2026-08-12 | Case Report: Glucocorticoids with Early Aggressive Rituximab and Upadacitinib regimen in the treatment of three patients with anti-MDA5 dermatomyositis-associated interstitial lung disease.
Anti-melanoma differentiation-associated gene 5 (anti-MDA5) dermatomyositis (DM) is a severe phenotype that is frequently complicated by rapidly progressive interstitial lung disease (RP-ILD), which carries high mortality despite conventional immunosuppression. Given the dual pathogenesis involving B-cell activation and dysregulated interferon (IFN) pathways, there is a rationale for combining rituximab with Janus kinase (JAK) inhibitors. We present three patients with anti-MDA5 DM and severe ILD who were treated at a single center with a novel aggressive triple regimen consisting of high-dose glucocorticoids, rituximab, and upadacitinib. Patients were reviewed for clinical, biochemical, and radiological responses. All three patients demonstrated significant clinical and objective improvement following the initiation of the glucocorticoid-rituximab-upadacitinib regimen, including resolution of skin disease, improvement in lung function and high-resolution computed tomography (HRCT) findings, and reduction in disease activity markers. No deaths were observed during at least 6 month of follow-up. Serious opportunistic infections occurred in one patient. Concomitant intravenous immunoglobulin (IVIG) therapy was administered to one patient with dysphagia and to one patient with superimposed infection. An early aggressive regimen of dual-pathway inhibition combining rituximab and upadacitinib, in addition to high-dose glucocorticoids and adjunctive IVIG therapy, may be an effective therapeutic strategy for severe anti-MDA5-associated ILD.
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2026-08-11 | Dermatomyositis as a paraneoplastic sign of breast cancer: a case report and literature review.
Dermatomyositis (DM) is a rare autoimmune inflammatory disorder characterized by skin and muscle involvement and is well known for its association with malignancy. Clinically amyopathic dermatomyositis (ADM) is a distinct subtype that presents with characteristic cutaneous manifestations in the absence of clinically significant muscle weakness, which may delay diagnosis. ADM can occur as a paraneoplastic syndrome and warrants thorough evaluation for underlying malignancy. A 39-year-old Iranian premenopausal woman presented with a one-year history of a neglected right breast mass and progressive pruritic erythematous skin lesions involving the trunk and upper extremities. Physical examination revealed no muscle weakness. Imaging demonstrated a large right breast mass with bilateral axillary lymphadenopathy. Core needle biopsy confirmed grade 3 invasive ductal carcinoma (ER-positive, PR-positive, HER2-negative). Laboratory tests showed mild elevated inflammatory markers and muscle enzymes. Skin biopsy findings were consistent with dermatomyositis. Corticosteroid therapy was ineffective. A diagnosis of paraneoplastic clinically amyopathic dermatomyositis was established. Following initiation of neoadjuvant chemotherapy with doxorubicin and cyclophosphamide, cutaneous lesions resolved completely after the first treatment cycle. The patient subsequently underwent surgery and adjuvant radiotherapy, with no recurrence during follow-up. DM may represent a paraneoplastic manifestation of breast cancer and poses diagnostic challenges, particularly in the absence of muscle involvement. Steroid resistance and rapid resolution following chemotherapy support a tumor-driven immune mechanism. Early recognition of paraneoplastic ADM and prompt cancer-directed therapy are essential for optimal outcomes.
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2026-08-11 | Effectiveness and safety of upadacitinib on adult dermatomyositis: a cohort study in China.
This retrospective study investigated the efficacy and safety of upadacitinib in adult patients with dermatomyositis (DM). Adult patients with DM who received upadacitinib between January 2024 and April 2025 were included and followed up for 6 months. Clinical data and complications were collected. The cohort included 40 adult patients with DM, of whom 21 (52.5%) were female. The mean age was 46.93 ± 11.04 years. The median disease duration was 14 (8.25, 31) months. At baseline, the mean disease visual analog scale (VAS) score was 3.93 ± 1.75, and the mean daily glucocorticoid (GC) dose was 29.03 ± 17.02 mg. By the 3-month follow-up, the mean VAS score decreased to 2.57 ± 1.56 (p < 0.001), with significant improvements in cutaneous involvement, interstitial lung disease and manual muscle test 8 score (all p < 0.05). The mean daily GC dose was reduced to 17.66 ± 10.35 mg (p < 0.001). At 6 months, the mean VAS score further decreased to 1.74 ± 1.46 (p < 0.001). The mean daily GC dose was further reduced to 10.65 ± 5.97 mg (p < 0.001). Only four patients with anti-MDA5 antibody showed disease aggravation or relapsed in the cohort. Infection was the most common complication affecting 17 patients, including 6 patients with CMV viraemia and 3 patients with herpes zoster. Upadacitinib may be a promising treatment option for adult DM, contributing to clinical improvement and GC dose reduction.
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2026-08-10 | Outcomes of Immune Check Point Inhibitor Use in US Veterans With Pre-Existing Idiopathic Inflammatory Myopathies: Case Series and Literature Review.
Our objective was to identify and describe the clinical characteristics and outcomes in patients with pre-existing idiopathic inflammatory myopathies (IIMs) in the Veterans Health Administration (VHA) treated with immune checkpoint inhibitors (ICIs). All veterans receiving ICI infusions were identified. Patients with at least two International Classification of Disease codes for IIM before first ICI infusion underwent chart review to identify patients with confirmed IIM by American College of Rheumatology/EULAR criteria. The demographics, cancer diagnoses, laboratory findings, clinical course, and mortality rates were reported. IIM cases were also reviewed to determine if patients developed IIM flare following ICI treatment. We identified 29,539 veterans who received at least one ICI infusion in the VHA between June 6, 2011, and February 14, 2023. The eight patients with confirmed IIM before ICI treatment were mostly White men with an average age of 71.7 years at first ICI infusion. The IIM diagnoses were dermatomyositis in three patients, polymyositis in two, antisynthetase syndrome in two, and one rheumatoid arthritis (RA)/myositis overlap. Cancer diagnoses were lung (two), melanoma (two), head and neck (two), kidney/other urinary (one), and mesothelioma (one). Diagnosis of IIM was made on average 3.1 years before ICI treatment. One patient had an IIM flare after ICI. No IIM flares were identified in other patients. Our findings show that an IIM flare after ICI therapy can occur, but most patients did not develop a flare. These observations suggest ICIs can be considered as an option in patients with cancer with IIM with shared decision-making and close monitoring.
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2026-08-09 | Spontaneous Retroperitoneal Hemorrhage in Anti-NXP2-Positive Dermatomyositis: a Rare but Severe Complication.
Spontaneous hemorrhage is a rare but serious complication of dermatomyositis (DM). We report a 61-year-old man with anti-NXP2-positive DM who developed a retroperitoneal hematoma without anticoagulation, shortly after corticosteroid initiation. Laboratory investigations demonstrated rhabdomyolysis, hepatic injury and coagulopathy. The patient was managed with red blood cell transfusion, factor VIII, high-dose corticosteroids and intravenous immunoglobulin, resulting in complete recovery. Although hemorrhagic events in DM are most often linked to anti-MDA5 or anti-Ro52 antibodies, their association with anti-NXP2 antibodies remains uncertain. This case highlights the importance of early recognition and prompt intervention in managing spontaneous hemorrhage in DM.
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