Tena SML, Artigas C, Assia-Zamora S, Leyva A, Gutiérrez R, Rembao BD, Fernández VF

Language: Spanish
References: 20
Page: 133-137
PDF size: 225.69 Kb.

ABSTRACT

The autoimmune polyglandular syndrome (APS) is characterized by a variable coexistence of several autoimmune diseases, affecting predominantly endocrine glands. The diagnosis is established when two out of three of these symptoms are present. We case report a 30 year old man with controlated and asyntomatic myasthenia gravis. He presented a ocular pain and progressive visual disturvances. The CT-Scan was normal and the chest X-ray showed no mediastinal abnormality, however, chest computed tomography (CT) showed a soft tissue mass in the thymus, he develop profound peripheral neuropathy, and ventilatory muscle failure, and he died despite aggressive out come. Autopsy signs of neurogenic atrophy confined to the ventilatory muscles. Bronchoneumony with bronchoaspirations sings. Thymolipoma mediastinal and necrotic pancreatitis. Lynfocyticic tiroiditis and linfocytic infiltrated was also found in parotid, and suprarenal glands. This rare association between with autoimmune polyglandular syndrome type II with myatenia gravis and thymolipoma and necrotic pancreatitis is a very rare association. Bronchoneumony is usually the case of dead in patients with myasthenia gravis. Few cases report has been published with those rare autopsies findings and this is case is the firth case with thymolipoma and necrotic pancreatitis.

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