Laura Gallego , Jhon Buitrago , Diana Guavita-Navarro , Jairo Cajamarca-Barón , Ana María Arredondo , José Fernando Polo Nieto , Juan Pablo Castañeda-González , Alejandro Escobar
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引用次数: 0
摘要
斯约格伦综合征(SS)是一种继发于淋巴细胞浸润的腺体和/或腺外损害的自身免疫性疾病。临床病程因遗传易感性、合并症、患者年龄和环境风险因素而异。淋巴细胞的增殖和分化是 SS 演变为血液恶性肿瘤或淀粉样变性疾病的关键因素。淀粉样变性是慢性感染、炎症、肿瘤和血液淋巴过程中产生的可溶性血浆蛋白异常积累的继发性疾病。淀粉样变性的临床表现各不相同,取决于构成蛋白和患者的年龄;可能有腺体或腺外、局部或全身损害。在受影响的器官中,肺部受累因其病程和临床表现多变而成为诊断和治疗的难题。以下病例报告了一名 70 多岁的女性患者,她患有 SS 和淀粉样变性,在肺部有腺体和腺体外表现。
Haematological complication with pulmonary impact in a patient with Sjögren’s syndrome
Sjögren’s Syndrome (SS) is an autoimmune pathology with glandular and/or extraglandular compromise, secondary to the infiltration of lymphoid cells. The clinical course varies depending on genetic susceptibility, comorbidities, patient’s age, and environmental risk factors. Lymphoid proliferation and differentiation are key factors in the progression of SS to haematological malignancies or amyloidosis. Amyloidosis is a secondary entity to the aberrant accumulation of soluble plasma proteins, derived from chronic infectious, inflammatory, neoplastic and haematolymphoid processes. The clinical manifestations vary and depend on the constitutive protein and the age of the patient; and may have glandular or extraglandular, local, or systemic compromise. Among the affected organs, pulmonary involvement poses a diagnostic and therapeutic challenge due to its variable course and clinical manifestation. The following is a case report of a woman over 70 years old, with SS and amyloidosis with glandular and extra glandular manifestations at pulmonary level.