Eosinophilic gastrointestinal disorders associated with autoimmune connective tissue disease

Abstract Objectives 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 Sjogren'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. Conclusion 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.