MEGAESOPHAGUS IN A PATIENT WITH AUTOIMMUNE POLYGLANDULAR SYNDROME TYPE-II

Dysphagia and vomiting are frequently present in untreated Addison's d isease. These non-specific symptoms may be due either to the metabolic disorder and myopathy or to disorders associated with Addison's disea se. We describe a patient with autoimmune adrenal failure as a feature of autoimmune polyglandular syndrome (APS) type II. This patient was referred initially because of megaoesophagus. The association of megao esophagus with Addison's disease or any of the three types of APS has not previously been described in humans. The association of megaoesoph agus and adrenal failure, however, is known to occur in Allgrove's syn drome, a disease with primary manifestation in childhood characterized by adrenal failure, achalasia and alacrimia. Moreover, there are seve ral reports on the association of megaoesophagus with adrenocortical i nsufficiency and other autoimmune endocrine diseases in dogs. Vomiting and dysphagia usually resolve with hormone substitution in patients w ith isolated Addison's disease. In our patient these symptoms disappea red in spite of the radiological persistence of megaoesophagus, which might have been overlooked if the diagnosis of Addison's disease had b een made earlier. The occurrence of megaoesophagus might be more commo n than previously suspected and we suggest a systematic search for sim ilar findings in other patients with autoimmune Addison's disease, eve n when minor dysphagia is present.