The strongest associations between CD and systemic autoimmune diseases include Addison's disease, autoimmune thyroid disease, AIDDM, Sjogren's syndrome, PBC, and autoimmune hepatitis (Figure 1). Though more cost-benefit analyses need to be done to support CD screening of these enriched populations and facilitate development of diagnostic algorithms, maintaining a low threshold for screening and high clinical suspicion among these groups is recommended. There is clear association between dermatitis herpetiformis (DH) and CD such that a definitive diagnosis of DH by direct immunofluorescence is sufficient evidence to justify initiation of a gluten free diet and positive tissue transglutaminase autoantibodies in the context of DH implies substantial villous atrophy. Testing among arthritic patients is less clear given the limited large scale studies, prevalence differences between adult and pediatric populations, and the varying types of arthritis studied to date. Maintaining a lower threshold for screening among pediatric as compared to adult populations is recommended. Screening for CD among SLE patients is not recommended, though should be considered in the differential diagnosis of lupus patients with gastrointestinal symptoms. Neurological patients with the broad spectrum of clinical symptoms described should be considered for CD screening and also evaluated for gluten-dependent conditions occurring in the absence of enteropathy. Additional autoimmune diseases of the small bowel should be considered in the absence of a clinical response to dietary gluten restriction and/or lack of the recommended diagnostic criteria for CD.