Duodenal lymphocytosis
| Duodenal lymphocytosis | |
|---|---|
| Other names | Lymphocytic duodenitis, Lymphocytic duodenosis, Duodenal intraepithelial lymphocytosis |
| Specialty | Gastroenterology |
| Causes | Coeliac disease, environmental enteropathy and others |
| Diagnostic method | Histological examination of duodenal biopsy |
| Frequency | 3–7% of people having duodenal biopsy |
Duodenal lymphocytosis, sometimes called lymphocytic duodenitis, lymphocytic duodenosis, or duodenal intraepithelial lymphocytosis, is a condition where an increased number of intra-epithelial lymphocytes is seen in biopsies of the duodenal mucosa when these are examined microscopically. This form of lymphocytosis is often a feature of coeliac disease but may be found in other disorders.
Presentation
[edit]The condition is characterised by an increased proportion of lymphocytes in the epithelium of the duodenum, usually when this is greater than 20–25 per 100 enterocytes.[1] Intra-epithelial lymphocyte (IEL) are normally present in intestine and numbers are normally greater in the crypts and in the jejunum; these are distinct from those found in the lamina propria of the intestinal mucosa. IELs are mostly T cells.[1] Increased numbers of IELs are reported in around 3% of in duodenal biopsies, depending on case mix, but may be increasingly being found, in up to 7%.[2][3]
Causes
[edit]The list of possible causes is wide, including coeliac disease, environmental enteropathy (tropical sprue), autoimmune enteropathy, small intestinal bacterial overgrowth, NSAID damage, Helicobacter pylori, other infections and Crohn's disease.[1]
Diagnosis
[edit]Diagnosis is made by accurate counting of intraepithelial lymphocytes during histological examination of the duodenum.[1] The definition of the condition includes the requirement that the duodenal histological appearances are otherwise unremarkable, specifically with normal villous architecture.[2]
In coeliac disease (also known as gluten-sensitive enteropathy), duodenal lymphocytosis is found in untreated or partially treated cases. This is the least severe type of change, known as the Marsh I stage, in the classification of histological changes in coeliac disease. Additional features including villous atrophy and crypt hyperplasia are the other findings in other Marsh stages of coeliac disease.[4][1]
Antibodies associated with coeliac disease were reported in around 11% of cases.[1] These IgA endomysial antibodies and anti-transglutaminase antibodies are very sensitive and specific for coeliac disease implying that this proportion of duodenal lymphocytosis cases has definite coeliac disease. Around 33% of cases have the HLA-DQ2 allele, which is found in over 90% of people with coeliac disease. Absence of HLA-DQ2 (and the rarer HLA-DQ8) makes coeliac disease most unlikely.[5] As antibody-negative coeliac disease is recognised, HLA status, persistence or progression of the duodenal IEL numbers following a gluten challenge, followed by symptomatic improvement on a gluten-free diet, has been used to be more certain about the diagnosis, which was made in 22% of one series of over 200 adult cases. [5]
Helicobacter infection is a common finding at endoscopy and although duodenal IEL counts were found to be slightly higher with this infection, this was not considered to be a meaningful cause in children.[6] Other infections, including Cryptosporidiosis and Giardiasis can also be associated with an increase in IELs.[2]
Management
[edit]The management is that of any identified associated disorder such as a gluten free diet for cases with coeliac disease[5] or treatment of associated infections.[2]
Prognosis
[edit]When duodenal lymphocytosis is associated with other features of coeliac disease, in particular positive antibodies, or HLA-DQ2/8 and a family history, treatment with a gluten-free diet produces an improvement in IEL numbers.[5] Diarrhoea, thyroiditis, weakness and folate deficiency were other predictors of the development of gluten sensitivity and coeliac disease, which developed in 23 of 85 patients over 2 years in one series.[7]
References
[edit]- ^ a b c d e f Lauwers, Gregory Y; Fasano, Alessio; Brown, Ian S (2015). "Duodenal lymphocytosis with no or minimal enteropathy: much ado about nothing?". Modern Pathology. 28 (S1): S22–S29. doi:10.1038/modpathol.2014.135. ISSN 0893-3952. PMID 25560597.
- ^ a b c d Hammer, Suntrea T. G.; Greenson, Joel K. (2013). "The Clinical Significance of Duodenal Lymphocytosis With Normal Villus Architecture". Archives of Pathology & Laboratory Medicine. 137 (9): 1216–1219. doi:10.5858/arpa.2013-0261-ra. ISSN 0003-9985. PMID 23991733.
- ^ Shmidt, Eugenia; Smyrk, Thomas C.; Boswell, Christopher L.; Enders, Felicity T.; Oxentenko, Amy S. (2014). "Increasing duodenal intraepithelial lymphocytosis found at upper endoscopy: time trends and associations". Gastrointestinal Endoscopy. 80 (1): 105–111. doi:10.1016/j.gie.2014.01.008. ISSN 0016-5107. PMID 24565068.
- ^ Marsh, Michael N. (1992). "Gluten, major histocompatibility complex, and the small intestine". Gastroenterology. 102 (1): 330–354. doi:10.1016/0016-5085(92)91819-p. ISSN 0016-5085. PMID 1727768.
- ^ a b c d Aziz, Imran; Key, Tim; Goodwin, John G.; Sanders, David S. (2014). "Predictors for Celiac Disease in Adult Cases of Duodenal Intraepithelial Lymphocytosis". Journal of Clinical Gastroenterology. 49 (6): 477–82. doi:10.1097/mcg.0000000000000184. ISSN 0192-0790. PMID 25014240. S2CID 13090956.
- ^ Guz-Mark, A.; Zevit, N.; Morgenstern, S.; Shamir, R. (2014-04-07). "Duodenal intraepithelial lymphocytosis is common in children without coeliac disease, and is not meaningfully influenced by Helicobacter pylori infection". Alimentary Pharmacology & Therapeutics. 39 (11): 1314–1320. doi:10.1111/apt.12739. ISSN 0269-2813. PMID 24702235. S2CID 22316105.
- ^ Losurdo, Giuseppe; Piscitelli, Domenico; Giangaspero, Antonio; Principi, Mariabeatrice; Buffelli, Francesca; Giorgio, Floriana; Montenegro, Lucia; Sorrentino, Claudia; Amoruso, Annacinzia; Ierardi, Enzo; Leo, Alfredo Di (2015-06-28). "Evolution of nonspecific duodenal lymphocytosis over 2 years of follow-up". World Journal of Gastroenterology. 21 (24): 7545–52. doi:10.3748/wjg.v21.i24.7545. PMC 4481450. PMID 26140001.