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Collagenous colitis is a form of microscopic colitis, while Collagenous Sprue (CS) is a malabsorptive disorder that mimics celiac disease. Both conditions show increased Intraepithelial Lymphocytes (IELs) and basement membrane thickening due to collagen deposition. The simultaneous occurrence of both disorders is rare, and only a few such cases have been reported in the literature. A 60-year-old female presented with chronic, non bloody diarrhoea persisting for two years. Investigations revealed megaloblastic anaemia and hypoalbuminaemia. Gastroenteroscopy showed flattening and atrophy of the small intestinal mucosa. Biopsy results showed increased IELs, normal crypt architecture, and stripping of the epithelium. There was also patchy deposition of hyalinised band subepithelially, confirmed to be collagen through Masson’s stain. As both the upper and lower Gastrointestinal (GI) tracts were involved, a diagnosis of collagenous enterocolitis was made. A 70-year-old female presented with intermittent watery diarrhoea for the past 10 days, accompanied by urgency and tenesmus. She had a history of colicky abdominal pain four years ago, which improved with steroids, but the symptoms recurred. Clinically, she was considered to have inflammatory bowel disease, with recurrent anaemia. Both gastroduodenoscopy and colonoscopy were normal. Mapping biopsy showed increased IELs and focal atrophy in the duodenum, while the colon exhibited mild mononuclear inflammation with occasional minimal activity. Focal, irregular deposition of subepithelial collagen was also observed and confirmed through Masson’s stain. A diagnosis of collagenous enterocolitis was made. She is currently being monitored for any recurrence of symptoms, after which steroid treatment will be initiated. Collagenous colitis, CS, and collagenous duodenitis share similar histological features and can rarely co-exist. Further studies are needed to better understand their aetiology and association.

Collagenous colitis is a form of microscopic colitis, while Collagenous Sprue (CS) is a malabsorptive disorder that mimics celiac disease. Both conditions show increased Intraepithelial Lymphocytes (IELs) and basement membrane thickening due to collagen deposition. The simultaneous occurrence of both disorders is rare, and only a few such cases have been reported in the literature. A 60-year-old female presented with chronic, non bloody diarrhoea persisting for two years. Investigations revealed megaloblastic anaemia and hypoalbuminaemia. Gastroenteroscopy showed flattening and atrophy of the small intestinal mucosa. Biopsy results showed increased IELs, normal crypt architecture, and stripping of the epithelium. There was also patchy deposition of hyalinised band subepithelially, confirmed to be collagen through Masson’s stain. As both the upper and lower Gastrointestinal (GI) tracts were involved, a diagnosis of collagenous enterocolitis was made. A 70-year-old female presented with intermittent watery diarrhoea for the past 10 days, accompanied by urgency and tenesmus. She had a history of colicky abdominal pain four years ago, which improved with steroids, but the symptoms recurred. Clinically, she was considered to have inflammatory bowel disease, with recurrent anaemia. Both gastroduodenoscopy and colonoscopy were normal. Mapping biopsy showed increased IELs and focal atrophy in the duodenum, while the colon exhibited mild mononuclear inflammation with occasional minimal activity. Focal, irregular deposition of subepithelial collagen was also observed and confirmed through Masson’s stain. A diagnosis of collagenous enterocolitis was made. She is currently being monitored for any recurrence of symptoms, after which steroid treatment will be initiated. Collagenous colitis, CS, and collagenous duodenitis share similar histological features and can rarely co-exist. Further studies are needed to better understand their aetiology and association. [ABSTRACT FROM AUTHOR]

