Dataset Information

Brachio-Cervical Inflammatory Myopathy: A Distinct Syndrome with Variable Association to Systemic Sclerosis — Insights from Clinical, Histopathological, and Transcriptomic Profiling

ABSTRACT: Brachio-cervical inflammatory myopathy (BCIM) is a rare and under-characterized subtype of idiopathic inflammatory myopathies (IIM), marked by prominent upper limb and neck muscle involvement. Since its initial description in 2006, few studies have addressed its clinical spectrum or pathogenesis, and its potential overlap with systemic sclerosis (SSc) remains a matter of debate. Here, we aimed to characterize the clinical, histopathological, and molecular features of BCIM in a large multicenter cohort and assess whether BCIM constitutes a distinct entity within the IIM spectrum.

INSTRUMENT(S):

ORGANISM(S): Homo Sapiens (human)

TISSUE(S): Skeletal Muscle

SUBMITTER: Andreas Hentschel

LAB HEAD: Prof. Dr. Albert Sickmann

PROVIDER: PXD070103 | Pride | 2026-04-13

REPOSITORIES: Pride

ACCESS DATA

Dataset's files

Source:

			Action	DRS
	20240703_072649_AH_BCIM_Kleefeld_adult_ctrls.sne	Other
	20240703_072649_AH_BCIM_Kleefeld_adult_ctrls_Report.csv	Csv
	Data_and_Sample_Matrix.xlsx	Xlsx
	QExactiveHF02_19010.mgf	Mgf
	QExactiveHF02_19010.raw	Raw

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Publications

Brachio-cervical inflammatory myopathy: multilevel clinical, histopathological and multi-omic analyses of a syndrome variably associated with systemic sclerosis.

Kleefeld Felix F Teran Gamboa Joanna J Pinal-Fernandez Iago I Preusse Corinna C Nelke Christopher C Goebel Hans-Hilmar HH Mensch Alexander A Mossakowski Agata A Miah Mohi-Uddin MU Diaz-Manera Jordi J Torchia Eleonora E Bortolani Sara S Hentschel Andreas A Funke Andreas A Souvannanorath Sarah S Authier François-Jérôme FJ Malfatti Edoardo E Gehrig Johannes J Mammen Andrew L AL Casal-Dominguez Maria M De Winter Jonathan J De Ridder Willem W Ruck Tobias T Schneider Udo U Roos Andreas A Gallardo Eduard E Tasca Giorgio G Stenzel Werner W

Acta neuropathologica 20260404 1

Brachio-cervical inflammatory myopathy (BCIM) is a rare and under-characterized subtype of idiopathic inflammatory myopathies (IIM), marked by prominent upper limb and neck muscle involvement. Since its initial description in 2006, few studies have addressed its clinical spectrum or pathogenesis, and its potential overlap with systemic sclerosis (SSc) remains a matter of debate. Here, we aimed to characterize the clinical, histopathological, and molecular features of BCIM in a large multicenter ...[more]

PMID: 41934478

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