title: Eosinophilic gastrointestinal disorders associated with autoimmune connective tissue disease.
abstract: OBJECTIVE
  To determine the clinical and pathological characteristics of eosinophilic gastrointestinal disease (EGID) associated with autoimmune connective tissue disease (CTD).
  
  
  METHODS
  Systematic literature review.
  
  
  RESULTS
  Twenty cases of CTD associated with EGID were identified. Systemic lupus erythematosus was the main EGID-associated CTD (35%), followed by rheumatoid arthritis (20%), systemic sclerosis or inflammatory myopathies (15%, each), and Sjögren's syndrome, scleromyositis or other overlapping connective tissue disease (5%, each). No patient had a history of atopy. In contrast with classical EGID among which eosinophilic esophagitis is the most frequent type, eosinophilic gastritis and/or enteritis represented 95% of cases. Gastrointestinal symptoms were often unspecific. Peripheral eosinophilia was found in 67% of cases. Upper and lower gastrointestinal endoscopy showed abnormal findings in only 40% and 30% of cases, respectively. EGID was confirmed by evidence of digestive eosinophilic infiltration, mainly in mucosal or submucosal layer. In all but one patient, the CTD was diagnosed prior to the occurrence of the EGID. In total, 95% of EGID had a favorable outcome, with corticosteroids being used in almost all cases.
  
  
  CONCLUSIONS
  Clinicians should consider EGID as a possible diagnosis and perform gastrointestinal tract biopsies in patients with CTD presenting with gastrointestinal symptoms and unexplained eosinophilia. Conversely, more rarely extra-digestive features during follow-up in patients with EGID may lead to a diagnosis of an associated CTD. More research is needed to better understand the underlying pathophysiological processes leading to eosinophilic gastrointestinal infiltration in patients with CTD.
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Hôpital Cochin, Université Paris Descartes, National Referral Center for Rare Systemic and Autoimmune Diseases, 27, rue du Faubourg-Saint-Jacques, 75679 Paris cedex 14, France; Hôtel-Dieu University Hospital, Department of Internal Medicine, 44000 Nantes, France.
authors.forename: Marie
authors.initials: M
authors.lastname: Lecouffe-Desprets
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Hôpital Cochin, Université Paris Descartes, National Referral Center for Rare Systemic and Autoimmune Diseases, 27, rue du Faubourg-Saint-Jacques, 75679 Paris cedex 14, France.
authors.forename: Matthieu
authors.initials: M
authors.lastname: Groh
authors.affiliation: Le Mans General Hospital, Department of Gastroenterology, 72037 Le Mans, France.
authors.forename: Bruno
authors.initials: B
authors.lastname: Bour
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Hôpital Cochin, Université Paris Descartes, National Referral Center for Rare Systemic and Autoimmune Diseases, 27, rue du Faubourg-Saint-Jacques, 75679 Paris cedex 14, France.
authors.forename: Claire
authors.initials: C
authors.lastname: Le Jeunne
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Hôpital Cochin, Université Paris Descartes, National Referral Center for Rare Systemic and Autoimmune Diseases, 27, rue du Faubourg-Saint-Jacques, 75679 Paris cedex 14, France. Electronic address: xavier.puechal@aphp.fr.
authors.forename: Xavier
authors.initials: X
authors.lastname: Puéchal
mesh_terms: D001327:Autoimmune Diseases / Q000175:diagnosis / Q000276:immunology*; D003240:Connective Tissue Diseases / Q000175:diagnosis / Q000276:immunology*; D004751:Enteritis / Q000175:diagnosis / Q000276:immunology*; D004802:Eosinophilia / Q000175:diagnosis / Q000276:immunology*; D057765:Eosinophilic Esophagitis / Q000175:diagnosis / Q000276:immunology*; D005756:Gastritis / Q000175:diagnosis / Q000276:immunology*; D041981:Gastrointestinal Tract / Q000276:immunology; D006801:Humans
keywords: Connective tissue disease; Eosinophilic colitis; Eosinophilic esophagitis; Eosinophilic gastroenteritis; Eosinophilic gastrointestinal disorder; Sjögren syndrome; Systemic lupus erythematosus