Almost a half-century ago, an unusual and distinct form of colitis was first recognized, collagenous colitis, characterized by subepithelial 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Purpose: Collagenous sprue (CS) is a rare enteropathy characterized by villous atrophy and a thickened subepithelial collagen band. The aim of this study is to describe the cross-sectional imaging findings of CS. Methods: A case-control, retrospective study with cases of all CS patients from January 2000 to 2015 was performed. Inclusion criteria were (1) Histopathologic diagnosis and (2) Imaging with computed tomography abdomen/pelvis (CT A/P), CT enterography (CTE), or magnetic resonance enterography within 6 months of small bowel (SB) biopsy. Control subjects were irritable bowel syndrome (IBS) patients who underwent CTE. Imaging studies were examined by two GI radiologists, blinded to patient data. Results: 108 patients (54 CS; 54 IBS) were included. Mean age was 56.7 ± 16.5 years, and 68% were female (72% in CS group vs. 63% in IBS group; p = 0.3). CS patients were significantly older (67 ± 12 vs. 47 ± 15 year; p < 0.001) and more likely to be on angiotensin receptor blockers (41% vs. 6%; p < 0.001) as compared to the IBS group. Compared to IBS, CS patients were more likely to have mesenteric lymph node (LN) prominence (56% vs. 15%; p < 0.001), jejunoileal fold pattern reversal (46% vs. 6%; p < 0.001), SB dilation (28% vs. 0%; p < 0.001), SB conformational change (28% vs. 6%; p = 0.002), SB wall thickening (13% vs. 2%; p = 0.03), and ulcerative jejunoileitis (4% vs. 0%; p = 0.01). Radiologists suspected malabsorption in 72% in the CS group and 2% in the IBS group ( p < 0.001). Conclusion: Imaging findings suggestive of mucosal malabsorption are commonly demonstrated in CS. [ABSTRACT FROM AUTHOR]

Background Collagenous sprue (CS) is a rare form of enteropathy that had been reported to be associated with celiac disease (CD) and collagenous colitis (CC). The aim of our study was to compare the clinical features, treatments, and outcomes of CS, CD, and CC. Methods All patients with histologic diagnosis of CS, CD, or CC with complete clinical data were extracted from our pathology database between 1990 and 2015. Demographic and clinical features were recorded along with treatments and outcomes. Results A total of 21 patients with CS were included. Overall CS patients were more symptomatic with 17 (81.0%) patients with diarrhea and 15 (71.4%) with unintentional weight loss. Positive celiac serology was noted in 5 (23.8%) CS patients. CS patients had higher rates for disease-related temporary total parenteral nutrition (TPN) use (38.1% vs. 1.1% vs. 1.0%, P < 0.0001) and disease-related hospitalization (52.4% vs. 3.3% vs. 8.2%, P < 0.0001) than that in CD and CC patients. Twenty CS patients received treatments, including the combination of gluten-free diet (GFD) and corticosteroids ( n = 12), GFD only ( n = 2), and corticosteroids only ( n = 6). All CS patients showed symptomatic reliefs with treatment. Although CS patients had a higher rate for hospitalization and TPN use, disease-related death was not observed in all three groups. Conclusions Collagenous sprue patients had more severe clinical presentation than patients with CD and CC and therefore had higher demand for temporary TPN and hospitalization. Nevertheless, a prompt use of steroids and/or GFD upon histologic diagnosis of CS may have contributed to an overall excellent prognosis. [ABSTRACT FROM AUTHOR]

Hypogammaglobulinemia/common variable immunodeficiency (CVID) may lead to disruption of the gut mucosal immune barrier. Collagenous infiltrative disorders of the intestinal tract (colitis, gastritis, sprue) constitute a relatively new spectrum of gastrointestinal disorders. Our aims were (1) to determine the association between immunoglobulin deficiency state like CVID and collagenous infiltrative disorders of the gut and (2) to study the clinic-pathologic characteristics and treatment outcomes in these patients. A retrospective search was conducted to identify cases with concurrence of these two conditions at an academic center from 2007 to 2013. Four such patients were identified from our database: three with collagenous colitis and one with collagenous gastritis. All patients with collagenous colitis had normal colonic mucosa while the patient with collagenous gastritis had nodular gastric mucosa. Only one patient out of four had decreased plasma cells in the submucosa as expected in low immunoglobulin states. All patients had improvement in their symptoms on immunoglobulin therapy with considerable remission on budesonide. Literature search revealed reporting of four similar patients. In conclusion, (1) the association between collagenous infiltrative disorders of the gut and CVID and its prompt response to immunoglobulins with effective maintenance with budesonide are novel findings. Our study also shows that the presence of plasma cells should not rule out the possibility of CVID. (2) In patients with chronic diarrhea, hypogammaglobulinemia and collagenous colitis/sprue should be considered for the available effective treatments such as immunoglobulins and budesonide. [ABSTRACT FROM AUTHOR]

