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[结节病作为一种多器官疾病]

[Sarcoidosis as a multi-organ disease].

作者信息

Kummer F, Klech H

机构信息

II. Medizinische Abteilung des Wilhelminenspitals Wien.

出版信息

Pneumologie. 1990 Feb;44 Suppl 1:158-61.

PMID:2195508
Abstract

Sarcoidosis meets the criteria for multi-organ disease, which differs from systemic disease in the following points: The preferred primary organ (lungs) is known, immunological processes take place only in the organs involved (T helper lymphocyte accumulation), the clinical picture depends upon the organ involvement, and granulomas are typical of the disorder. The clinical significance of the various organ manifestations covers a broad spectrum, which can also include a threat to life (myocardium), blindness, chronic invalidism (pulmonary fibrosis, hepatopathy, nephropathy), and cosmetic impairment (cutaneous sarcoidosis). Numerous organ manifestations have no clinical significance, and some are both rare and limited (tumorous CNS involvement). So-called overlap syndromes have characteristic features common to both multi-organ and systemic diseases. In addition to characteristic sarcoidosis manifestation in an organ, signs of a generalized disorder are also detectable. Examples are primary biliary cirrhosis, Crohn's disease, coeliac disease, Whipple's disease, TASS (Thyrotoxicosis, Addison, Sjögren, Sarcoidosis) as lymphomas and inflammatory diseases (tuberculosis). Outside of the primary organ, these manifestations are often clinically latent. Selective investigations bronchoalveolar lavage), however, can demonstrate the presence of sarcoidosis alveolitis. It is demonstrated that for the clinician, differentiation of multi-organ from systemic disease is meaningful in particular with respect to therapeutic consequences. In the individual case, however, possible overlapping must always be borne in mind.

摘要

结节病符合多器官疾病的标准,它在以下方面与全身性疾病不同:已知首选的主要器官(肺),免疫过程仅发生在受累器官(T辅助淋巴细胞积聚),临床表现取决于器官受累情况,并且肉芽肿是该疾病的典型特征。各种器官表现的临床意义涵盖广泛范围,其中还可能包括对生命的威胁(心肌)、失明、慢性残疾(肺纤维化、肝病、肾病)以及外观损害(皮肤结节病)。许多器官表现没有临床意义,有些既罕见又局限(肿瘤性中枢神经系统受累)。所谓的重叠综合征具有多器官疾病和全身性疾病共有的特征性表现。除了器官中典型的结节病表现外,还可检测到全身性疾病的体征。例如原发性胆汁性肝硬化、克罗恩病、乳糜泻、惠普尔病、TASS(甲状腺毒症、艾迪生病、干燥综合征、结节病)以及作为淋巴瘤和炎症性疾病(结核病)。在主要器官之外,这些表现通常在临床上呈潜伏状态。然而,选择性检查(支气管肺泡灌洗)可以证明结节病肺泡炎的存在。事实表明,对于临床医生而言,区分多器官疾病和全身性疾病在治疗后果方面尤其有意义。然而,在个别病例中,必须始终牢记可能存在的重叠情况。

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Pneumologie. 1990 Feb;44 Suppl 1:158-61.
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