Polyglandular autoimmune endocrine insufficiency complicated by severe osteoporosis.
In literature different cases of polyglandular autoimmune type II syndrome (PGA II) are reported, where Addison's disease is associated with gonadal insufficiency. The lack in the production of sexual steroids causes a severe postmenopausal osteoporosis. The case we report is related to a 38-year-old woman we met in 1988 and who was suffering from deep asthenia, cramps, cutaneous hyperpigmentation, nausea, vomiting, abdominal pain, weight loss and hypotension. The biochemical data were indicative for autoimmune adrenal failure. Between 1988 and 1997 the patient developed a progressive insufficiency of other endocrine glands, leading to the classic feature of PGA II. In 1998, this clinical status was complicated by a severe osteoporosis. We thought that the sudden decrease in the bony mineral density was due to the lack of the protective role played by adrenal gland androgens in postmenopausal osteoporosis. They would directly act on the bony tissue, independently from oestrogens peripheral conversion, thus producing a stimulant effect on the bone formation. A new therapeutical approach, in case of osteoporosis, is today represented by DHEA replacement therapy in women showing low hormone levels.
Bassotti
G
G
Department of Clinical Sciences, Policlinico Umberto I, La Sapienza University, Rome, Italy.
Di Sarra
D
D
Pietrobono
D
D
Schiaffini
R
R
Battocletti
T
T
D'Amico
T
T
Gargiulo
P
P
D000224:Addison Disease / Q000209:etiology; D000328:Adult; D001247:Asthenia / Q000209:etiology; D015519:Bone Density / Q000187:drug effects; D003687:Dehydroepiandrosterone / Q000627:therapeutic use*; D005260:Female; D012739:Gonadal Steroid Hormones / Q000172:deficiency; D020249:Hormone Replacement Therapy; D006801:Humans; D006943:Hyperglycemia / Q000188:drug therapy / Q000209:etiology; D007328:Insulin / Q000627:therapeutic use; D015663:Osteoporosis, Postmenopausal / Q000188:drug therapy / Q000209:etiology*; D016884:Polyendocrinopathies, Autoimmune / Q000097:blood / Q000150:complications* / Q000175:diagnosis
D012739:Gonadal Steroid Hormones; D007328:Insulin; D003687:Dehydroepiandrosterone