Celiac disease (CD) is an immunoallergic enteropathy affecting genetically susceptible individuals upon dietary exposure to gluten. In current clinical practice, the diagnosis of CD is based on a combination of clinical, serologic, and histologic factors with the possible exception of pediatric patients. Histopathologic evaluation of small intestinal tissue plays a critical role in the disease diagnosis and management, despite many practical challenges. Recently published best practice guidelines help to standardize biopsy sample procurement, tissue preparation, histology interpretation, and reporting, to optimize patient care. In addition, an increasing demand for monitoring the disease course, particularly demonstrating the efficacy of dietary and nondietary interventions for disease management, calls for the use of quantitative histology. With the advent of a gradual transition toward digital pathology in routine diagnostic practice, quantitative histopathologic evaluation in CD shows a promising future., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objective. Inflammatory bowel disease (IBD), microscopic colitis and celiac disease are all diseases with worldwide distribution and increased incidence has been reported from many areas. There is a shortage of studies investigating the occurrence of these diseases in the same individual and whether those affected demonstrate any particular phenotype. The aim of the study was to describe the concomitant incidence of microscopic colitis and celiac disease in a population-based IBD cohort. Methods. All 790 individuals in a prospective population-based cohort included 2005-09 from Uppsala region, Sweden, were reviewed regarding the appearance of microscopic or celiac disease before or after IBD diagnosis. Results. Fifty percent (396/790) of the patients had been examined for the possibility of celiac disease. Seventeen patients with celiac disease were found, representing 2.2% of the cohort. Patients with celiac disease were younger compared to the non-celiac patients and those with colitis had more often an extensive inflammation of the colon. Seventy-one percent (12/17) were women. The majority of the patients were diagnosed with celiac disease before IBD. Five patients with IBD had an earlier diagnosis of microscopic colitis or developed it after the IBD diagnosis. One teenager developed collagenous sprue, misinterpreted as a severe relapse of ulcerative colitis (UC) resulting in colectomy. Conclusions. The risk for celiac disease seems not to be increased in IBD, but those affected by both diseases seem to be predominantly women with extensive UC. There is a potential association between microscopic colitis and IBD. [ABSTRACT FROM AUTHOR]

Collagenous sprue ( CS) is a severe malabsorption disorder, the etiology of which has not been well defined. Herein, we report the case of a 3-month-old infant with CS who responded to steroid and immunomodulator treatment and presented a thick subepithelial collagen band. A 3-month-old Japanese girl presented with severe watery diarrhea that lasted for 2 weeks. She was admitted to the referring hospital, but symptomatic improvement was not achieved with fasting and rehydration. Gastroduodenal endoscopy showed an edematous duodenal mucosal surface. Duodenal biopsy indicated severe villous atrophy with infiltration of mostly CD8-positive T cells; and deposition of subepithelial collagen was confirmed. The subepithelial collagen deposits, however, had disappeared after treatment. Historically, child-onset CS is extremely rare and this case is likely to be the youngest case of infantile CS. The present case suggests that CS should be considered as a differential diagnosis for intractable diarrhea, even in infants. [ABSTRACT FROM AUTHOR]

