Review

Authors: Kenya Kamimura, Masaaki Kobayashi, Yuichi Sato...

Collagenous gastritis is a rare disease characterized by the subepithelial deposition of collagen bands thicker than 10 μm and the infiltration of inflammatory mononuclear cells in the lamina propria. Collagenous colitis and collagenous sprue have similar histological characteristics to collagenous gastritis and are thought to be part of the same disease entity. However, while collagenous colitis has become more common in the field of gastroenterology, presenting with clinical symptoms of chronic diarrhea in older patients, collagenous gastritis is rare. Since the disease was first reported in 1989, only 60 cases have been documented in the English literature. No safe and effective treatments have been identified from randomized, controlled trials. Therefore, better understanding of the disease and the reporting of more cases will help to establish diagnostic criteria and to develop therapeutic strategies. Therefore, here we review the clinical characteristics, endoscopic and histological findings, treatment, and clinical outcomes from case reports and case series published to date, and provide a summary of the latest information on the disease. This information will contribute to improved knowledge of collagenous gastritis so physicians can recognize and correctly diagnose the disease, and will help to develop a standard therapeutic strategy for future clinical trials.

PubMed: 25789098
DOI: 10.4253/wjge.v7.i3.265

Review

Authors: Hugh James Freeman

Collagenous sprue is a small bowel mucosal lesion that has been historically associated with persistent diarrhea, progressive weight loss and severe malabsorption causing multiple nutrient deficiencies. A severe to variably severe mucosal lesion with distinct subepithelial collagen deposits occurs. Celiac disease has been intimately linked to collagenous sprue and, similar to celiac disease, small bowel ulceration, perforation and lymphoma may complicate the clinical course of collagenous sprue. In collagenous sprue, concomitant collagen deposits may also occur in gastric or colonic mucosal sites (or both), indicating that this unusual mucosal process may be very heterogeneous and far more extensive in the intestinal tract than previously appreciated. Moreover, reports of diagnosis during infancy suggest that the natural history of the disorder could be more prolonged than is currently appreciated. Finally, the collagen deposits, per se, may be due to different causes and, in some, even represent a novel paraneoplastic histopathological marker. Future studies are needed to more precisely define molecular and genetic biomarkers that identify homogeneous groups and permit the development of improved treatment strategies for this increasingly recognized disorder.

Topics: Adrenal Cortex Hormones; Celiac Disease; Collagen; Diarrhea; Diet, Gluten-Free; Disease Progression; Disease-Free Survival; Drug Therapy, Combination; Humans; Immunosuppressive Agents; Intestinal Absorption; Intestinal Mucosa; Intestine, Small; Malnutrition; Weight Loss

PubMed: 21523258
DOI: 10.1155/2011/821976

Review

Authors: Hugh J Freeman

Almost a half-century ago, an unusual and distinct form of colitis was first recognized, collagenous colitis, characterized by sub-epithelial trichrome-positive deposits having the ultrastructural features of collagen. Later, other reports documented more extensive collagenous dis-ease in these patients, sometimes in the stomach and small bowel, a close linkage with other forms of microscopic colitis and its association with celiac and other immune-mediated diseases. Moreover, emerging genetic methods permitted large studies of collagenous colitis to complement these intriguing clinical and pathological studies. Finally, recent and related studies have further demonstrated these immune-based forms of colitis, with new sprue-like intestinal diseases caused by novel medications, recently detected viral infections and vaccinations.

PubMed: 37554741
DOI: 10.22037/ghfbb.v16i2.2698

Authors: Luís Fernandes, Bárbara Machado, António José Cruz...

Collagenous sprue is a rare and unrecognized cause of diarrhea and weight loss, mainly affecting the duodenum and small bowel. The clinical picture often resembles that of coeliac sprue, the main differential diagnosis, albeit, being refractory to a gluten-free diet. The histological features are fundamentally characterized by the deposition of collagen beneath the basement membrane of gut mucosa. Treatment should be initiated as soon as the diagnosis is established, so as to prevent the progression of fibrosis. We will describe the case of a 76-year-old woman with collagenous sprue, her diagnostic workup, histopathological examination, and response to treatment.

PubMed: 37070112
DOI: 10.22037/ghfbb.v16i1.2671

Authors: Andreas Hallan, Michael Dam Jensen, Rikke Hjarnø Hagemann-Madsen...

Collagenous sprue is a rare enteropathy affecting the small intestinal mucosa and can resemble and coincide with coeliac disease or collagenous colitis. To our knowledge, less than 175 cases of collagenous sprue have been described. Both clinicians and pathologists should be aware of the condition in order to adequately target their investigations and treatment. This is a case report of severe collagenous sprue in a 78-year-old male resulting in severe diarrhoea, weight loss, malnutrition and acute kidney failure. The disorder improved on a lactose and gluten-free diet, loperamide and corticosteroids.

Topics: Aged; Celiac Disease; Collagenous Sprue; Diet, Gluten-Free; Duodenum; Humans; Male; Malnutrition

PubMed: 35485780
DOI: No ID Found

Review

Authors: Xiangrong Zhao, Rebecca L Johnson

Collagenous sprue is a severe malabsorptive disorder, histologically characterized by small intestinal villous and crypt atrophy, and a subepithelial collagen deposit, thicker than 12 µm, that entraps lamina propria cellular elements. Collagenous sprue is a rare disease entity, with only about 60 sporadic cases reported worldwide since it was first described in 1947. Its exact etiology is still under investigation, and its relationship with classic celiac disease and other refractory, spruelike intestinal disorders remains controversial. Two larger-scale studies, in 2009, brought new insights into this elusive, yet emerging, topic. Here, we present a review of the literature on the possible etiology of collagenous sprue, its proposed links to classic celiac disease and to refractory sprue, and its clinical, biochemical, histologic, and molecular features. To our knowledge, all case reports on collagenous sprue in the medical literature to date are summarized.

