Category: Other - Autoimmune

Monitor: 17

17 - RARE CASE OF AUTOIMMUNE ADDISON'S DISEASE, HYPOTHYROIDISM AND LATENT AUTOIMMUNE DIABETES IN A HISPANIC MAN

Saturday, Apr 27
11:00 AM – 11:30 AM

Objective :

Autoimmune Addison's disease (AD) is a relatively uncommon clinical entity. We present a rare case of autoimmune AD, hypothyroidism and latent autoimmune diabetes (LADA) in a Hispanic man.


Methods : N/A


Results : Case Presentation
A 59-year-old man with a history of hypothyroidism and diabetes mellitus (DM) presented with progressive weakness, fatigability, anorexia and skin hyperpigmentation. He reported a history of severe hypotension, chronic hyperkalemia and weight loss. Family history was not significant. On examination the patient was emaciated, hypotensive and tachycardic. He had bluish black hyperpigmentation on the face, anterior abdomen and back. Laboratory work-up was significant for hyperkalemia- 6.0mmol/L (N: 3.5-5.1mmol/L), hyponatremia- 128mmol/L (N: 132-143mmol/L), HbA1c–9.1% (N: 4.3-6.1%), hypercalcemia- 10.4mg/dl (N: 8.6-10.3 mg/dl), hypocortisolemia- 3μg/dl (N: 6.7-22.6μg/dl), hypoaldosteronemia- 250 IU/mL (N: <5IU/mL). Celiac disease and tuberculosis were ruled out while IA-2, ZNT8, intrinsic factor and TRAB antibodies were all negative.  TPO antibody test was unequivocal at presentation. Patient was subsequently placed on hydrocortisone and fludrocortisone supplementation and continued insulin and levothyroxine with significant clinical improvement and hypercalcemia resolution.


Discussion :

There are about 6 cases per million per year of primary AD in the US. About 70-90% are believed to be autoimmune, occurring around the 4th-5th decade of life, mostly as part of a syndrome and majorly in women.  AD is a cardinal feature of polyglandular autoimmune syndrome type 2 (PAS-2) and there may also be associated autoimmune thyroid disease and or type 1 DM. Interestingly our patient’s autoimmune profile was positive only for GAD – 65, but negative for IA-2 and ZnT8; all expected to be positive in type 1 DM. Similarly, his TPO was unequivocal on presentation but normal after commencement of steroids. Hence, he does not classically fit as a case of PAS-2. It is however highly plausible that patients, particularly belonging to certain ethnic groups might not have classical presentation of syndromes. Nevertheless, he continues to be on adrenal replacement steroid therapy and he is being closely monitored.  


Conclusion :

In patients diagnosed with autoimmune AD, other possible autoimmune disorders should be thoroughly investigated. Prompt clinical suspicion and management will lead to reduced morbidity and mortality.

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Fatimah O. Bello

Resident
University of Texas RGV-DHR
Edinburg, Texas

Fatimah Bello is a 2nd year Internal Medicine Resident and is interested in pursuing an Endocrinology fellowship.

Brandon Cantazaro

Assistant Professor
University of Texas RGV-DHR

Dr. Cantazaro is an Assistant Professor and Associate Program Director at the UTRGV -DHR Internal Medicine Program

Leonardo Pozo Garcia

Resident
University of Texas RGV-DHR
McAllen, Texas

Leonardo is a first year Internal Medicine Resident at the UTRGV-DHR Internal Medicine program and is interested in pursuing a fellowship in Endocrinology.

Juan Palacios Merchan

Assistant Professor/ Endocrinologist
University of Texas RGV-DHR

Dr. Palacios is an Endocrinologist at Doctor's Hospital Renaissance, Edinburg and is also affiliated with the UTRGV School of Medicine as an Assistant Professor.