Capillary pathology with prominent basement membrane reduplication is the hallmark histopathological feature of scleromyositis

Aims We aim to perform ultrastructural and histopathological analysis of muscle biopsies from a large group of systemic sclerosis (SSc) patients, including some with early/mild SSc features, and examine whether capillary pathology differentiates ‘scleromyositis’ (SM) from other auto‐immune myositis...

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Published in:Neuropathology and applied neurobiology 2022-12, Vol.48 (7), p.e12840-n/a
Main Authors: Ellezam, Benjamin, Leclair, Valérie, Troyanov, Yves, Bersali, Imane, Giannini, Margherita, Hoa, Sabrina, Bourré‐Tessier, Josiane, Nadon, Valérie, Drouin, Julie, Karamchandani, Jason, O'Ferrall, Erin, Lannes, Béatrice, Satoh, Minoru, Fritzler, Marvin J., Senécal, Jean‐Luc, Hudson, Marie, Meyer, Alain, Landon‐Cardinal, Océane
Format: Article
Language:English
Subjects:
auto‐immune myositis
Basement membranes
Biopsy
Blood vessels
Capillaries
Collagen
Electron microscopy
Immunofluorescence
Inflammation
Inflammatory diseases
Light microscopy
Microscopy
muscle biopsy
Musculoskeletal diseases
Myopathy
Myositis
Pathology
pericytes
Scleroderma
Systemic sclerosis
vasculopathy
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Summary:Aims We aim to perform ultrastructural and histopathological analysis of muscle biopsies from a large group of systemic sclerosis (SSc) patients, including some with early/mild SSc features, and examine whether capillary pathology differentiates ‘scleromyositis’ (SM) from other auto‐immune myositis (AIM) subsets. Methods Muscle biopsies from a total of 60 SM patients and 43 AIM controls from two independent cohorts were examined by electron microscopy, collagen‐4 immunofluorescence (Col4IF) and routine light microscopy. Results Ultrastructural examination revealed prominent capillary basement membrane (BM) reduplication (4+ layers in >50% of capillaries) in 65% of SM vs 0% of AIM controls (p 
ISSN:0305-1846
1365-2990
DOI:10.1111/nan.12840