Collagenous gastritis (CG) is a rare disorder characterized by the thick collagenous subepithelial bands associated with mucosal inflammation. There have been approximately fifty reports in the literature since it was first described in 1989. According to previous reports, CG is heterogeneous and classified into two groups-(1) cases limited to the gastric mucosa in children or young adults, and (2) CG associated with collagenous colitis in elderly adults presenting with chronic watery diarrhea. In Japan, only nine previous cases were reported, and all of them were young adults. We report a case of CG with collagenous duodenitis in a 22-year-old female. She had repeated upper gastrointestinal bleeding from a Dieulafoy lesion of the fornix, but had no symptoms of malabsorption or diarrhea. Endoscopic findings revealed striking nodularity with a smooth islet-shaped normal area in the antrum and the body. The pathological findings of nodular mucosa showed the deposition of collagen bands just under the mucoepithelial lesion. In addition, she had collagenous duodenitis in part of the bulbs, and a colonoscopy showed no abnormalities. We provide a literature review of CG and collagenous gastroduodenitis without colonic involvement. [ABSTRACT FROM AUTHOR]

Aim. While collagenous colitis represents the most common form of the collagenous gastroenteritides, the collagenous entities affecting the proximal part of the gastrointestinal tract are much less recognized and possibly overlooked. The aim was to summarize the latest information through a systematic review of collagenous gastritis, collagenous sprue, and a combination thereof. Methods. The search yielded 117 studies which were suitable for inclusion in the systematic review. Excluding repeated cases, 89 case reports and 28 case series were reported, whereas no prospective studies with or without control groups were identified. Further, no randomized, controlled trials were identified. The total number of patients with proximal collagenous gastroenteritides reported was 330. Results. An overview of clinical presentations, prognosis, pathophysiology and histopathology, as well as management of these disorders is presented. The prognosis of both collagenous gastritis and sprue seems not to be as dismal as considered previously. Data point to involvement of immune or autoimmune mechanisms potentially driven by luminal antigens initiating the fibroinflammatory condition. Conclusions. To reach the diagnosis it is recommended that biopsies are obtained during gastroduodenoscopies. Therapies with anti-secretory strategies, glucocorticoids, and in some cases iron supplementation are suggested, although rational treatment options from randomized, controlled trials do not exist for these rare or even overlooked disorders. [ABSTRACT FROM AUTHOR]

Double balloon endoscopy ( DBE) is useful for diagnosing many intestinal diseases and for endoscopic procedures. We report a case of chronic diarrhea in a 58-year-old Japanese man. He was initially suspected to have malabsorption syndrome. DBE showed reduction of folds, scalloping, mucosal nodularity and granularity. Pathological examinations of biopsies from the jejunum showed severe villous atrophy with subepithelial collagen bands. These findings led to the final diagnosis of collagenous sprue ( CS). With1 month of total parenteral nutrition followed by a low-gluten diet, his symptoms gradually improved. CS has never been reported before in Japan. DBE is useful for making a diagnosis of CS, and may be considered for patients who are suffering from diarrhea of unknown cause. [ABSTRACT FROM AUTHOR]

Collagenous sprue is a rare enteropathy whose etiology is unknown, but immune-mediated mechanisms are one of several possibilities. However, the role of immunoglobulin G4 (IgG4)-positive plasma cells has not been studied in collagenous sprue. Endoscopic biopsies from the duodenum with a histologic diagnosis of collagenous sprue (n = 40 from 35 patients), celiac disease (n = 25), peptic duodenitis (n = 15) and normal duodenum (n = 25) were immunohistochemically stained with IgG4 and CD138 antibodies. For each case, the quantities of IgG4- and CD138-positive plasma cells in the lamina propria were estimated by averaging the number in 3 high-power fields (hpf) that showed the highest concentration. Nine of forty collagenous sprue samples showed a mean of 10 or more IgG4 plasma cells per hpf, whereas none of the duodenal control biopsies showed 10 or more IgG4 plasma cells per hpf: celiac disease (P = .01), peptic duodenitis (P = .05), and normal duodenum (P = .01). Our study demonstrates that increased IgG4-positive plasma cells are present in a subset (23%) of collagenous sprue and may play a role in its pathogenesis. [ABSTRACT FROM AUTHOR]

