Case Presentation: 37 year old Hispanic male with Hyperlipidemia presented with diffuse abdominal pain and cramping for 2 days duration. Physical exam showed facial hyper pigmentation, soft palate – 2×2 mm hyper pigmented macules, hyper pigmentation on nails of all extremities. Patient had stocky neck without Goiter and scant axillary hair. Significant laboratory findings were: Serum Sodium in range of 122 – 130, hyperkalemia (peaked at 7). Please refer to table 1 for Endocrine work up. Initial CT abdomen showed dilated appendix to 14 mm concerning for appendicitis and normal adrenal glands. Serum Sodium was 122 and was in range of 125 – 130 during the hospital course. Renal workup was negative and TSH was high (5.81), hence was started on Synthroid. Also hyperkalemia was treated with Kayexalate. On medicine floor, patient became hypotensive and potassium increased to 7 with peaked T waves on EKG and was monitored in telemetry unit. ACTH stimulation test along with other Endocrine workup was done as recommended by Endocrinology and started on fludrocortisone, hydrocortisone.
Discussion: Polyglandular syndromes type 2 involves multiple endocrine glands. Genetic factors tend to play a major role, PGA2 is more prevalent than PGA1 and is associated with HLA-DR3 and HLA-DR4 haplotypes. Environmental like viral infections, dietary factors are thought to trigger the precipitation of the disease. Presents in early adulthood, peaks in third and fourth decade, more common in women. It may be associated with several other autoimmune diseases like Vitiligo, chronic atrophic gastritis, Alopecia, autoimmune hepatitis, Rheumatoid arthritis. More commonly patients present with autoimmune adrenal insufficiency (100%), autoimmune thyroid disease (69-82%) and Diabetes Mellitus (30-52%). Most of the patients present to the hospital with symptoms of adrenal insufficiency like weakness, anorexia, nausea, vomiting, abdominal pain, salt craving, diarrhea, constipation and Hypovolemia.
Conclusions: Schmidt’s syndrome is an autoimmune disease involving Adrenal insufficiency with thyroid glands and/ or Diabetes mellitus- Insulin dependent. Very few cases have been reported so far which can be attributed to its unusual presentation and rarity. We are reporting a case of a young Hispanic male admitted for abdominal pain and found to have incidental Hyponatremia which led to the diagnosis of this rare syndrome where thyroid replacement before hydrocortisone precipitated adrenal crisis. Patient was started on adrenal replacement therapy and work up was consistent with the diagnosis of Type II polyglandular autoimmune syndrome (PGA type II).