A RARE CASE OF SEVERE TYPE 4 POLYGLANDULAR AUTOIMMUNE SYNDROME IN A YOUNG ADULT

Abstract

Objective: The association of type 1 diabetes mellitus with autoimmune thyroiditis or with celiac disease is frequently mentioned in literature, but the concomitant presence of these three autoimmune diseases, especially in adults, represents a rarity.

Case report: We present the case of a young man with severe generalized oedema admitted to the emergency department and diagnosed with severe hypothyroidism (TSH=100 μUI/mL, fT4 = 0.835 pmol/L) in the context of a long-lasting autoimmune thyroiditis (anti-TPO antibodies 64 UI/mL, anti-TG antibodies 17 UI/mL, the thyroid ultrasonography). At the same time, he was diagnosed with type 1 diabetes mellitus. He was also submitted to further tests which confirmed the diagnosis of celiac disease (endoscopy with intestinal mucosa biopsy, confirmed by immunological tests). The association of these three diseases slows down the process of reaching a final diagnosis and delays the adoption of a therapeutic strategy.

Conclusion: This case underlines the difficulty of differential diagnosis of severe oedema syndrome with polyserositis in a patient with polyglandular autoimmune syndrome. Whenever there is a suspicion of the association of these autoimmune diseases, the evolution of the patient is unpredictable and most medical results are highly dependent upon the decision of applying a concomitant treatment.

Keywords: polyglandular autoimmune syndrome; polyserositis; severe hypothyroidism.

Figure 1.

Facial oedema in the patient with anasarca.

Figure 2.

Ultrasonography of the right testicle (10 ml volume, normal structure) and right scrotum showing scrotal fluid.

Figure 3.

Chest X-rays showing pleural and pericardial effusions.

Figure 4.

Thyroid ultrasonography shows thyroid volume of 15 ml, hypoechogenic, non-homogeneous echostructure.

Figure 5.

X-ray of the wrist revealed the presence of fertile growing cartilage and a delayed bone age of 12 years.