A 79-year-old woman was evaluated for weakness, dysphagia, and elevated levels of creatinine kinase. Her medical history included stage IIIB cervical cancer eight years previously, which improved after undergoing radiotherapy. Two years later, cancer recurred in the right hilar and mediastinal lymph nodes, and the patient was successfully treated with chemotherapy. Physical examinations showed Gottron's sign, the V sign, the Holster sign, and nailfold erythema. Computed tomography revealed left supraclavicular lymphadenopathy, compatible with cancer recurrence. Anti-nuclear matrix protein 2 (NXP2) antibody was found in her sera and a diagnosis of cancer-associated dermatomyositis was thus made. This case suggests that cancer progression over a period of years may have triggered the onset of autoimmunity against NXP2.
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http://dx.doi.org/10.2169/internalmedicine.9415-22 | DOI Listing |
Medicine (Baltimore)
November 2024
Department of Neurosurgery, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, China.
This retrospective study aimed to investigate differences in clinical characteristics between different antibody phenotypes in patients with dermatomyositis (DM). Two hundred and ninety-three patients with DM were included in this study from September 2018 to September 2023. We collected basic clinical data from the patients, using statistical methods to analyze the clinical characteristics, and used survival analysis and COX regression to assess the prognosis of the patients.
View Article and Find Full Text PDFZhonghua Nei Ke Za Zhi
October 2024
Department of Rheumatology, China-Japan Friendship Hospital, Beijing 100029, China.
Rheumatology (Oxford)
August 2024
Department of Neurology, Peking University First Hospital, Beijing 100034, China.
Objectives: Dermatomyositis (DM) is a rare type I interferon (IFN-I)-driven autoimmune disease, and anti-nuclear matrix protein 2 (NXP2) antibody is related to severe muscle disease and poor prognosis. Circulating cell-free DNA (ccf-DNA), including ccf-mitochondrial DNA and ccf-nuclear DNA, activates cGAS/STING pathway to induce IFN-I production in autoimmune diseases. We investigated whether serum-derived ccf-DNA played a pathogenic role on skeletal muscle in anti-NXP2 antibody-positive DM.
View Article and Find Full Text PDFMod Rheumatol Case Rep
August 2024
Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Vesiculobullous dermatomyositis (VD) is a rare manifestation of dermatomyositis (DM) and has been suggested to be associated with malignancy. Although the myositis-specific autoantibodies are associated with distinct clinical presentations of DM, those associated with VD remain unclear. Here, we present the case of a 54-year-old man with VD who tested positive for anti-nuclear matrix protein 2 (NXP-2) antibody, one of the DM-specific autoantibodies.
View Article and Find Full Text PDFFront Pediatr
July 2024
Department of Allergy, Immunology and Rheumatology, Liuzhou Hospital, Guangzhou Women and Children's Medical Center, Liuzhou, China.
Juvenile dermatomyositis (JDM) is a rare autoimmune disorder with multi-system involvement, often presenting with a heliotrope rash, Gottron's papules, and proximal muscle weakness. JDM patients with anti-nuclear matrix protein 2 (anti-NXP2) positivity tend to have more severe manifestations, including a higher risk of gastrointestinal (GI) complications such as dysphagia, intestinal motility changes, edema, malabsorption, ulcers, and perforations. These complications are associated with poor outcomes and high mortality rates, particularly in patients with anti-NXP2 positivity.
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