ArticleRMD open2020
Recognising the spectrum of scleromyositis: HEp-2 ANA patterns allow identification of a novel clinical subset with anti-SMN autoantibodies.
Article in RMD open, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers, 1 of them a synthesis that pooled it.
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Who cites it
17 citing papers in PubMed, 1 synthesis or guideline pooled it, 35 citations in OpenAlex.
- Inflammatory myopathies overlapping with systemic sclerosis: a systematic review.Clinical rheumatology · 2022Pooled it
- Clinical Associations of Anti-RNPC3 Autoantibodies in Mixed Connective Tissue Disease.ACR open rheumatology · 2026Article
- Anti-survival of motor neuron antibodies in rheumatic and musculoskeletal diseases: prevalence, clinical associations, and biomarker potential, with novel insights into disease activity in SLE.Inflammation and regeneration · 2025Article
- Revisiting the link between oropharyngeal dysphagia and cancer in autoimmune myositis: a descriptive study.Arthritis research & therapy · 2025Article
- Autoantibodies in systemic sclerosis: From disease bystanders to pathogenic players.Journal of translational autoimmunity · 2025Review
- An update on autoantibodies in the idiopathic inflammatory myopathies.Nature reviews. Rheumatology · 2025Review
- The clinical features, muscle pathology, and role of autophagy in anti-Ku-positive patients.Frontiers in immunology · 2025Article
- Systemic sclerosis associated myopathy: how to treat.Current treatment options in rheumatology · 2023Article
- Anti-SMN autoantibodies in mixed connective tissue disease are associated with a severe systemic sclerosis phenotype.RMD open · 2023Article
- Anti-U11/U12 Antibodies as a Rare but Important Biomarker in Patients with Systemic Sclerosis: A Narrative Review.Diagnostics (Basel, Switzerland) · 2023Review
- Patients with inflammatory myopathies overlapping with systemic sclerosis: A Brazilian-Japanese bicentric study.Archives of rheumatology · 2023Article
- Anti-RuvBL1/2 Autoantibodies Detection in a Patient with Overlap Systemic Sclerosis and Polymyositis.Antibodies (Basel, Switzerland) · 2023Article
- The Involvement of Smooth Muscle, Striated Muscle, and the Myocardium in Scleroderma: A Review.International journal of molecular sciences · 2022Review
- Article
- Scleromyositis: A distinct novel entity within the systemic sclerosis and autoimmune myositis spectrum. Implications for care and pathogenesis.Frontiers in immunology · 2022Review
- Capillary basement membrane reduplication in myositis patients with mild clinical features of systemic sclerosis supports the concept of 'scleromyositis'.Acta neuropathologica · 2021Article
- The First Orally Deliverable Small Molecule for the Treatment of Spinal Muscular Atrophy.Neuroscience insights · 2020Review
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Authors and funding
22 authors at 9 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveTo describe systemic sclerosis (SSc) with myopathy in patients without classic SSc-specific and SSc-overlap autoantibodies (aAbs), referred to as seronegative scleromyositis.
methodsTwenty patients with seronegative scleromyositis diagnosed by expert opinion were analysed retrospectively for SSc features at myositis diagnosis and follow-up, and stratified based on HEp-2 nuclear patterns by indirect immunofluorescence (IIF) according to International Consensus of Autoantibody Patterns. Specificities were analysed by protein A-assisted immunoprecipitation. Myopathy was considered an organ involvement of SSc.
resultsSSc sine scleroderma was a frequent presentation (45%) at myositis diagnosis. Myositis was the most common first non-Raynaud manifestation of SSc (55%). Lower oesophagal dysmotility was present in 10 of 11 (91%) investigated patients. At follow-up, 80% of the patients met the American College of Rheumatology/EULAR SSc classification criteria. Two-thirds of patients had a positive HEp-2 IIF nuclear pattern (all with titers ≥1/320), defining three novel scleromyositis subsets. First, antinuclear antibody (ANA)-negative scleromyositis was associated with interstitial lung disease (ILD) and renal crisis. Second, a speckled pattern uncovered multiple rare SSc-specific aAbs. Third, the nuclear dots pattern was associated with aAbs to survival of motor neuron (SMN) complex and a novel scleromyositis subset characteriszed by calcinosis but infrequent ILD and renal crisis.
conclusionsSSc skin involvement is often absent in early seronegative scleromyositis. ANA positivity, Raynaud phenomenon, SSc-type capillaroscopy and/or lower oesophagal dysmotility may be clues for scleromyositis. Using HEp-2 IIF patterns, three novel clinicoserological subsets of scleromyositis emerged, notably (1) ANA-negative, (2) ANA-positive with a speckled pattern and (3) ANA-positive with nuclear dots and anti-SMN aAbs.
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