A coeliac conundrum

October 27, 2015 By: Ruchit Sood

Figure 1 | The endoscopic view of the duodenal mucosa of the case patient, visualised by both white-light and narrow-band imaging.

Figure 2 | Haemotoxylin and eosin (H&E) stain of a biopsy sample taken from the duodenal mucosa of the case patient.

Ruchit Sood is an advanced endoscopy fellow at Leeds Teaching Hospitals, UK. He graduated from the University of Sheffield in 2005 and commenced his postgraduate training in gastroenterology in 2009. He is in the process of completing a Doctorate of Medicine from the University of Leeds, and to date he has published over 20 peer-reviewed articles. His main interest is in advanced therapeutic endoscopy, in particular upper and lower gastrointestinal tract endoscopic mucosal resection.

A coeliac conundrum

What would you do next for this middle-aged woman previously diagnosed with coeliac disease?

A middle-aged woman presented with loose stool and weight loss. Initially, she refused an endoscopy and a diagnosis of coeliac disease was made on the basis of a high tissue transglutaminase (tTG) antibody titre. She was started on a gluten-free diet (GFD) but her symptoms remained despite adherence to the GFD. After several months she agreed to undergo an endoscopy. The images show the endoscopic view of the duodenal mucosa (figure 1) and the corresponding histology slide (figure 2) stained with haemotoxylin and eosin (H&E).

WHAT WOULD YOU DO NEXT?

a)     Refer the patient for a dietary review

b)     Request a clonal analysis of the intraepithelial lymphocytes

c)     Refer for HLA DQ2/DQ8 testing

d)     Refer the patient for capsule endoscopy

e)     Add prednisolone to the dietary restrictions

Discussion

Initially, the patient appeared to have classic coeliac disease, with diarrhoea and weight loss together with a positive serology. The endoscopy was crucial as the duodenal biopsies identified the presence of a thick band-like deposit of collagen just below the duodenal epithelium. On the basis of this finding, collagenous sprue was diagnosed.

Collagenous sprue was first described in 1947,1 but it was not until 1970 that Weinstein et al. introduced it as a diagnostic term to the medical nomenclature.2 Collagenous sprue is more frequent in females and in individuals who have other autoimmune diseases.3 It is now recognised that collagenous sprue shares similar clinical features with coeliac disease, such as chronic diarrhoea, anaemia and weight loss. In addition, the endoscopic and histological features of both diseases are similar, with an atrophic and scalloped duodenal mucosa. However, the histological hallmark of collagenous sprue is the presence of a thick subepithelial collagen band. Such collagen bands may also be found in collagenous gastritis and collagenous colitis.4

It has been proposed that collagenous sprue may be a heterogenous condition of collagenous gastroenteritides, including conditions such as collagenous colitis and coeliac disease.5,6 Unlike coeliac disease, in collagenous sprue, the typical histological changes may also be found in the stomach and colon.7 Furthermore, greater numbers of IgG4 plasma cells have been reported in the duodenal mucosa of patients with collagenous sprue when compared with the numbers in patients with coeliac disease, duodenitis or normal duodenal mucosa.8

As in this case, patients with collagenous colitis usually also have positive coeliac serology. For this reason, some believe that the collagen band is simply a marker of particularly severe coeliac disease, which may also be associated with ulceration, perforation and T-cell or B-cell lymphoma.9

An interesting report of paraneoplastic collagenous colitis was reported by Freeman et.al.10 In that case a patient with collagen deposits in both the small and large intestine was also found to have a coincidental colon cancer. After surgery, both the malabsorption and the histopathological changes completely resolved! For this reason, a search for underlying malignant disease should be considered.

The response to a GFD is usually disappointing, and for this reason the outlook used to be grave for patients with collagenous sprue. However, remission of the condition has been reported with courses of corticosteroids11 or immunosuppressive agents such as infliximab.12


References

  1. Schein J. Syndrome on non tropical sprue with hitherto undescribed lesions of the intestine. Gastroenterology 1947; 8: 438–460.
  2. Weinstein WM, Saunders DR, Tytgat GN, et al. Collagenous sprue—an unrecognized type of malabsorption. N Engl J Med 1970; 283: 1297–1301. 
  3. Rubio-Tapia A, Talley NJ, Gurudu SR, et al. Gluten-free diet and steroid treatment are effective therapy for most patients with collagenous sprue. Clin Gastroenterol Hepatol 2010; 8: 344–349.e3. 
  4. Zhao X and Johnson RL. Collagenous sprue: a rare, severe small-bowel malabsorptive disorder. Arch Pathol Lab Med 2011; 135: 803–809. 
  5. Maguire AA, Greenson JK, Lauwers GY, et al. Collagenous sprue. A clinicopathologic study of 12 cases. Am J Surg Pathol 2009; 33: 1440–1449. 
  6. Nielsen OH, Riis LB, Danese S, et al. Proximal collagenous gastroenteritides: Clinical management. A systematic review. Ann Med 2014; 46: 311–317. 
  7. Robert ME, Ament ME and Weinstein WM. The histologic spectrum and clinical outcome of refractory and unclassified sprue. Am J Surg Pathol 2000; 24: 676–687. 
  8. Schoolmeester JK, Jenkins SM, Murray JA, et al. Increased immunoglobulin G4-positive plasma cells in collagenous sprue. Hum Pathol 2013; 44: 1624–1629. 
  9. Freeman HJ. Collagenous sprue associated with an extensive T-cell lymphoma. J Clin Gastroenterol 2003; 36(2): 144–146. 
  10. Freeman HJ and Berean KW. Resolution of paraneoplastic collagenous enterocolitis after resection of colon cancer. Can J Gastroenterol Hepatol 2006; 20: 357–360. 
  11. Freeman HJ, Davis JE and Myers DM. Complete histologic resolution of collagenous sprue. Can J Gastroenterol Hepatol 2004; 18: 333–336. 
  12. Schmidt C, Kasim E, Schlake W, et al. TNF- antibody treatment in refractory collagenous sprue: Report of a case and review of the literature. Z Gastroenterol 2009; 47: 575–578. 

 

 

Answer

Correct answer: e.

About the author

Ruchit Sood is an advanced endoscopy fellow at Leeds Teaching Hospitals, UK. He graduated from the University of Sheffield in 2005 and commenced his postgraduate training in gastroenterology in 2009. He is in the process of completing a Doctorate of Medicine from the University of Leeds, and to date he has published over 20 peer-reviewed articles. His main interest is in advanced therapeutic endoscopy, in particular upper and lower gastrointestinal tract endoscopic mucosal resection.

 

Comments

ali ghavidel, October 31, 2015 08:38
B
Mileidis Esther San Juan Acosta, October 29, 2015 17:10
a
Pauline Riviere, October 28, 2015 14:39
A B
Gang Qing, October 28, 2015 13:53
B
Hana Svecov, October 28, 2015 13:41
B
Julia Bayer, October 28, 2015 13:36
B
Amedeo Montale, October 28, 2015 13:20
B and if clonal analysis shows a negative results E
Farid Belghanem, October 28, 2015 13:13
B
Must exclude a lymphoma
Astapkevich Kristina, October 28, 2015 13:11
B
Zemiri Yacine, October 28, 2015 13:07
Coeliac disease biopsy and histology duodenal atrophic villosity crypt hyperplasia increase a intraepithelial lymphocyts treatment is regime without gluten

More Education News