Sarcoidosis: A Clinical Overview from Symptoms to Diagnosis

Cells

François Durupt¹  Alain Calender²  Pascal Sève³  Mathieu Gerfaud-Valentin³  ThomasEl Jammal³  Yvan Jamilloux³  Géraldine Androdias⁴  Sylvie Isaac⁵  Dominique Valeyre⁶  Loïc Boussel⁷  Yves Pacheco⁸ 

[1]Department of Dermatology, Lyon University Hospital, 69004 Lyon, France

[2]Department of Genetics, Lyon University Hospital, 69500 Bron, France

[3]Department of Internal Medicine, Lyon University Hospital, 69007 Lyon, France

[4]Department of Neurology, Service Sclérose en Plaques, Pathologies de la Myéline et Neuro-Inflammation, Hôpital Neurologique Pierre Wertheimer, Lyon University Hospital, F-69677 Bron, France

[5]Department of Pathology, Lyon University Hospital, 69310 Pierre Bénite, France

[6]Department of Pneumology, Assistance Publique-Hôpitaux de Paris, Hôpital Avicenne et Université Paris 13, Sorbonne Paris Cité, 93008 Bobigny, France

[7]Department of Radiology, Lyon University Hospital, 69004 Lyon, France

[8]Faculty of Medicine, University Claude Bernard Lyon 1, F-69007 Lyon, France

关键词: sarcoidosis;    granulomatosis;    cardiac sarcoidosis;    neurosarcoidosis;    diagnostics;    differentials;   

DOI  :  10.3390/cells10040766

来源: DOAJ

【 摘 要 】

Sarcoidosis is a multi-system disease of unknown etiology characterized by the formation of granulomas in various organs. It affects people of all ethnic backgrounds and occurs at any time of life but is more frequent in African Americans and Scandinavians and in adults between 30 and 50 years of age. Sarcoidosis can affect any organ with a frequency varying according to ethnicity, sex and age. Intrathoracic involvement occurs in 90% of patients with symmetrical bilateral hilar adenopathy and/or diffuse lung micronodules, mainly along the lymphatic structures which are the most affected system. Among extrapulmonary manifestations, skin lesions, uveitis, liver or splenic involvement, peripheral and abdominal lymphadenopathy and peripheral arthritis are the most frequent with a prevalence of 25–50%. Finally, cardiac and neurological manifestations which can be the initial manifestation of sarcoidosis, as can be bilateral parotitis, nasosinusal or laryngeal signs, hypercalcemia and renal dysfunction, affect less than 10% of patients. The diagnosis is not standardized but is based on three major criteria: a compatible clinical and/or radiological presentation, the histological evidence of non-necrotizing granulomatous inflammation in one or more tissues and the exclusion of alternative causes of granulomatous disease. Certain clinical features are considered to be highly specific of the disease (e.g., Löfgren’s syndrome, lupus pernio, Heerfordt’s syndrome) and do not require histological confirmation. New diagnostic guidelines were recently published. Specific clinical criteria have been developed for the diagnosis of cardiac, neurological and ocular sarcoidosis. This article focuses on the clinical presentation and the common differentials that need to be considered when appropriate.

【 授权许可】

Unknown