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一位 60 岁男性,表现为复发性多软骨炎伴噬血细胞性淋巴组织细胞增生症。

A 60-year-old Man Presented with Relapsing Polychondritis with Haemophagocytic Lymphohistiocytosis.

机构信息

Dr Abul Khair Ahmedullah, Associate Professor, Department of Rheumatology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh; E-mail:

出版信息

Mymensingh Med J. 2023 Jan;32(1):261-264.

Abstract

Relapsing polychondritis is a rare autoimmune disorder of unknown etiology, which can affect multiple organs. It usually presents with involvement of elastic cartilage of ear and nose and involvement of other organs like kidney and central nervous system. Here, we report a case of 60-year-old man, who initially presented with fever, polyarthritis, erythema nodosum and painful swelling and redness of both external ears admitted in Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh on 13th December 2019. Two days after hospitalization, he developed sudden onset of proptosis with ophthalmoplegia. After evaluation, he was diagnosed as relapsing polychondritis with haemophagocytic lymphohistiocytosis and was treated with high dose prednisolone. His clinical condition and laboratory parameters significantly improved after treating with prednisolone during follow up.

摘要

复发性多软骨炎是一种病因不明的罕见自身免疫性疾病,可影响多个器官。它通常表现为耳和鼻的弹性软骨受累,以及肾脏和中枢神经系统等其他器官受累。在这里,我们报告了一例 60 岁男性患者的病例,他于 2019 年 12 月 13 日首次在孟加拉国达卡的 Bangabandhu Sheikh Mujib 医学大学(BSMMU)就诊,当时表现为发热、多发性关节炎、结节性红斑以及双侧外耳疼痛性肿胀和发红。住院后两天,他突发眼球突出伴眼肌麻痹。经过评估,他被诊断为复发性多软骨炎伴噬血细胞性淋巴组织细胞增多症,并接受了大剂量泼尼松龙治疗。在随访期间,他接受泼尼松龙治疗后,临床状况和实验室参数显著改善。

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