Case Report

Atypical Autoimmune Polyglandular Syndrome Type 3 Overlapped by Chronic HCV Infection Resulting in Carcinogenesis and Fatal Infection

Authors: Ioannis S. Elefsiniotis, MD, PhD, Georgios Papatsimpas, MD, George D. Liatsos, MD, PhD, Anna Tasidou, MD, Antonios Moulakakis, MD, PhD

Abstract

The case of a woman with insulin-dependent diabetes mellitus, autoimmune thyroiditis, atrophic gastritis, pernicious anemia, and immunologic thrombocytopenic purpura consisting of autoimmune polyglandular syndrome type 3 associated with a history of gonadal failure is reported. Hepatitis C viral infection added xerophthalmia, lymphocytic sialadenitis, and exacerbation of idiopathic thrombocytopenic purpura. This unique disease constellation was complicated with splenic marginal zone lymphoma and gastric carcinoids. A lung infection, initially treated on an outpatient basis, proved fatal to the patient.


Key Points


* Hepatitis C viral infection (HCV) can exacerbate autoimmunity in autoimmune polyglandular syndrome (APS) type 3.


* Patients with APS 3 and HCV need close follow-up for the possible development of related malignancies.


* Reports of high fever in cases of APS type 3 and HCV should be taken seriously; patients should be hospitalized immediately, and investigations for rare infections, including diffuse interstitial lung disease, should be performed.

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