Celiac disease and Sjögren’s syndrome:A case report and review of literature

BACKGROUND Celiac disease(CD)is a systemic,chronic immune-mediated disease triggered by gluten ingestion in genetically-susceptible individuals,with a prevalence of 1%worldwide.Sjogren's syndrome(SS)is also a systemic autoimmune disease,mainly characterized by ocular and oral sicca symptoms and signs.Sharing a common genetic background,CD and SS are known associated autoimmune diseases,but currently available guidelines are not reporting it.CASE SUMMARY We report the case of a 39-year-old woman,who was in the care of her rheumatologist for 2 years with SS.On routine follow-up she was found to have iron deficiency,without anemia.She had no gastrointestinal complaints and denied any obvious source of blood loss.IgA tissue transglutaminase antibodies were positive and endoscopy with duodenal biopsies revealed crypt hyperplasia and villous atrophy.A diagnosis of CD was set and gluten-free diet was recommended.CONCLUSION We present a review of existing data in the literature regarding the association of the two diseases,summarizing prevalence studies of CD in SS patients and the other way around.Screening recommendations and future research perspectives are also discussed,highlighting clinically relevant unanswered questions with respect to the association of CD with SS.

Celiac crisis, a rare occurrence in adult celiac disease: A systematic review

BACKGROUND Celiac crisis(CC), a potentially life-threatening condition, is one of the rare clinical presentations of celiac disease(CD). Several cases have been documented in the literature, mostly in children.AIM To perform a review of CC cases reported in adult CD patients.METHODS A systematic search of the literature was conducted in two databases,PubMed/MEDLINE and EMBASE, using the term "celiac crisis" and its variant"coeliac crisis", from January 1970 onwards. Altogether, 29 articles reporting 42 biopsy-proven cases were found in the search. Here, we summarized the demographic, clinical characteristics, laboratory and diagnostic work-ups, and therapeutic management in these patients.RESULTS Among the 42 CD cases, the median age was 50 years(range 23-83), with a 2:1 female to male ratio. The majority of patients(88.1%) developed CC prior to CDdiagnosis, while the remaining were previously diagnosed CD cases reporting low adherence to a gluten-free diet(GFD). Clinically, patients presented with severe diarrhea(all cases), weight loss(about two thirds) and, in particular situations, with neurologic(6 cases) or cardiovascular(1 case) manifestations or bleeding diathesis(4 cases). One in four patients had a precipitating factor that could have triggered the CC(e.g. trauma, surgery, infections). Laboratory workup of patients revealed a severe malabsorptive state with metabolic acidosis,dehydration, hypoalbuminemia and anemia. The evolution of GFD was favorable in all cases except one, in whom death was reported due to refeeding syndrome.CONCLUSION Celiac crisis is a rare but severe and potentially fatal clinical feature of CD. A high index of suspicion is needed to recognize this clinical entity and to deliver proper therapy consisting of supportive care and, subsequently, GFD.