[19-year-old patient with adrenal cortex insufficiency--only the tip of the iceberg. Polyendocrine autoimmune syndrome type II (Schmidt syndrome)].
We report on a 19-year-old woman with polyglandular autoimmune syndrome type II (APS II). She was diagnosed with addison's disease and hypothyroidism due to chronic autoimmune thyroiditis. Her mother had celiac disease and her brohter had diabetes mellitus typ 1. Chronic autoimmune thyroiditis was diagnosed in her mother, subsequently. In patients and their relatives, who have autoimmune disorders, a search for autoimmune polyglandular syndrome is crucial. Consequently, it would be appropriate that the patient and all family members are asked for clinical signs and symptoms of autoimmune disorders. Annual measurement of morning cortisol, TSH and fasting plasma glucose may useful. Screening of affected individuals as well as their first-degree relatives for celiac disease is recommended. Therapy of APS II consists of hormone replacement therapy, but thyroxin replacement may induce life-threatening adrenal failure in a patient with untreated Addison's disease. Thus, in case of doubt hydrocortisone should be given before the thyroxine administration is started.
Lipowsky
C
C
Fachbereich für Endokrinologie und Diabetologie, Departement für Innere Medizin, Kantonsspital St. Gallen. christof.lipowsky@kssg.ch
Schorl-Schweikardt
B A
BA
Kehl
Oth
O
Brändle
M
M
D000224:Addison Disease / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000328:Adult; D003348:Cortisone / Q000031:analogs & derivatives / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D005221:Fatigue / Q000209:etiology; D005260:Female; D005438:Fludrocortisone / Q000627:therapeutic use; D020022:Genetic Predisposition to Disease / Q000235:genetics; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D018908:Muscle Weakness / Q000209:etiology; D016884:Polyendocrinopathies, Autoimmune / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics; D013967:Thyroiditis, Autoimmune / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics; D013974:Thyroxine / Q000627:therapeutic use; D015431:Weight Loss
D000324:Adrenocorticotropic Hormone; D013974:Thyroxine; D005438:Fludrocortisone; D003348:Cortisone; D006854:Hydrocortisone