Paraspinal and Scapular Myopathy Associated With Scleroderma

Rojana-udomsart, Arada; Fabian, V.; Hollingsworth, Peter; Walters, Sue; Zilko, P. J.; Mastaglia, Frank

doi:10.1097/cnd.0b013e3181c139f6

Cited by 22 publications

(19 citation statements)

References 32 publications

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“…A PubMED literature search retrieved 122 articles for morphoea and myositis. The majority of these articles describe an association between systemic sclerosis and myositis usually in a setting of established scleroderma disease [2]. The most similar case identified was described by Warin in 1973 and described a young man with morphoea overlying myositis; unlike our case, this patient did have a raised ESR and was ANA positive (ENA was not assessed [3]).…”

Section: Discussionmentioning

confidence: 78%

Morphoea with Myositis: A Rare Association

Sommerlad

Bull

Gorman

2011

Case Reports in Rheumatology

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Section: Discussionmentioning

confidence: 78%

Morphoea with Myositis: A Rare Association

Sommerlad

Bull

Gorman

2011

Case Reports in Rheumatology

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“…1 There are less than 30 cases described in the literature so far. [1][2][3] In most of them, myositis was associated with other immune-mediated disorders such as myasthenia gravis, rheumatoid arthritis, or mixed connective tissue disease.…”

mentioning

confidence: 99%

Thrombospondin-1 mediates muscle damage in brachio-cervical inflammatory myopathy and systemic sclerosis

Suárez‐Calvet

Alonso‐Pérez

Castellví

et al. 2020

Neurol Neuroimmunol Neuroinflamm

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ObjectiveTo describe the clinical, serologic and histologic features of a cohort of patients with brachio-cervical inflammatory myopathy (BCIM) associated with systemic sclerosis (SSc) and unravel disease-specific pathophysiologic mechanisms occurring in these patients.MethodsWe reviewed clinical, immunologic, muscle MRI, nailfold videocapillaroscopy, muscle biopsy, and response to treatment data from 8 patients with BCIM-SSc. We compared cytokine profiles between patients with BCIM-SSc and SSc without muscle involvement and controls. We analyzed the effect of the deregulated cytokines in vitro (fibroblasts, endothelial cells, and muscle cells) and in vivo.ResultsAll patients with BCIM-SSc presented with muscle weakness involving cervical and proximal muscles of the upper limbs plus Raynaud syndrome, telangiectasia and/or sclerodactilia, hypotonia of the esophagus, and interstitial lung disease. Immunosuppressive treatment stopped the progression of the disease. Muscle biopsy showed pathologic changes including the presence of necrotic fibers, fibrosis, and reduced capillary number and size. Cytokines involved in inflammation, angiogenesis, and fibrosis were deregulated. Thrombospondin-1 (TSP-1), which participates in all these 3 processes, was upregulated in patients with BCIM-SSc. In vitro, TSP-1 and serum of patients with BCIM-SSc promoted proliferation and upregulation of collagen, fibronectin, and transforming growth factor beta in fibroblasts. TSP-1 disrupted vascular network, decreased muscle differentiation, and promoted hypotrophic myotubes. In vivo, TSP-1 increased fibrotic tissue and profibrotic macrophage infiltration in the muscle.ConclusionsPatients with SSc may present with a clinically and pathologically distinct myopathy. A prompt and correct diagnosis has important implications for treatment. Finally, TSP-1 may participate in the pathologic changes observed in muscle.

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“…No official classification system currently exists, as disagreement continues. Many clinical patterns of inflammatory myopathy other than PM, DM, sIBM, and IMNM have been described and include brachiocervical inflammatory myopathy, 81 paraspinal and scapular inflammatory myopathy, 82 and focal myositis. 83 The existence of such entities lends further support to the need for a new classification scheme.…”

Section: Classificationmentioning

confidence: 99%