We present the evolution, the clinic and the treatment of a patient diagnosed with Schmidt Syndrome. Addison disease; Hashimoto's thyroiditis; Peritoneal dialysis; Polyglandular autoimmune syndrome ; Schmidt Syndrome.
Autoimmune polyglandular syndrome is a rare disease, that is defined by the presence of two or more glandular insufficiencies caused by autoimmune mechanisms and that may be associated with other pathologies and immunological phenomena. It is characterized by the presence of circulating organospecific antibodies and lymphocytic infiltration of the affected gland.
It is classified into 4 types. Polyglandular syndrome type 2 Schmidt Syndrome includes adrenal insufficiency associated with autoimmune thyroid disease or type 1 diabetes mellitus [].
A year-old woman with end-stage renal disease of unknown etiology in long-standing peritoneal dialysis who went to the emergency room for marked arterial hypotension and was subsequently diagnosed with autoimmune polyglandular syndrome. We present the evolution, the clinic and the treatment of the syndrome. A year-old patient with the following background: end-stage renal failure in the peritoneal dialysis program since March , diagnosed in with autoimmune thyroid disease with fine needle aspiration suggestive of Hashimoto's thyroiditis and presence of anti-TPO antibodies in blood.
In September , a total parathyroidectomy and bilateral total thyroidectomy was done. After the biopsy was made, she was diagnosed with classic multifocal papillary carcinoma and confirming lymphocytic thyroiditis.
The patient was treated by the endocrinology service with iodine without incidents. The patient received the following peritoneal dialysis pattern: 3 exchanges of cc with physioneal 35 to 1. An echocardiogram was performed with the following findings: undilated cardiac chambers. Hyperdynamic left ventricular function. Chest radiology: rule out acute pathology. Abdominal ultrasound: kidneys of small size 6 cm, difficult to delimit, intra-abdominal fluid in relation to peritoneal dialysis.
Standards of medical care in diabetes. Diabetes Care. Italian addison network study: update of diagnostic criteria for the etiological classification of primary adrenal insufficiency. Autoimmune polyendocrine syndromes. N Engl J Med. Gumieniak O, Farwell AP. Schmidt's syndrome and severe hyponatremia: report of an unusual case and review of the related literature.
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Contact afpserv aafp. Want to use this article elsewhere? Get Permissions. Read the Issue. Sign Up Now. Next: Iron Deficiency Anemia. Mar 1, Issue. C 11 , 13 Each condition of autoimmune polyglandular syndrome should be treated the same way they would be if they occurred separately.
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If you do not want your question posted, please let us know. Submit a new question. What causes autoimmune polyglandular syndrome type 2? Is it inherited? See answer. National Institutes of Health.
COVID is an emerging, rapidly evolving situation. Menu Search Home Diseases Autoimmune polyglandular syndrome type 2. You can help advance rare disease research! This site is in-development and may not reflect the final version. Preview the new GARD site. Other Names:. Diabetes mellitus, Addison's disease, myxedema; Multiple endocrine deficiency syndrome, type 2; Polyglandular autoimmune syndrome, type 2; Diabetes mellitus, Addison's disease, myxedema; Multiple endocrine deficiency syndrome, type 2; Polyglandular autoimmune syndrome, type 2; Autoimmune polyendocrine syndrome type 2; Schmidt syndrome; Schmidt's syndrome; APS2; PGA 2; Polyglandular deficiency syndrome type 2; Autoimmune polyglandular syndrome type II; PGA-II See More.
Summary Summary. Symptoms Symptoms. Showing of 30 View All. Type 1 diabetes. Type I diabetes. Muscular abnormality. Hair loss.
Decreased activity of gonads. Decreased parathyroid hormone secretion. Patchy loss of skin color. Absent spleen. Clouding of the lens of the eye. Cloudy lens. Chronic liver inflammation. Scar tissue replaces healthy tissue in the liver. Inability to properly digest food due to lack of pancreatic digestive enzymes.
Overactive thyroid. Underactive thyroid. According to recent epidemiological data, the frequency of PAS Type II is estimated to be 4 - 5 cases per , individuals in the general population [ 1 ]. The pattern of inheritance is autosomal dominance, it is more frequently encountered in women, and the male-to-female ratio is 1: 3 [ 2 ]. This syndrome has a peak incidence at ages 20 - 60 years, mostly in the third or fourth decade, and it is common for multiple generations to be affected by one or more component diseases.
There is familial clustering and family members of patients are often affected [ 8 ]. It is highly likely that there is a complex interaction between non-HLA loci and environmental factors [ 9 ]. Many of the endocrine disorders of PAS are adequately treated with hormonal replacement therapy if the disease is recognized early. The therapies regarding the different components of Type II PAS are similar whether they occur as single or in multiple associations with other autoimmune diseases.
Diagnosing of Schimdt's syndrome can be challenging and misleading too. Early recognition and treatment of such cases can stand between the life and death of a patient. In our case, patient did not have Type I diabetes, but he developed GH deficiency. Adrenal insufficiency and autoimmune thyroiditis were the presenting endocrinopathies as well as presumptive autoimmune hypophysitis.
This combination constitutes rare manifestations of the syndrome, especially at a young age. Very few cases of this syndrome have been reported in males.
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