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Thymome associé aux maladies autoimmunes, 85 cas et revue de la littérature

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Langue d'expression : français, anglais
Date de parution :  2015

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To describe autoimmune diseases in patients with thymoma; and 2) to perform a literature review concerning this issue. Hospices Civils de Lyon Pathological records from 2005 to 2011 were reviewed to identify patients with thymoma, either benign or malignant. All the patients who had thymoma and autoimmune phenomena were analyzed. The diagnosis of autoimmune diseases was based on both clinical and serological findings. 46 of the 85 (54.1%) cases of thymoma had an AD including: myasthenia gravis (MG) (n=33), Hashimoto's thyroiditis (n=4), Isaac’s syndrome (n=3), Morvan syndrome (n=2), pure red cell aplasia (n=2), systemic lupus (n=2), lichen planus (n=2), and one case of each following conditions: aplastic anemia, autoimmune hemolytic anemia, Good’s syndrome, pemphigus, autoimmune hepatitis, Graves’ disease, limbic encephalitis and inflammatory myopathy. Six patients (7%) presented at least 2 ADs. The median age at the diagnosis of thymoma was 58 years (21-82 years) and 53 years (21-81 years) at the diagnosis of AD. The median duration of follow-up after surgery was 60 months (3-241 months). According to the WHO classification, there were 12 cases class A thymoma, 44 class B, 27 class AB, one carcinoma and one carcinoid tumor. In 33 patients the diagnosis of AD preceded the diagnosis of thymoma, in 7 patients thymoma was diagnosed at the same time as the AD and 7 patients had been operated when they developed an AD. There were two cases of exacerbation of AD immediately after surgery. MG patients and another AD patients were statistically similar in terms of age, gender and duration of follow-up. Thymectomy was ineffective in 14 MG patients (41.2 %) who presented a relapse after surgery and in 6 patients with another AD (64.7%). The first occurrence of an AD was observed despite thymectomy especially in the youngest patients (median age of 35 years) (p<0.05). One single patient with MG developed another AD after thymectomy. The presence of MG or another AD before or concomitantly with thymoma was not a predisposition of the development of an AD after thymectomy. We describe here the outcome of a large series of AD related to thymoma. Our results confirm previous data concerning an AD occurrence in patients with thymoma and suggest individualized subgroups at risk to develop autoimmune manifestations after thymectomy


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