Abstract
Objective Adult idiopathic isolated ACTH deficiencv(AIIAD) is an underestimated disorder which is frequently misdiagnosed.In this paper,we presented 3 new cases of AIIAD and summarized the clinical characteristics by analyzing these cases and available literature for further understanding this disease.Methods Three cases of AIIAD in our hospital from 1998 to 2003 were retrospectively identified.Data of clinical features,laboratory findings,and response to treatment were collected.The clinical characteristics and pertinent literature were reviewed and analysed.Results These 3 cases of AIIAD aged 52-68 years old were misdiagnosed and incorrectly treated for 7 months to 4 years.All three cases presented the symptoms of fatigue and loss of weight at the onset,and were misdiagnosed as climacteric syndrome,thyroiditis,and gastrointestinal disease.The prevalence of AIIAD was very low,and it was thought to be an underestimated disorder.Through analyzing the 3 cases and literature review,the clinical features of this disease were summarized as follows:(1) Most patients were aged over 40 years and had no history of glucocorticoid medication.(2) Clinical presentations were insidious,with symptoms such as fatigue and weight loss.(3) Patients usually had no excessive pigmentation.(4) Hypogonadism in men and amenorrhea in women could be present.(5) Blood chemistry usually revealed hyponatremia but rarely hyperkalemia.(6) Blood lymphocytosis and eosinophilia were common.(7) Plasma ACTH and cortisol levels markedly decreased with normal secretion of other pituitary hormones.(8) High prevalence of thvroid disorder or positive anti-thyroid autoantibodv.(9) Usually the other accompanied autoimmune diseases might be ameliorated with glucocorticoid replacement.(10) MRI revealed no structural pituitary defects except for empty sella.(11) No evidence of infiltration,trauma,surgery,infection,and radiotherapy of the pituitary gland was found.Conclusions AIIAD is an unspecified and underestimated condition with quite high incidence of misdiagnosis.Patients with unexplained hyponatremia,fatigue,and weight loss should be evaluated for the possibility of AIIAD.Fully understanding clinical features of AIIAD will help to improve the diagnosis and reduce misdiagnosis. Key words: Adult idiopathic isolated ACTH deficiency; Clinical features; Diagnosis
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