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￭ S-711 ￭
Case Report: Hemophagocytic Syndrome in a Patient with Dermatomyositis
2Division of Rheumatology, 1Department of Internal Medicine, Keimyung University Dongsan Medical Center, Daegu, Korea, 3Departments of Pathology, Keimyung University School of Medicine, Daegu, Korea
*Byung Wook Song1, So-Yeon Lee1, Ji Min Lee1, Hye-Jin Jeong1,2, Chang-Nam Son1,2, Hye Ra Jung3, Sang-Hyon Kim1,2, Ji-Min Kim1,2
Introduction: Dermatomyositis (DM) is characterized by chronic inflammation of striated muscle and characteristic cutaneous manifestations.
Hemophagocytic syndrome (HPS) is a rare life-threatening condition caused by uncontrolled activation of histiocytes resulting in prominent hemophagocytosis. Particularly, occurrence of HPS in the patients with DM is extremely rare. We report the first case of HPS in patient with DM suc- cessfully treated in Korea. Case: A 56-year-old female visited our hospital, complaining of general weakness with whole body skin rash for 2 months.
On physical examination, she had symmetric proximal muscle weakness (motor power grade 1/5 in both upper and lower extremities) and characteristic skin lesions including heliotrope rash, gottron’s papules and V sign. She also had a swallowing difficulty proven by abnormal videofluoroscopic swal- lowing test. Laboratory findings showed thrombocytopenia, elevated serum levels of muscle enzymes (creatine kinase, myoglobin, and lactate de- hydrogenase), ferritin and triglyceride. Although antinuclear antibody was positive (1:160), anti Jo-1 antibody was negative. We identified the in- flammatory myopathy through electromyography and performed a biopsy of inflamed muscle on magnetic resonance image. Based on these data, she was diagnosed with DM. We also performed bone marrow biopsy to find the cause of a microangiopathic hemolytic anemia and thrombocytopenia in peripheral blood smear. Numerous CD68-positive macrophages engulfing erythrocytes and platelets were revealed in bone marrow study. She was fi- nally diagnosed as DM with secondary HPS. After steroid pulse therapy (methylprednisolone 1 g/day) for 3 days was initiated, we continued high dose steroid therapy for 1 month. After then, we gradually tapered the steroid and started methotrexate. After 1 year of treatment, she was completely recov- ered from muscle weakness, swallowing difficulty, skin lesions and cytopenia without any other sequelae. Conclusions: With this unique case, we would like to assert that HPS should be considered when cytopenia is observed in the patients with DM and that early aggressive therapy is needed.
￭ S-712 ￭
A case of refractory oral aphthous ulcer successfully treated with adalimumab
1Department of Internal Medicine, College of Medicine, Hanyang university, 2Keimyung University Dongsan Medical Center, Daegu, 3Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases, Seoul, South Korea
*Jinyeong Kim1, Myung-jin Song1, Hye-Jin Jeong2, Soo-Kyung Cho1,3, Yoon-Kyoung Sung1,3
Oral aphthous ulcer is common lesion characterized by loss of dermal tissue with various shapes in many diseases. It is not serious symptom but may affect quality of life owing to pain and discomfort. In many cases, it is recovered spontaneously without treatment or with topical agents including anti- septics, analgesics and corticosteroid in a few days. However, rarely, there is a few cases of refractory aphthous ulcer in spite of appropriate treatment.
Recently, tumor necrosis factor (TNF) -α inhibitors have been used in treatment of severe mucosal ulcer in behcet’s disease. Herein, we report a patient suffered from refractory oral aphthous ulcers were resistant to topical and systemic agents, which were successfully treated with adalimumab. Our pa- tient had 2-years lasting oral ulcer which was not easily cured with appropriate therapy. We tried to find his underlying disease which can induce in- tractable and persisting ulcer. We should have focused on aphthous ulcer itself because we could not find specific systemic disease to him for evaluation He treated with from topical agents to systemic corticosteroid and colchicine. All theses medical treatment and even surgical repair were failed.
Moreover, we could not increase the usage of corticosteroid because of diabetes mellitus. His oral aphthous ulcer was proven refractory to conventional treatments. We chose adalimumab among the TNF-a inhibitors in spite of the cost from its off-label use, and started subcutaneous injection of adalimu- mab 40mg every 2 weeks for him. The dose of oral corticosteroid got tapered steadly out after 5th injection and completely stopped after 11th injection.
Interval of administration of adalimumab also extended from every 2 weeks to 4 weeks. After two months later starting treatment with adalimumab, the patient had an improvement of pain. And oral ulcer was completely healed within seven months of treatment. He has remained in remission state and followed up in the outpatient department maintaining adalimumab 40mg every 4 weeks without systemic corticosteroid. This study is the first to report the remission of intractable aphthous oral ulcer without underlying disease by administrating adalimumab in Korea.
