Usual interstitial pneumonia in a patient with celiac disease and polyglandular syndrome IIIA

We present for the first time a patient, who from his first months of life developed several autoimmune endocrine and non disorders such as type 1 diabetes, autoimmune thyroiditis, and vitiligo [the constellation of polyglandular autoimmune syndrome IIIA (PG IIIA) manifestations], severe osteoporosis, as well as celiac disease (CD) diagnosed at the age of 9 years-old. Corticosteroids were administered in addition to gluten-free diet, because of partial response, for two decades. During the last 3 years he developed finger clubbing and progressive dyspnea on exertion and was hospitalized repeatedly because of lower respiratory tract infections. The persisting and aggravating chronic dyspnea and diffuse interstitial pattern on chest imaging, led to extensive investigation in order to further characterize his lung disease, disclosing usual interstitial pneumonia (UIP) histologic type lung involvement. On his last admission in our department because of fever and severe deterioration of respiratory status, the patient developed acute lung injury (ALI)/acute respiratory distress syndrome (ARDS), the so called UIP “acute exacerbation”, ending to death. Pneumon 2014, 27(4):345-349. Likurgos Kolilekas1 Effrosyni D. Manali2, Melpomeni Peppa3, Themistocles Chamogeorgakis4, Amalia Papadopoulou2, Christina Triantafillidou5, Konstantinos Kagouridis2, Dimitra Rontogianni6, Anna Karakatsani2, Spyros A. Papiris2 17th Pulmonary Department, Athens Chest Hospital, Greece 22nd Pulmonary Department, “Attikon” University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Greece 32nd Department of Internal Medicine, “Attikon” University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Greece 4Department of Cardiothoracic Surgery, “Attikon” University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Greece 56th Pulmonary Department, Athens Chest Hospital, Greece 6Department of Pathology, “Evangelismos” General Hospital, Athens, Greece