The adult onset Still’s disease is a rare affection characterised by occurrence of fever, arthralgia or arthritis and evanescent cutaneous eruption. Multiple other systemic lesions make the diagnosis more difficult. ...The adult onset Still’s disease is a rare affection characterised by occurrence of fever, arthralgia or arthritis and evanescent cutaneous eruption. Multiple other systemic lesions make the diagnosis more difficult. Several diagnostic criteria were formulated to confirm this disease. The physiopathology of the adult onset Still’s disease is not well elucidated. However, several studies based on new facts in physiopathology, improved the therapy of refractory forms for which the biotherapy was an interesting alternative. The TNF alpha receptor antagonists are efficient on systemic and articular manifestations of this disease and allow a corticosteroid’s saving. Tocilizumab (interleukin 6 receptor antagonist), and Anakinra (interleukin 1 receptor antagonist) are also new promising treatments for resistant forms.展开更多
The adult onset Still’s disease is a rare inflammatory pathology of unknown pathogeny. The clinical features are variable. The diagnosis is difficult since exclusion of infectious, systemic and tumoral pathologies sh...The adult onset Still’s disease is a rare inflammatory pathology of unknown pathogeny. The clinical features are variable. The diagnosis is difficult since exclusion of infectious, systemic and tumoral pathologies should be done. The articular complications are frequent and can be revelatory of this pathology. The articular prognosis depends on the diagnosis delay and the treatment efficiency. Our study aims to analyze different aspects of articular manifestations complicating adult onset Still’s disease to define epidemiological, clinical and evolving characteristics of these complications. It was a cross-sectional study concerning 20 cases of adult onset Still’s disease diagnosed from 1990 to 2015 in the internal medicine A department of Charles Nicolle Hospital in Tunis, meeting Yamaguchi criteria. We identified clinical, radiological, evolving and therapeutic profile of the articular manifestations occurred in these patients. There were 13 women and 7 men. The average age was 25 years. The arthralgias were reported in all cases;while, the arthritis interested fifteen patients. A hand deformation was found in four patients. A wrist ankylosis was noted in one case and a flexion elbow in one patient. The standard articular radiographs were normal in twelve cases. The treatment associated essentially non-steroidal anti-inflammatory and/or corticosteroids and/or methotrexate. Concerning the evolving profile, the monocyclic form was present in 25% of the cases, the intermittent form in 45% and the chronic articular form in 30% of our patients. The adult onset Still’s disease is rare and heterogeneous. The articular disturbances are frequent and have various outcomes.展开更多
目的总结成人Still病(adult-onset Still disease,AOSD)病理形态特征、诊断及鉴别诊断,避免误诊误治。方法回顾分析3例AOSD淋巴结病理形态学特征、免疫表型、分子检测结果。结果病例1淋巴结活检组织见显著的淋巴滤泡增生,伴滤泡间区增生...目的总结成人Still病(adult-onset Still disease,AOSD)病理形态特征、诊断及鉴别诊断,避免误诊误治。方法回顾分析3例AOSD淋巴结病理形态学特征、免疫表型、分子检测结果。结果病例1淋巴结活检组织见显著的淋巴滤泡增生,伴滤泡间区增生;生发中心可见星空现象,未见明显组织细胞增生灶及浆细胞、中性粒细胞等;副皮质区CD3、CD5 T淋巴细胞阳性,CD20、CD79α染色提示B淋巴细胞主要位于滤泡区,CD21滤泡树突细胞阳性,CD68组织细胞阳性。病例2淋巴结穿刺组织中可见副皮质区增生,滤泡数目减少、体积缩小,未见明显组织细胞增生灶及浆细胞、中性粒细胞等;副皮质区CD3、CD5 T淋巴细胞阳性,滤泡区CD20、CD79αB淋巴细胞阳性。病例3淋巴结穿刺组织中见淋巴结正常结构部分保存,副皮质区淋巴组织弥漫性增生,组织细胞增生呈片状伴部分坏死,坏死区可见明显核碎屑、散在浆细胞及嗜酸性粒细胞,未见明显中性粒细胞浸润;CD21、CD23滤泡树突细胞阳性,部分淋巴细胞Bcl-2、Bcl-6、CD3、CD5、CD20、CD79α、多发性骨髓瘤致癌蛋白1(multiple myeloma protein 1,MUM1)阳性,Ki-67增殖指数较高(约70%阳性),少量浆细胞CD138阳性,个别细胞CD1α阳性,CD10、CyclinD1阴性,组织细胞骨髓过氧化物酶(myeloperoxidase,MPO)阳性,原位杂交EBER阴性。3例病例TCR基因重排、IG基因重排结果均为阴性。结论AOSD免疫表型多样,须综合临床、病理形态特点及免疫表型,除外感染性疾病、恶性肿瘤、淋巴瘤等疾病后诊断。展开更多
文摘The adult onset Still’s disease is a rare affection characterised by occurrence of fever, arthralgia or arthritis and evanescent cutaneous eruption. Multiple other systemic lesions make the diagnosis more difficult. Several diagnostic criteria were formulated to confirm this disease. The physiopathology of the adult onset Still’s disease is not well elucidated. However, several studies based on new facts in physiopathology, improved the therapy of refractory forms for which the biotherapy was an interesting alternative. The TNF alpha receptor antagonists are efficient on systemic and articular manifestations of this disease and allow a corticosteroid’s saving. Tocilizumab (interleukin 6 receptor antagonist), and Anakinra (interleukin 1 receptor antagonist) are also new promising treatments for resistant forms.
