Open research questions in Inflammatory Myopathies and Dermatomyositis
45 unresolved questions extracted from the limitations and future-work sections of 162 Inflammatory Myopathies and Dermatomyositis papers in our library. Each links back to the study that raised it.
What the literature leaves open
The pathogenicity of the FHL1 c.401A>C (p.Gln134Pro) variant remains unproven. Further studies are necessary to confirm the relationship between the variant and RBM.
Early diagnosis can be challenging due to limited initial laboratory abnormalities. Delayed treatment can lead to severe complications. The disease can involve multiple systems, making management challenging.
Dermatomyositis With Necrotizing Vasculitis, Pulmonary Arterial Hypertension, and Extensive Calcinosis Following Delayed Diagnosis · 2026 · DOIThe treatment of PAH in DM is challenging due to the limited understanding of the condition. The current treatment options for PAH in DM are limited, and new approaches are needed. The patient's condition was complicated by the presence of refractory cutaneous ulcers and PAH.
Improvement of Refractory Cutaneous Ulcers and Pulmonary Arterial Hypertension Following Rituximab and Mycophenolate Mofetil Therapy in Anti-melanoma Differentiation-Associated Gene 5 (Anti-MDA5) Antibody-Positive Dermatomyositis · 2026 · DOIThe diagnosis of dermatomiositis por anti-MDA5 can be challenging due to its rarity and nonspecific clinical features. The treatment of hepatic involvement in these patients can be difficult due to the lack of effective therapies.
Esteatosis hepática en la presentación inicial de dos pacientes con dermatomiositis por anti-MDA5 · 2026 · DOIThe patient had a complex medical history, including type 2 diabetes, hypertension, dyslipidemia, lacunar stroke, and knee osteoarthritis - The patient had no history of malignancy, autoimmune disease, or smoking - The patient had received the SARS-CoV-2 vaccine several times, but the exact number was unknown
New-onset Anti-melanoma Differentiation-associated Gene 5 Antibody Positive Clinically Amyopathic Dermatomyositis Following COVID-19 · 2026 · DOIDiagnostic uncertainty due to uncommon presentation. Potential for unnecessary surgical intervention. Need for increased awareness and recognition of musculoskeletal complications.
Acute Necrotizing Pancreatitis Presenting With Extensive Unilateral Lower Limb Myositis, Fasciitis, and Sterile Reactive Knee Synovitis: A Rare Extra-pancreatic Manifestation · 2026 · DOILarger multi-center studies are needed to validate the metabolic responder classification criteria and determine predictors of treatment response across diverse IBM populations.
Modifying muscle metabolic dysregulation in inclusion body myositis with pioglitazone: a single-arm trial · 2026 · DOIThe diagnostic utility of nailfold capillaroscopy in juvenile dermatomyositis remains insufficiently defined. Longitudinal outcomes beyond 16 years were previously undocumented.
Use of nailfold capillaroscopy for evaluation of disease activity in juvenile dermatomyositis: Results of a two-center retrospective study · 2026 · DOIVariable availability of IVIG. High costs of IVIG. Need for new treatments with better outcomes.
There is a need for new treatments for dermatomyositis with better outcomes. Current treatment often has disappointing outcomes. This paper aims to address this gap by testing a new potential treatment, brepocitinib.
The first deltoid biopsy was insufficient to establish a diagnosis, likely due to the focal and asymmetric histopathological involvement typical of RBM [5,6].
The case report lacks long-term follow-up to determine the effect of anesthetic management on disease progression. The study has a small sample size, with only one patient. The study does not provide a control group for comparison.
Cesarean delivery in a patient with inclusion body myositis: is general anesthesia safe? A case report · 2026 · DOIThere is a lack of studies on the use of general anesthesia in IBM patients undergoing cesarean delivery. There is a need for more research on the safety and feasibility of general anesthesia in IBM patients.
