Usual interstitial pneumonia in a patient with celiac disease and polyglandular syndrome IIIA
More details
Hide details
7th Pulmonary Department, Athens Chest Hospital, Greece
2nd Pulmonary Department, “Attikon” University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Greece
2nd Department of Internal Medicine, “Attikon” University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Greece
Department of Cardiothoracic Surgery, “Attikon” University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Greece
6th Pulmonary Department, Athens Chest Hospital, Greece
Department of Pathology, “Evangelismos” General Hospital, Athens, Greece
Corresponding author
Likurgos Kolilekas   

7th Pulmonary Department, Athens Chest Hospital “Sotiria”, 152 Mesogion Ave, 11527, Athens, Greece
Pneumon 2014;27(4):345-349
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.
All the authors declare that they do not have a financial relationship with a commercial entity that has an interest in the subject of this manuscript.
Di Sabatino A, Corazza GR. Coeliac disease. Lancet 2009; 373:1480-93.
Kahaly GJ. Polyglandular autoimmune syndromes. Eur J Endocrinol 2009; 161:11-20.
Kumar V, Rajadhyaksha M, Wortsman J. Celiac disease-associated autoimmune endocrinopathies. Clin Diagn Lab Immunol 2001; 8:678-85.
Katzenstein A-L, Mukhopadhyay S, Myers JL. Diagnosis of usual interstitial pneumonia and distinction from other fibrosing interstitial lung diseases. Hum Pathol 2008; 39:1275-94.
An Official ATS/ERS/JRS/ALAT Statement. Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management. Am J Respir Crit Care Med 2011; 183:788-824.
Tzelepis GE, Toya SP, Moutsopoulos HM. Occult connective tissue diseases mimicking idiopathic interstitial pneumonias. Eur Respir J 2008; 31:11-20.
Collard HR, Moore BB, Flaherty KR, et al; Idiopathic Pulmonary Fibrosis Clinical Research Network Investigators. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2007; 176: 636-43.
du Bois RM. Strategies for treating idiopathic pulmonary fibrosis. Nat Rev Drug Discov 2010; 9:129-40.
Papiris SA, Manali ED, Kolilekas L, et al. Idiopathic Pulmonary Fibrosis Acute Exacerbations: Unravelling Ariadne's thread. Crit Care 2010; 14:246.
Papadopoulos KI, Hallengren B. Polyglandular autoimmune syndrome type III associated with coeliac disease and sarcoidosis. Postgrad Med J 1993; 69:72-5.
Hammami S, Ghédira Besbès L, Hadded S, Chouchane S, Ben Meriem CH, Gueddiche MN. Co-occurrence pulmonary haemosiderosis with coeliac disease in child. Respir Med 2008; 102:935-6.
Hwang E, McBride R, Neugut AI, Green PH. Sarcoidosis in Patients with Celiac Disease. Dig Dis Sci 2008; 53:977-81.
Lancaster-Smith MJ, Benson MK, Strickland ID. Coeliac disease and diffuse interstitial lung disease. Lancet 1971; i:473-6.
Mastruzzo C, Vancheri C, Mura DL, Poletti V, Failla M, Crimi N. A case of pulmonary Langerhans cell histiocytosis in a young woman with coeliac disease. Swiss Med Wkly 2007; 137:326-7.
Brightling CE, Symon FA, Birring SS, Wardlaw AJ, Robinson R, Pavord ID. A case of cough, lymphocytic bronchoalveolitis and coeliac disease with improvement following a gluten free diet. Thorax 2002; 57:91-2.
Mahadeva R, Flower C, Shneerson J. Bronchiectasis in association with coeliac disease. Thorax 1998; 53:527-9.
Kero J, Gissler M, Hemminki E, Isolauri E. Could TH1 and TH2 diseases coexist? Evaluation of asthma incidence in children with coeliac disease, type 1 diabetes, or rheumatoid arthritis: A register study. J Allergy Clin Immunol 2001; 108:781-3.
Cooper BT, Holmes GKT, Cooke WT. Coeliac disease and immunological disorders. BMJ 1978; 1:537-9.
Drakopanagiotakis F, Xifteri A, Polychronopoulos V, Bouros D. Apoptosis in lung injury and fibrosis. Eur Respir J 2008; 32:1631-8.
Bouros D. Pirfenidone for idiopathic pulmonary fibrosis. Lancet 2011; 377:1727-9.
Falfan-Valencia R, Camarena A, Juarez A, et al. Major histocompatibility complex and alveolar epithelial apoptosis in idiopathic pulmonary fibrosis. Hum Genet 2005; 118:235-44.
Luzina IG, Todd NW, Iacono AT, Atamas SP. Roles of T lymphocytes in pulmonary fibrosis. J Leukoc Biol 2008; 83:237-44.
Journals System - logo
Scroll to top