Topics: Celiac Disease; Endoscopy, Gastrointestinal; Female; Humans; Intestinal Absorption; Intestine, Small; Male; Malnutrition; Sex Factors

PubMed: 21631278
DOI: 10.5858/2010-0028-RS.1

Authors:

Topics: Biopsy; Celiac Disease; Collagen Diseases; Diet Therapy; Female; Humans; Intestinal Mucosa; Male; Middle Aged

PubMed: 5551269
DOI: No ID Found

Authors: Alberto Rubio-Tapia, Nicholas J Talley, Suryakanth R Gurudu...

BACKGROUND & AIMS

Collagenous sprue (CS) is characterized by the presence of a distinctive band of subepithelial collagen deposition in the small bowel. We evaluated the clinical characteristics, treatments, and outcomes of patients with CS.

METHODS

Thirty patients with CS were identified at the 3 Mayo Clinic sites between 1993 and 2009. Clinical data from medical records were reviewed.

RESULTS

The study cohort was 70% female (age range, 53-91 years). Most patients had severe diarrhea and weight loss. Hospitalization to treat dehydration was necessary in 16 (53%) patients. Associated immune-mediated diseases were noted in 70% of the patients; celiac disease was the most frequent. Other associated diseases were microscopic colitis, hypothyroidism, and autoimmune enteropathy. The median thickness of the layer of subepithelial collagen deposition in the small bowel was 29 mum (20-56.5 mum). Subepithelial collagen deposition in the colon or stomach was noted in 8 patients. A clinical response was observed in 24 (80%) patients after treatment with a combination of a gluten-free diet and immunosuppressive drugs. Histologic improvement was confirmed in 9 patients, with complete remission in 5. Two patients died (1 of complications of CS and 1 of another illness).

CONCLUSIONS

Most patients with CS are treated effectively with a combination of gluten-free diet and steroids. CS is often associated with collagen deposition or chronic inflammation in other segments of the gastrointestinal tract as well as other immune-mediated disorders.

Topics: Aged; Aged, 80 and over; Colitis, Collagenous; Diet, Gluten-Free; Female; Humans; Immunologic Factors; Male; Middle Aged; Steroids; Treatment Outcome

PubMed: 20060071
DOI: 10.1016/j.cgh.2009.12.023

Authors: Tanner Storozuk, Ian Brown, Stephen Lagana...

AIMS

Olmesartan, an angiotensin receptor blocker (ARB) used for hypertension management, is known to cause a sprue-like enteropathy in a subset of patients. Rare cases of gastritis occurring with ARB use have also been reported, but the histological features of ARB-induced gastritis and the response to drug cessation have not been examined in a dedicated case-series.

METHODS AND RESULTS

Cases of suspected ARB-induced gastritis were identified from the pathology archives of four institutions. Haematoxylin and eosin (H&E) slides from gastric biopsies were reviewed. Fifteen patients (14 female, one male) were identified. The most common presenting symptoms were diarrhoea (10) and weight loss (six). Gastric biopsies commonly showed a full-thickness active chronic gastritis with surface epithelial injury involving the antrum and body. Glandular atrophy, intra-epithelial lymphocytosis and/or subepithelial collagen thickening were also present in some cases. Duodenal involvement, including villous atrophy, intra-epithelial lymphocytosis and/or collagenous sprue, was identified in 11 of 13 cases with concurrent duodenal biopsies. Following drug cessation, symptomatic improvement occurred in all 11 cases for which follow-up data were available. Histological resolution occurred in five of eight cases with follow-up gastric biopsies, with improvement seen in the remaining three biopsies.

CONCLUSION

ARB-induced gastritis typically presents as active chronic gastritis, frequently with associated surface epithelial injury. Glandular atrophy, intra-epithelial lymphocytosis and/or subepithelial collagen thickening may also be present. These gastric changes can be seen without associated duodenal injury in rare cases, and they should alert the pathologist to the possibility of ARB-induced injury. Drug cessation results in marked symptomatic and histological improvement.

Topics: Angiotensin Receptor Antagonists; Angiotensin-Converting Enzyme Inhibitors; Atrophy; Collagen; Eosine Yellowish-(YS); Female; Gastritis; Humans; Lymphocytosis; Male

PubMed: 35945664
DOI: 10.1111/his.14766

Review

Authors: Claudine Desruisseaux, Michaël Bensoussan, Etienne Désilets...

Collagenous sprue (CS) is a distinct clinicopathological disorder histologically defined by a thickened subepithelial band (Freeman, 2011). It is a rare condition which has been recently observed in a significant proportion of sprue-like enteropathy associated with olmesartan, a novel entity described by Rubio-Tapia et al. in 2012. CS is historically associated with a poor prognosis (Marthey et al., 2014). However, histological and clinical improvements have been described in most studies with concomitant usage of corticosteroids and/or gluten-free diet (Marthey et al., 2014). We report a unique case of olmesartan-induced collagenous sprue in a 79-year-old man that showed complete histological and clinical remission with the sole withdrawal of the incriminating drug. The literature on this topic is briefly reviewed.

Topics: Aged; Antihypertensive Agents; Collagenous Sprue; Diarrhea; Humans; Hypertension; Imidazoles; Male; Tetrazoles; Withholding Treatment

PubMed: 27446843
DOI: 10.1155/2016/4837270