title: A rare case of Addison's disease, hepatitis, thyreoiditis, positive IgG anti-tissue transglutaminase antibodies and partial IgA deficiency.
abstract: BACKGROUND
  Selective IgA deficiency (IgAD) is the most prevalent type of primary immune deficiencies, but partial IgA deficiency is even more common. Addison's disease is a rare condition associated with primary adrenal insufficiency due to infection or autoimmune destruction of the adrenals. The association between IgA deficiency and Addison's disease is very rare.
  We observed a 22-year-old male patient with marked darkening of the skin, especially on the palms and areolae, jaundice on the skin and sclera, astheno-adynamia, hypotension (80/50 mm Hg), and pain in the right hypochondrium. The laboratory investigations revealed increased serum levels of total and indirect bilirubin, AST, ALT, GGT and LDH, negative HBsAg, anti-HBc IgM, anti-HCV and anti-HAV IgM, very low serum IgA levels (0.16 g/l) with normal IgG and IgM, negative ANA, ANCA, AMA, LKM-1, anti-GAD-60, anti-IA-2, anti-thyroglobulin antibodies, a mild increase in anti-TPO antibodies titer, a marked increase in IgG anti-tissue transglutaminase antibodies, with no typical changes in cellular immunity, negative T-SPOT-TB test, HLA - A*01; B*08; DRB1*03; DQB1*02, karyotype - 46, XY.
  
  
  CONCLUSIONS
  We present a rare case of partial IgA deficiency with Addison's disease, hepatitis, thyroiditis and positive anti-tissue transglutaminase antibodies. IgAD and some autoimmune disorders share several predisposing HLA genes, thus explaining the increased prevalence of IgAD in certain patient groups.
authors.affiliation: Department of Clinical Immunology, University Hospital Alexandrovska, Medical University-Sofia, Sofia, Bulgaria.
authors.forename: Marta P
authors.initials: MP
authors.lastname: Baleva
authors.affiliation: Department of Clinical Immunology, University Hospital Alexandrovska, Medical University-Sofia, Sofia, Bulgaria.
authors.forename: Snejina
authors.initials: S
authors.lastname: Mihaylova
authors.affiliation: Department of Clinical Immunology, University Hospital Alexandrovska, Medical University-Sofia, Sofia, Bulgaria.
authors.forename: Petja
authors.initials: P
authors.lastname: Yankova
authors.affiliation: University Hospital for Endocrinology, Medical University-Sofia, Sofia, Bulgaria.
authors.forename: Iliana
authors.initials: I
authors.lastname: Atanasova
authors.affiliation: Department of Nephrology, University Hospital Alexandrovska, Medical University-Sofia, Sofia, Bulgaria.
authors.forename: Milena
authors.initials: M
authors.lastname: Nikolova-Vlahova
authors.affiliation: Department of Clinical Immunology, University Hospital Alexandrovska, Medical University-Sofia, Sofia, Bulgaria.
authors.forename: Elissaveta
authors.initials: E
authors.lastname: Naumova
keywords: Addison‘s disease; partial IgA deficiency; thyreoiditis; tissue transglutaminase antibodie