Cesarean delivery in a patient with inclusion body myositis: is general anesthesia safe? A case report · 2026 · DOIThe diagnosis of dermatomyositis can be challenging when classic cutaneous features are absent. Overlap phenotypes, such as PM/Scl-positive overlap myositis, are not well understood and can delay recognition.
Rash-Negative Dermatomyositis-Spectrum Overlap Myositis With PM/Scl Positivity Presenting With Progressive Pharyngeal Dysphagia · 2026 · DOIFurther studies are needed to investigate the mechanisms underlying the association between anti-Ro52 antibodies and interstitial lung disease. Future research should examine the potential of anti-Ro52 antibodies as a biomarker for identifying patients with idiopathic inflammatory myopathies who are at risk of developing interstitial lung disease.
Antibodies against Ro52 in idiopathic inflammatory myopathies are associated with objective sicca symptoms · 2026 · DOIThe association between anti-Ro52 antibodies and objective sicca symptoms in patients with idiopathic inflammatory myopathies is not well understood. The clinical phenotype of idiopathic inflammatory myopathies is not fully characterized.
Antibodies against Ro52 in idiopathic inflammatory myopathies are associated with objective sicca symptoms · 2026 · DOIThe study is limited to a single tertiary neuromuscular center. The study does not provide a comprehensive review of the literature. The study does not discuss the cost-effectiveness of muscle biopsy.
Muscle biopsy in genomic era: real-world diagnostic and clinical implications over 10 years · 2026 · DOIThe study is limited to a single case report, which may not be representative of all MRH cases. The treatment of the patient's MRH was not effective in improving symptoms, despite the use of surgery and chemotherapy for the associated bladder cancer.
Malignancy-associated Multicentric Reticulohistocytosis Mimicking Dermatomyositis-like Features, with a Significant Ultrasound Finding · 2026 · DOIThe diagnosis of MRH can be challenging due to similarities with autoimmune diseases. The treatment of MRH is not well established, and the use of surgery and chemotherapy for associated malignancies may not always be effective.
Malignancy-associated Multicentric Reticulohistocytosis Mimicking Dermatomyositis-like Features, with a Significant Ultrasound Finding · 2026 · DOIThere is a need for early recognition and treatment of anti-MDA5 dermatomyositis. The disease has a poor prognosis and requires aggressive management.
Future research should investigate the mechanisms by which myositis-specific autoantibodies contribute to the development of cancer in dermatomyositis patients. Studies should explore the potential of myositis-specific autoantibodies as biomarkers for malignancy risk in dermatomyositis patients.
Malignancy Risk and Myositis-Specific Autoantibodies in Dermatomyositis: A Comprehensive Review · 2026 · DOIFurther studies are needed to fully understand the role of GZMB+CD4+ CTLs in the pathogenesis of DM and ASS. The clinical utility of GZMB+CD4+ CTLs as a biomarker for DM and ASS should be further evaluated. The potential of targeting GZMB+CD4+ CTLs as a therapeutic strategy for DM and ASS should be explored.
Dermatomyositis and antisynthetase syndrome disease activity is associated with the expansion of peripheral GZMB+CD4+ cytotoxic T cells · 2026 · DOIThe role of CD4+ cytotoxic T lymphocytes in the pathogenesis of DM and ASS is insufficiently defined. There is a need for innovative diagnostic and therapeutic approaches for DM and ASS. The current understanding of the immune responses involved in DM and ASS is incomplete.
Dermatomyositis and antisynthetase syndrome disease activity is associated with the expansion of peripheral GZMB+CD4+ cytotoxic T cells · 2026 · DOIThe study is limited to a single case report, and further studies are needed to confirm the efficacy of the treatment approach. The patient's anti-MDA5 antibody levels were not serially assessed during the active phase, making it difficult to evaluate the relationship between changes in antibody levels and clinical improvement.
