Hypophysitis Research Center

Hypophysitis News

Was the first patient with Autoimmune Hypophysitis described at JHU?

All the world literature cites Goudie and Pinkerton as the authors that reported the first case of autoimmune hypophysitis (specifically, lymphocytic adenohypophysitis). There is no doubt that they were the first to postulate the autoimmune nature of this condition, at a time when the field of autoimmunity had just begun. They describe a 22 year old woman who died 14 months after delivering her second child, probably due to adrenal insufficiency. A year after delivery the patient felt increasingly tired and listless and noticed an increased size of her neck. She was admitted to the hospital because of severe lower abdominal pain, radiating to the right iliac fossa, associated with vomiting and diarrhea. She was brought to the operating room for supsected appendicitis. Surgery revealed an acutely inflamed, gangrenous appendix that, however, had not ruptured. The appendix was removed but 8 hours after the operation the patient developed peripheral circulatory shock and quickly died. The autopsy revealed a firm enlarged thyroid gland, atrophic adrenal glands, so atrophic that they could not even be found, and a small pituitary. Histology showed the classic findings of Hashimoto's thyroiditis in the thyroid gland. Surprisingly for that time, the anterior lobe of the pituitary gland was extensively infiltrated by lymphocytes and few plasma cells that in some areas aggregated to form lymphoid follicles. The endocrine cells in the areas of lymphocytic infiltration were shrunken. The authors basically described a case of what is now called autoimmune polyglandular syndrome type II. Noting the presence in the same patient of Hashimoto's thyroiditis, a more extensively characterized disease of autoimmune nature, the authors concluded the discussion by writing.

"It seems reasonable to assume that the coexistence of Hashimoto's disease and the mononuclear cells infiltration of the anterior pituitary is not fortuitous. Both may be explained by the onset of atuo-immune reaction to thyroid and pituitary antigens released during the puerperal involution of these glands"

We reviewed the records of the first 13,000 autopsies performed at the Johns Hopkins Hospital, looking for cases of Addison's disease. We found 15 cases. One of them (autopsy 12343), has in our impression what we would now call lymphocytic adenohypophysitis. Because of the historical interest of this case, we present here the entire autopsy report and some images captured from the slides prepared in the year 1932.

Clinical History (case 4 from the paper by Duff GL and Bernstein C in Bull Johns Hopkins Hospital 52: 67-83, 1933)

View the autopsy report

R.M.C., a white married woman, 36 years of age, first noticed weakness six months after her first pregnancy in 1923. Early in 1927 hear blood pressure was reported to have been 95 systolic and 62 diastolic, and in September of the same year a diffuse brown pigmentation of the skin was observed. From that time until June 1931, she experienced recurrent attacks of weakness and nausea and there was a steady loss of weight. The patient was then admitted to the Massachusetts General Hospital where she suffered a rather severe relapse. However, she showed marked improvement after treatment with large doses of extract of suprarenal cortex. The injections were discontinued on September 23, and for a time she did well without them. On October 28 1931, the patient was transferred to the Johns Hopkins Hospital. At that time there was a typical diffuse brownish pigmentation of the skin and slight buccal pigmentation. The blood pressure was 100 systolic and 60 diastolic. A relapse occurred shortly after admission to the hospital but subsided under treatment with cortical extract and after the removal of two badly infected teeth. On November 9, jaundice was first noticed and this steadily increased. The patient had a series of episodes, each lasting for several days, in which there was severe nausea, anorexia, vomiting and stupor. There was marked hypotension and a considerable secondary anemia developed. During the last days of life, oedema of the legs and ascites appeared, and there was bleeding from the gums. The patient died on December 29 1931. A complete autopsy was performed 2 hours after death. The anatomical diagnosis was as follows: Addison's disease; peculiar cirrhosis of the liver; partial destruction of the hypophysis, with round-cell infiltration; atrophy of the ovary; lymphocytic infiltration in the adrenals, kidneys, pancreas, voluntary muscles, thyroid gland and meninges; mediastinal lymphoid tumor in the thymic region.