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A case of Rosai-Dorfman disease with anterior chest mass and myositis
대구파티마병원
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주영락, 강종완, 이선아, 장은영, 김건우
Introduction: Rosai-Dorfman disease, also known as the sinus histiocytosis with massive lymphadenopathy, is a rare benign histioproliferative disease of unknown etiology. Extra-lymph node involvement of Rosai-Dorfman disease is uncommon. Here, we present a case of 54-year-old female, presenting ante- rior chest pain with unilateral hip pain. Excisional biopsy revealed that the mass was consistent with the Rosai-Dorfman disease and the patient was treated successfully with systemic corticosteroid therapy. Case Report: A 54-year-old female presented with anterior chest pain and left hip pain for a month.
There was no previous underlying disease. On physical examination, there was no palpable lymph node but, local tender area on left hip coccyx area. A chest X-ray image showed mediastinal widening and chest computed tomography (CT) scan confirmed right anterior mass lesion. Laboratory examination showed that erythrocyte sediment rate 54mm/hr and C-reactive protein 0.55mg/dL. Muscle enzymes were within the normal range. PET CT scan confirmed an increased in FDG uptake in the anterior chest mass and left hip muscle (Figure 1A and 1B). Anterior chest mass was surgically removed and diffuse lym- phoplasmatic infiltration and histiocytes with phagocytized lymphocytes were seen histologically to confirm Rosai-Dorfman disease. We administered oral prednisolone at 1 mg/kg. After 3 months, follow up PET CT scan showed that decreased FDG uptake on not only anterior chest mass but left hip lesion (Figure 1C and 1D).
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Correlation between disease activity/autoantibody level and mast cell in rheumatoid arthriritis
건국대학교병원 내과
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김백천, 민홍기, 이상헌, 김해림
Background: Rheumatoid arthritis (RA) is chronic inflammatory arthritis which is induced by complex immunologic pathways. Mast cell (MC) belongs to innate immune cells, and several researches have shown increased MC infiltration in RA synovitis. MC showed dual effect on RA by im- mune-suppression when stimulated with IL-33, whereas MC could stimulate B cell response and autoantibody production and MC number of RA synovium correlated with disease activities. In present study, we aimed to evaluate the correlation between serum level of MC mediators (histamine, tryptase, and chymase) and disease activity/autoantibody levels of RA. Method: Total 20 active RA patients
(DAS-28>3.2) were enrolled in single tertiary hospital, Konkuk university medical center. Serum lev- els of histamine, tryptase, and chymase were measured by ELISA assay, and levels of acute reactants (ESR, CRP), rheumatoid factor (RF), and anti-CCP antibody (ACPA) were also checked. Correlation coefficients were measure between histamine/tryptase/chymase and RF/ACPA/ESR/CRP/DAS-28.
Results: Among 20 patients, 12 patients were female with mean age 60, and 75% were seropositive RA. Serum level of histamine, tryptase, and chymase were 101.74 ± 83.65 pg/uL, 3.94 ± 3.67 pg/uL, and 35.25 ± 12.90, respectively. Neither of correlation coefficient between MC mediators (histamine, tryptase, and chymase) and RF/ACPA/ESR/CRP/DAS-28 were significant. Conclusion: In present study, serum levels of mast cell mediators did not show significant correlation with disease activities nor autoantibodies levels. It has limitations on some aspects including study design, cross-sectional study, and small sample size. Further research including larger sample size, and including follow up data might clearly clarify the relations of systemic level of MC mediators and RA disease activ- ities/autoantibodies level. Reference 1. Suurmond J. et al. Mast cells in rheumatic disease. Eur J Pharmacol. 2016 May 5;778:116-24. 2. Rivellese F. et al. Ability of Interleukin-33- and Immune Complex-Triggered Activation of Human Mast Cells to Down-Regulate Monocyte-Mediated Immune Responses. Arthritis Rheumatol. 2015 Sep;67(9):2343-53.