title: [Autoimmune polyglandular syndromes - literature review and analysis of clinical course in chosen cases].
abstract: Autoimmune polyglandular syndromes (APS) are the conditions characterized by coexistence of at least two organ-specific endocrine autoimmune disorders. The syndromes often coexist with connective tissue diseases with the presence non-organ specific antibodies. The aim of the study was to present two clinical cases of polymyositis and dermatomyositis in the course of APS type 3d. Case 1, a 24-year-old woman, with diabetes mellitus type 1 was diagnosed at the age of 17 years and polymyositis recognized at the age of 24 years based on clinical manifestations and additional tests (proximal muscle weakness, typical electromyographic changes, typical histopathological changes in skin and muscles biopsy, elevated muscle enzymes) accompanied by interstitial lung disorder. Moreover, Hashimoto`s autoimmune thyroiditis was diagnosed. Case 2, a 47-year-old man, with a several-year history of diabetes mellitus type 1, diagnosed with dermatomyositis and autoimmune thyroiditis. The immunosuppressive therapy was instituted in both cases, which reduced the symptoms of connective tissue disease. To sum up, about 25-30% of patients are affected by extraglandular autoimmune diseases, including connective tissue diseases, in the course of APS. An interdisciplinary approach is required in this group of conditions due to a multitude of disorders as well as diagnostic and therapeutic difficulties.
authors.affiliation: Katedra i Klinika Reumatologii i Układowych Chorób Tkanki Łącznej, Uniwersytet Medyczny, Lublin.
authors.forename: Ewa
authors.initials: E
authors.lastname: Wielosz
authors.affiliation: Katedra i Klinika Reumatologii i Układowych Chorób Tkanki Łącznej, Uniwersytet Medyczny, Lublin.
authors.forename: Maria
authors.initials: M
authors.lastname: Majdan
authors.affiliation: 2Katedra i Klinika Endokrynologii, Uniwersytet Medyczny, Lublin.
authors.forename: Maria
authors.initials: M
authors.lastname: Kurowska
authors.affiliation: Katedra i Klinika Reumatologii i Układowych Chorób Tkanki Łącznej, Uniwersytet Medyczny, Lublin.
authors.forename: Mariusz
authors.initials: M
authors.lastname: Piotrowski
authors.affiliation: Katedra i Klinika Reumatologii i Układowych Chorób Tkanki Łącznej, Uniwersytet Medyczny, Lublin.
authors.forename: Anna
authors.initials: A
authors.lastname: Wójcik
mesh_terms: D003922:Diabetes Mellitus, Type 1 / Q000150:complications* / Q000188:drug therapy; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008297:Male; D008875:Middle Aged; D016884:Polyendocrinopathies, Autoimmune / Q000150:complications* / Q000188:drug therapy* / Q000628:therapy; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents