encompass necrotizing myopathy, antisynthetase syndrome, dermatomyositis and inclusion body myositis. Recent data revealed that an additional disease subset, denominated "scleromyositis", should be recognized within both the systemic sclerosis and the autoimmune myositis spectrum. We performed an in-depth review of the literature with the aim of better delineating scleromyositis. Our review highlights that this concept is supported by recent clinical, serological and histopathological findings that have important implications for patient management and understanding of the disease pathophysiology. As compared with other subsets of systemic sclerosis and autoimmune myositis, scleromyositis patients can present with a characteristic pattern of muscle involvement (i.e. distribution of muscle weakness) along with multisystemic involvement, and some of these extra-muscular complications are associated with poor prognosis. Several autoantibodies have been specifically associated with scleromyositis, but they are not currently integrated in diagnostic and classification criteria for systemic sclerosis and autoimmune myositis. Finally, striking vasculopathic lesions at muscle biopsy have been shown to be hallmarks of scleromyositis, providing a strong anatomopathological substratum for the concept of scleromyositis. These findings bring new insights into the pathogenesis of scleromyositis and help to diagnose this condition, in patients with subtle SSc features and/or no autoantibodies (i.e. "seronegative" scleromyositis). No guidelines are available for the management of these patients, but recent data are showing the way towards a new therapeutic approach dedicated to these patients.

Keywords: antisynthetase syndrome; dermatomyositis; inflammatory myopathies; mixed connective tissue disease; myositis; scleroderma; scleromyositis; systemic sclerosis.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Capillary pathology in scleromyositis. (A) Immunofluorescence for collagen type 4 highlighting enlarged endomysial capillaries often with laminated appearance of basement membrane (open arrow). (B) Electron micrograph showing a collapsed capillary (black asterisk) with several concentric layers of reduplicated basement membrane (arrows) ensheathing many pericyte processes (open arrows). A fibroblast is also shown (white asterisk). Bars: A, 100 µm, B, 2 µm.
Figure 2
Figure 2
Clinico-sero-pathological definition of scleromyositis. Clinical phenotype, autoantibodies and main histopathological lesions associated to scleromyositis have been summarized.
See this image and copyright information in PMC

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Publication types

Grants and funding

Supported by Sclérodermie Québec. J-LS holds the University of Montreal Scleroderma Research Chair.