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[全身性免疫复合物性血管炎合并肺含铁血黄素沉着症及扩张型心肌病]

[Generalized immune-complex vasculitis combined with pulmonary hemosiderosis and dilated cardiomyopathy].

作者信息

Margulis E Ia, Savchenko V G, Idel'son L I, Pashkov V V, Smirnov A N, Vasil'ev S A, Titarenko M F, Zhirova N F

出版信息

Ter Arkh. 1990;62(7):143-8.

PMID:2147520
Abstract

The paper is concerned with observations over 3 patients in whom unusual vasculitis lay at the basis of the clinicopathological manifestations. All the patients were men of the young age. The disease debut was marked by fever, weakness, dyspnea, palpitation, cough, hemoptysis, the articulation syndrome. In two cases, there was hemorrhagic rash on the leg skin. All the patients manifested liver and spleen enlargement, two patients had lymphoadenopathy. The leading clinical symptoms included dilated cardiomyopathy, complete blockade of the inferior peduncle of His bundle and reduction of myocardial contractility. Anemia belonged to iron deficient one. The clinical examples provided indicate that immunocomplex vasculitis with evident lesions of the lungs and myocardium, not going into criteria for the known diseases, is not likely to be a casuistic rarity. Those syndromes may be associated with more or less pronounced hemosiderosis of the lungs (and, probably, of the lymph nodes, spleen and liver), with transitory or steady derangements of myocardial conduction, which attests to diffuse lesions of the myocardium possibly with both immune complexes and hemosiderin. The pathology requires further studies.

摘要

本文关注对3例患者的观察,这些患者的临床病理表现以不寻常的血管炎为基础。所有患者均为青年男性。疾病初发时表现为发热、乏力、呼吸困难、心悸、咳嗽、咯血及关节综合征。2例患者腿部皮肤出现出血性皮疹。所有患者均有肝脾肿大,2例患者有淋巴结病。主要临床症状包括扩张型心肌病、希氏束下支完全阻滞及心肌收缩力下降。贫血属于缺铁性贫血。所提供的临床实例表明,具有明显肺部和心肌病变、不符合已知疾病标准的免疫复合物性血管炎不太可能是偶发罕见病例。这些综合征可能与或多或少明显的肺部(可能还有淋巴结、脾脏和肝脏)含铁血黄素沉着症有关,伴有心肌传导的短暂或持续紊乱,这证明心肌可能同时受到免疫复合物和含铁血黄素的弥漫性损害。该病理情况需要进一步研究。

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