A paper published last year by Simons and colleagues identified a direct link between celiac disease and pneumococcal infection.1 While the incidence was higher in hospitalized patients versus the general population, the data has been building for years in the general population. The main cause of action for this increased risk involves the spleen.
In the most basic terms, the spleen is an organ in the lymphatic system that helps maintain fluid balance in the body and fight off infection. It is located on the left side of the body, under the ribs and above the stomach. It is about the size of a human fist. The spleen is the principle producer of antibodies and stores white blood cells that help fight off infection.
The clear connection between celiac disease and increased pneumococcal infection in the clinical literature is hyposplenism, which is a common comorbidity of celiac disease. Simply put, hyposplenism is a poorly functioning or under functioning spleen. Hyposplenism is a condition accompanying disease such as celiac disease, sickle cell disease, alcoholism induced liver disease, hepatic cirrhosis, lymphomas and autoimmune diseases. There is a higher incidence of hyposplenism in the pediatric celiac disease population. The prevalence of hyposplenism in patients with celiac disease ranges from 19% to 80%. Since the 1970s, there has been mounting clinical evidence, including laboratory, radiology and case histories, that hyposplenism and splenic atrophy is linked to pneumococcal infection.
Pneumococcal infections are caused by the sphere-shaped (coccal), gram-positive bacteria and include bronchitis, otitis media (inner ear infection), sinusitis, septicemia (blood infection), osteomyelitis (infection of the bone), septic arthritis (infection of a joint), pneumonia (infection of the lungs) and meningitis (infection of the meninges – the protective membranes that surround the brain and spinal cord).
Three studies from the United Kingdom, Sweden and Italy identified that celiac disease is associated with greater mortality from respiratory infections and sepsis (a serious blood infection).567 However, one study in the UK evaluated the risk of respiratory infections after the launch of the pneumococcal vaccine and found no increased risk of death.8 In 2016, a large retrospective cohort study in the UK identified unvaccinated patients with celiac disease to have an excess risk of community-acquired pneumonia not found in vaccinated patients with celiac disease.9 It has also been shown complicated cases of celiac disease including refractory celiac disease (a type of celiac disease that is resistant to treatment over a 12-month period) and cases that present with jejunoileitis, are associated with a smaller splenic volume.10 Regardless of these concerns, individuals with celiac disease have been shown to have an appropriate immunologic response to the pneumococcal vaccine.11
The recommendation from the study by Simons and colleagues is that the pneumococcal vaccination should be considered for those with celiac disease, with special attention to those aged 15-64 years who have not received the scheduled pneumococcal vaccination series as a child. Interestingly, typically you would expect those under 15 and over 64 years old to be at risk, right? Yet, this demographic typically receives the vaccination and thus experiences less incidence of infection. That’s how this risk group was identified.
1. Simons M, Scott-Sheldon LAJ, Risech-Neyman Y, Moss SF, Ludvigsson JF, Green PHR. Celiac Disease and Increased Risk of Pneumococcal Infection: A Systematic Review and Meta-Analysis. Am J Med. 2018 Jan;131(1):83-89. doi: 10.1016/j.amjmed.2017.07.021. Epub 2017 Aug 8. Review. PubMed PMID: 28801224.↩
2. Johnston SD, Robinson J. Fatal pneumococcal septicaemia in a coeliac patient. Eur J Gastroenterol Hepatol. 1998;10(4):353-354. 230.↩
3. O’Donoghue DJ. Fatal pneumococcal septicaemia in coeliac disease. Postgrad Med J. 1986;62(725):229-230.↩
4. Logan RF, Rifkind EA, Turner ID, Ferguson A. Mortality in celiac disease. Gastroenterology. 1989;97(2):265-271.↩
5. Grainge MJ, West J, Card TR, Holmes GK. Causes of death in people with celiac disease spanning the pre- and post-serology era: a population- based cohort study from Derby, UK. Am J Gastroenterol. 2011;106(5):933-939.↩
6. Peters U, Askling J, Gridley G, Ekbom A, Linet M. Causes of death in patients with celiac disease in a population-based Swedish cohort. Arch Intern Med. 2003;163(13):1566-1572.↩
7. Corrao G, Corazza GR, Bagnardi V, et al. Mortality in patients with coeliac disease and their relatives: a cohort study. Lancet. 2001;358(9279):356-361.↩
8. Abdul Sultan A, Crooks CJ, Card T, Tata LJ, Fleming KM, West J. Causes of death in people with coeliac disease in England compared with the general population: a competing risk analysis. Gut. 2015;64(8):1220- 1226.↩
9. Zingone F, Abdul Sultan A, Crooks CJ, Tata LJ, Ciacci C, West J. The risk of community-acquired pneumonia among 9803 patients with coeliac disease compared to the general population: a cohort study. Aliment Pharmacol Ther. 2016;44(1):57-67.↩
10. van Gils T, Nijeboer P, van Waesberghe JHT, et al. Splenic volume differentiates complicated and non-complicated celiac disease. United European Gastroenterol J. 2017;5(3):374-379.↩
11. McKinley M, Leibowitz S, Bronzo R, Zanzi I, Weissman G, Schiffman G. Appropriate response to pneumococcal vaccine in celiac sprue. J Clin Gastroenterol. 1995;20(2):113-116.↩