CLINICAL AND PARACLINICAL CHARACTERISTICS OF JUVENILE DERMATOMYOSITIS AT THE VIETNAM NATIONAL CHILDREN'S HOSPITAL FROM 2010 TO 2022
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Abstract
Objectives: This study aims to describe the clinical and paraclinical characteristics of pediatric patients diagnosed with JDM at the Vietnam National Children's Hospital. Methods: A retrospective study was conducted on 50 pediatric patients diagnosed with JDM from 2010 to 2022 at the Department of Immunology-Allergy-Rheumatology, Vietnam National Children's Hospital. Clinical manifestations, laboratory findings, imaging results, and disease severity were analyzed using descriptive statistical methods. Results: The median age at diagnosis was 6.5 years, with a female-to-male ratio of 1.7:1. The median time from symptom onset to diagnosis was 5.5 months. The most common systemic symptom was fatigue (78%). Skin involvement included Gottron’s papules (64%), heliotrope rash (40%), and periungual capillary changes (62%). Muscle weakness was observed in 98% of patients, predominantly affecting proximal limb muscles (98%), pelvic girdle (80%), and shoulder girdle (72%). According to the MMT8 (Manual Muscle Testing-8) scale, 90.9% of patients had severe muscle weakness (MMT8 score ≤ 63), while 9.1% had moderate muscle weakness (MMT8 score 64–71). Gastrointestinal involvement was present in 36% of cases, primarily with dysphagia (24%). Pulmonary involvement was rare (6%), and only one case (2%) had pericardial effusion. Conclusions: JDM in Vietnamese children presents with classic dermatologic and muscular manifestations, but systemic involvement is relatively uncommon. Early recognition and timely intervention are crucial in improving long-term outcomes. Further research is needed to optimize diagnostic and therapeutic approaches for JDM in Vietnam. Keywords: Juvenile dermatomyositis, autoimmune myopathy, Gottron’s papules, heliotrope rash, MMT8.
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Keywords
Juvenile dermatomyositis, autoimmune myopathy, Gottron’s papules, heliotrope rash, MMT8
References

2. Nguyễn Thị Oanh (2011). Nghiên cứu sử dụng cyclophosphamid phối hợp với methylprednisolon điều trị viêm da cơ có tổn thương phổi kẽ. Luận văn tốt nghiệp Thạc sĩ Y khoa, Đại học Y Hà Nội.

3. Nguyễn Thị Phương Thủy (2015). Nghiên cứu đặc điểm lâm sàng, cận lâm sàng và một số thay đổi miễn dịch trong bệnh viêm đa cơ và viêm da cơ. Luận văn tốt nghiệp Tiến sĩ Y khoa, Đại học Y Hà Nội.

4. Nguyễn Ngọc Chìu (2006). Đặc điểm lâm sàng và xét nghiệm của bệnh viêm da cơ điều trị tại khoa Dị ứng miễn dịch lâm sàng Bệnh viện Bạch Mai 1998-2005. Luận văn tốt nghiệp Bác sĩ đa khoa, Đại học Y Hà Nội.

5. Đặng Quốc Hương (2009). Nghiên cứu các tổn thương phổi ở bệnh nhân viêm da cơ. Luận văn Thạc sĩ Y khoa, Đại học Y Hà Nội.

6. Spencer C.H., Rouster-Stevens K., Gewanter H., et al. (2017). Biologic therapies for refractory juvenile dermatomyositis: five years of experience of the Childhood Arthritis and Rheumatology Research Alliance in North America. Pediatr Rheumatol Online J, 15(1), 50.

7. Aggarwal R., Bandos A., Reed A.M., et al. (2014). Predictors of Clinical Improvement in Rituximab-Treated Refractory Adult and Juvenile Dermatomyositis and Adult Polymyositis. Arthritis Rheumatol, 66(3), 740–749.

8. Oddis C.V., Reed A.M., Aggarwal R., et al. (2013). Rituximab in the Treatment of Refractory Adult and Juvenile Dermatomyositis and Adult Polymyositis: A Randomized, Placebo-phase Trial. Arthritis Rheum, 65(2), 314–324.

9. Wienke J., Deakin C.T., Wedderburn L.R., et al. (2018). Systemic and Tissue Inflammation in Juvenile Dermatomyositis: From Pathogenesis to the Quest for Monitoring Tools. Front Immunol, 9.