文摘The adult onset Still’s disease is a rare inflammatory pathology of unknown pathogeny. The clinical features are variable. The diagnosis is difficult since exclusion of infectious, systemic and tumoral pathologies should be done. The articular complications are frequent and can be revelatory of this pathology. The articular prognosis depends on the diagnosis delay and the treatment efficiency. Our study aims to analyze different aspects of articular manifestations complicating adult onset Still’s disease to define epidemiological, clinical and evolving characteristics of these complications. It was a cross-sectional study concerning 20 cases of adult onset Still’s disease diagnosed from 1990 to 2015 in the internal medicine A department of Charles Nicolle Hospital in Tunis, meeting Yamaguchi criteria. We identified clinical, radiological, evolving and therapeutic profile of the articular manifestations occurred in these patients. There were 13 women and 7 men. The average age was 25 years. The arthralgias were reported in all cases;while, the arthritis interested fifteen patients. A hand deformation was found in four patients. A wrist ankylosis was noted in one case and a flexion elbow in one patient. The standard articular radiographs were normal in twelve cases. The treatment associated essentially non-steroidal anti-inflammatory and/or corticosteroids and/or methotrexate. Concerning the evolving profile, the monocyclic form was present in 25% of the cases, the intermittent form in 45% and the chronic articular form in 30% of our patients. The adult onset Still’s disease is rare and heterogeneous. The articular disturbances are frequent and have various outcomes.
文摘目的总结成人Still病(adult-onset Still disease,AOSD)病理形态特征、诊断及鉴别诊断,避免误诊误治。方法回顾分析3例AOSD淋巴结病理形态学特征、免疫表型、分子检测结果。结果病例1淋巴结活检组织见显著的淋巴滤泡增生,伴滤泡间区增生;生发中心可见星空现象,未见明显组织细胞增生灶及浆细胞、中性粒细胞等;副皮质区CD3、CD5 T淋巴细胞阳性,CD20、CD79α染色提示B淋巴细胞主要位于滤泡区,CD21滤泡树突细胞阳性,CD68组织细胞阳性。病例2淋巴结穿刺组织中可见副皮质区增生,滤泡数目减少、体积缩小,未见明显组织细胞增生灶及浆细胞、中性粒细胞等;副皮质区CD3、CD5 T淋巴细胞阳性,滤泡区CD20、CD79αB淋巴细胞阳性。病例3淋巴结穿刺组织中见淋巴结正常结构部分保存,副皮质区淋巴组织弥漫性增生,组织细胞增生呈片状伴部分坏死,坏死区可见明显核碎屑、散在浆细胞及嗜酸性粒细胞,未见明显中性粒细胞浸润;CD21、CD23滤泡树突细胞阳性,部分淋巴细胞Bcl-2、Bcl-6、CD3、CD5、CD20、CD79α、多发性骨髓瘤致癌蛋白1(multiple myeloma protein 1,MUM1)阳性,Ki-67增殖指数较高(约70%阳性),少量浆细胞CD138阳性,个别细胞CD1α阳性,CD10、CyclinD1阴性,组织细胞骨髓过氧化物酶(myeloperoxidase,MPO)阳性,原位杂交EBER阴性。3例病例TCR基因重排、IG基因重排结果均为阴性。结论AOSD免疫表型多样,须综合临床、病理形态特点及免疫表型,除外感染性疾病、恶性肿瘤、淋巴瘤等疾病后诊断。
