Evidence map›Paper›PMID 42460087›Full record

ArticleFrontiers in medicine2026

Postpartum-onset anti-PM/Scl-positive dermatomyositis-systemic sclerosis overlap syndrome with reversible interstitial lung disease: a case report.

Joud Zghyer, Asad Omarion, Leen Zghyer, Yaman Ayasa, Omar Al-Deek, Adnan A M Wahdan

Erratum issuedAbstract readCase Reports
In one paragraph

Article in Frontiers in medicine, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Not yet cited in PubMed.

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1 · What the graph read from it

What it found

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2 · The registry

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3 · Its place in the literature

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4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

6 authors.

Joud Zghyer *Faculty of Medicine, Al-Quds University, Jerusalem, Palestine.
Asad Omarion *Faculty of Medicine, Al-Quds University, Jerusalem, Palestine.
Leen ZghyerFaculty of Medicine, Al-Quds University, Jerusalem, Palestine.
Yaman AyasaFaculty of Medicine, Al-Quds University, Jerusalem, Palestine.
Omar Al-DeekFaculty of Medicine, Al-Quds University, Jerusalem, Palestine.
Adnan A M WahdanDepartment of Internal Medicine, Palestine Medical Complex, Ramallah, Palestine.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Background: Polymyositis-scleroderma autoantibody (Anti-PM/Scl) associated connective tissue disease is a recognized overlap syndrome classically characterized by features of polymyositis- systemic sclerosis overlap. However, presentation with a dermatomyositis (DM) phenotype accompanied with interstitial lung disease (ILD) is less commonly reported. Pregnancy and the postpartum period are recognized immunological triggers for overlap syndromes, potentially leading to autoimmune disease. Postpartum-onset inflammatory myopathy with overlap features and significant pulmonary involvement poses substantial diagnostic challenges due to its heterogeneous clinical presentation and evolving serologic profiles. Case presentation: We present a case of a 25-year-old Palestinian woman presenting with progressive proximal muscle weakness, characteristic dermatomyositis cutaneous manifestations, notable unintentional weight loss, and systemic symptoms 4 months postpartum. Laboratory tests showed markedly elevated creatine kinase and positive antinuclear antibodies, positive anti-PM/Scl antibodies and positive anti-dsDNA antibodies, while anti-U1-RNP antibodies were negative. Imaging demonstrated hepatomegaly and splenomegaly, and pulmonary function testing showed interstitial lung disease (ILD). Muscle biopsy confirmed inflammatory myopathy without typical perifascicular atrophy. The patient showed incomplete clinical response to treatment with corticosteroids and mycophenolate mofetil; however, transitioning to rituximab resulted in substantial clinical, pulmonary, and functional improvement. Conclusion: This case illustrates a diagnostically challenging presentation of postpartum-onset Anti-PM/Scl-Positive dermatomyositis-systemic sclerosis overlap syndrome complicated by ILD and multisystem involvement. It underscores the diagnostic challenges associated with overlap syndrome and connective tissue disease phenotypes, and supports the potential effectiveness of rituximab in refractory overlap myositis and reversing inflammatory ILD.

Indexed as

dermatomyositisinterstitial lung diseaseoverlap syndromepostpartumrituximabsystemic sclerosis

Identifiers

PMID42460087
PMCPMC13368723

What Socratic holds

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