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

Table 1 Summary of studies reporting prevalence of celiac disease in Sjogren syndrome

Ref.	SS (n)	Country	CD prevalence (SS + CD)	Serological criteria	Histological criteria
Iltanen et al[16]	34 SS+, 28 controls	Finland	14.7% (n = 5)	SS+ CD; 3/5 IgA EMA+; 4/5 IgA AGA+; 5/5 IgA EMA or AGA +; SS – CD; 0/28 IgA EMA +; 9/28 IgA AGA +	All 5 seropositive - Marsh 3
Szodoray et al[17]	111 SS+	Hungary	4.5% (n =5 )	6/111 tTG and/or AGA and/or EMA +	5/6 CD+, one with jejunitis
Bartoloni et al[18]	354 SS+, 14298 controls	Italy	7.1% (n = 25), 6.8% (n = 24) previously diagnosed CD, 1 latent CD	24 previously diagnosed (2013 WGO guideline criteria), 1 IgA tTG+	All 25
Bizzaro et al[19]	100 SS+	Italy, Israel	1% (n = 1)	1 IgG tTG + (IgA tTG-)	Histology not done
Luft et al[20]	50 SS+, 50 controls 40 disease-controls (CD+)	Canada	12% (n = 6)	6 IgA tTG +	5/6 biopsy-proven CD
Caio et al[21]	52 SS+	Italy	5.8% (n = 3)	3/42 IgA tTG+, IgG DGP + and IgA EMA+	Histology not used

SS: Sjogren syndrome; CD: Celiac disease; AGA: Anti-gliadin antibodies; EMA: Antiendomysial antibodies.

Table 2 Summary of studies reporting prevalence of Sjogren syndrome in celiac disease

Ref.	CD (n)	Country	SS prevalence (SS + CD)	Diagnostic criteria
Erbasan et al[25]	82	Turkey	1.2%	29.3% dry eye, 24.4% dry mouth; 14.6% ANA+
Bibbò et al[26]	255	Italy	2.4%
Caglar et al[27]	31	Turkey	6.5%	12.9% ANA+; 6.5% SS-A/Ro+ and/or SS-B/La+
Collin et al[28]	335	Finland	3.3%
Ayar et al[29]	81	Turkey	4.9%	23.5% dry eye, 27.2% dry mouth

SS: Sjogren syndrome; CD: Celiac disease; ANA: Antinuclear antibodies.

Table 3 Clinical scenarios needing the search of celiac disease in Sjögren syndrome patients or Sjögren syndrome in celiac disease patients

When to consider CD in SS patients?	When to consider SS in CD patients?
Suggestive gastrointestinal symptoms; Suspected malabsorption; Hypogammaglobulinemia and low levels of total protein; High levels of transaminases without evident cause; Anemia of unknown cause; Herpetiform dermatitis; Autoimmune thyroiditis (frequent in SS patients)[47]1	Dry eyes and dry mouth symptoms; Enlarged parotid glands; Hypergammaglobulinemia and high levels of total protein; Polyneuropathy; Arthritis (arthralgias?); Purpura

¹The National Institute for Health and Clinical Excellence guideline recommends that all patients with autoimmune thyroid disease must be screened for celiac disease. CD: Celiac disease; SS: Sjögren syndrome.

Table 4 Future agenda

Describing the prevalence of SS in patients with CD using the new classification criteria for SS

Describing the prevalence of CD in SS patients using newer generation serology tests

Prospective studies in patients with both conditions regarding evolution and prognosis, especially regarding the possible additive risk of lymphoma

Describing the role of gluten free diet regarding SS symptoms in patients associating both diseases

Developing clear recommendations for screening for SS in CD and for CD in SS

CD: Celiac disease; SS: Sjögren syndrome.