Improvement of Refractory Cutaneous Ulcers and Pulmonary Arterial Hypertension Following Rituximab and Mycophenolate Mofetil Therapy in Anti-melanoma Differentiation-Associated Gene 5 (Anti-MDA5) Antibody-Positive Dermatomyositis · 2026 · DOIThe pathophysiology of hepatic involvement in dermatomiositis por anti-MDA5 is not fully understood. There is a need for further studies to investigate the relationship between hepatic involvement and patient outcomes.
Esteatosis hepática en la presentación inicial de dos pacientes con dermatomiositis por anti-MDA5 · 2026 · DOI
Most-cited papers in Inflammatory Myopathies and Dermatomyositis
- Polymyositis and Dermatomyositis · New England Journal of Medicine · 1975 · 4,054 citations
- CD19 CAR T-Cell Therapy in Autoimmune Disease — A Case Series with Follow-up · New England Journal of Medicine · 2024 · 945 citations
- 272nd ENMC international workshop: 10 Years of progress - revision of the ENMC 2013 diagnostic criteria for inclusion body myositis and clinical trial readiness. 16–18 June 2023, Hoofddorp, The Netherlands · Neuromuscular Disorders · 2024 · 91 citations
- Pathological autoantibody internalisation in myositis · Annals of the Rheumatic Diseases · 2024 · 90 citations
- Nasopharyngeal carcinoma in dermatomyositis patients: A 10-year retrospective review in Hospital Selayang, Malaysia · Reports of Practical Oncology & Radiotherapy · 2014 · 23 citations
- Congenital Rubella Syndrome and Diabetes: A Review of Epidemiologic, Genetic, and Immunologic Factors · American annals of the deaf · 1985 · 17 citations
- Myositis: von der Diagnose zur Therapie · Der Nervenarzt · 2023 · 4 citations
- Amyopathic Dermatomyositis Associated with Histopathological Findings of Organizing Pneumonia and Pulmonary Vasculitis · Balkan Medical Journal · 2017 · 4 citations
- Respiratory Failure in a Rare Case of Juvenile Dermatomyositis – Systemic Scleroderma Overlap Syndrome · MAEDICA – a Journal of Clinical Medicine · 2020 · 3 citations
- Overlap Syndromes in Autoimmune Connective Tissue Diseases · Acta Medica Bulgarica · 2024 · 2 citations
Most recent work
- The Diagnosis of SARS-CoV-2-Associated Myositis Requires Comprehensive Examination · International Journal of Advanced Multidisciplinary Research and Studies · 2026
- Modifying muscle metabolic dysregulation in inclusion body myositis with pioglitazone: a single-arm trial · Nature Communications · 2026
- Using B-cell-targeting drugs to achieve marked efficacy in anti-signal recognition particle myopathy: two case reports · Immunologic Research · 2026
- Early recognition and successful management of dermatomyositis-systemic lupus erythematosus overlap syndrome: case study of a young adult · Annals of Medicine & Surgery · 2026
- [Advances in the application of artificial intelligence in idiopathic inflammatory myopathies]. · PubMed · 2026
- Use of nailfold capillaroscopy for evaluation of disease activity in juvenile dermatomyositis: Results of a two-center retrospective study · World Journal of Clinical Pediatrics · 2026
- A Phase 3 Trial of Brepocitinib in Dermatomyositis · New England Journal of Medicine · 2026
- Dupilumab therapy facilitates the histopathologic diagnosis of dermatomyositis in a patient with intercurrent severe atopic dermatitis and dermatomyositis · JAAD Case Reports · 2026
- Computational Identification of ABL2, EPHB2, FLT1, and SIK1 as Therapeutic Targets in Dermatomyositis Skin via Single-Cell RNA Sequencing and Drug Repurposing · Zenodo (CERN European Organization for Nuclear Research) · 2026
- From Serendipity to Science: How Anti-HMGCR Antibodies Changed Our Understanding of Myositis · The Journal of Rheumatology · 2026
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