Abstract: Collagenous sprue (CS) is a progressive malabsorptive disorder characterized by collagen deposition beneath the basement membrane of small bowel epithelium in refractory celiac sprue. CS is a pathologically distinct entity from celiac disease, despite a similar clinical presentation. The etiology of CS is unclear, although there are speculations that CS and celiac disease may share similar pathogenetic pathways. On the other hand, HFE hemochromatosis (HH) is a distinct disease entity. Celiac disease and HH are common HLA-associated genetic disorders in Northern European populations. There are a few case reports linking celiac disease and HH. We present a patient diagnosed with concurrent CS and HH. [Copyright &y& Elsevier]

Abstract: Selective IgA deficiency is the most common primary immunoglobulin deficiency. The clinical manifestations of selective IgA deficiency, including gastrointestinal (GI) complications, are rare and typically milder than those seen with common variable immunodeficiency or X-linked agammaglobulinemia. We present a rare case of selective IgA deficiency that shows a number of interesting histological features in the GI tract, including diffuse nodular lymphoid hyperplasia involving the entire small and large intestine, celiac disease-like and collagenous sprue-like changes in the small intestine, as well as lymphocytic colitis pattern. However, this patient had no particular GI symptoms suggestive of celiac sprue or microscopic colitis. These findings suggest that the GI tract in patients with selective IgA deficiency can show peculiar histologic changes that mimic celiac disease, collagenous sprue, or lymphocytic colitis, which may be a pattern of injury related to infection or immunoglobulin immunodeficiency-associated autoimmune phenomena. [Copyright &y& Elsevier]

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.

Collagenous sprue is a clinicopathological entity with an unknown etiology. Its clinical features include progressive malabsorption, diarrhea, weight loss, unresponsiveness to treatment, and high mortality rates. The age interval of collagenous sprue is quite broad and ranges between 2 and 85 years. As far as to our knowledge, the presented case is the first reported case in infancy. [ABSTRACT FROM AUTHOR]

We present a rare case of collagenous sprue in an elderly woman with significant weight loss and malnutrition. Collagenous sprue is a rare, female-predominant and immune-mediated gastrointestinal disease that can affect any part of the gut, and shares a strong association with Coeliac disease. The diagnosis is confirmed by gut histopathology demonstrating a subepithelial collagenous band and inflammatory infiltrate in the lamina propria. The pathogenesis and natural history is poorly elucidated, and treatment involves a gluten-free diet and/or immunomodulatory therapy. [ABSTRACT FROM AUTHOR]

The following fictional case is intended as a learning tool within the Pathology Competencies for Medical Education (PCME), a set of national standards for teaching pathology. These are divided into three basic competencies: Disease Mechanisms and Processes, Organ System Pathology, and Diagnostic Medicine and Therapeutic Pathology. For additional information, and a full list of learning objectives for all three competencies, see http://journals.sagepub.com/doi/10.1177/2374289517715040., Competing Interests: Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Celiac disease features duodenal intraepithelial lymphocytosis with or without villous atrophy. Lymphocytosis without villous atrophy will be proven to represent celiac disease in 10% to 20% of cases. The differential diagnosis is broad: Helicobacter pylori gastritis, NSAID injury and bacterial overgrowth are considerations. Lymphocytosis with villous atrophy is very likely to be celiac disease, but there are mimics to consider, including collagenous sprue, tropical sprue, drug injury, and common variable immunodeficiency. Histologic clues to a diagnosis other than celiac disease include paucity of plasma cells, excess of neutrophils, granulomas, and relative paucity of intraepithelial lymphocytes., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Spontaneous free perforation of the small intestine is uncommon, especially if there is no prior history of visceral trauma. However, free, even recurrent, perforation may complicate a defined and established clinical disorder, such as Crohn's disease. In addition, free perforation may be the initial clinical presentation of an occult intestinal disorder, such as a lymphoma complicating celiac disease, causing diffuse peritonitis and an acute abdomen. Initial diagnosis of the precise cause may be difficult, but now has been aided by computerized tomographic imaging. The site of perforation may be helpful in defining a cause (e.g., ileal perforation in Crohn's disease, jejunal perforation in celiac disease, complicated by lymphoma or collagenous sprue). Urgent surgical intervention, however, is usually required for precise diagnosis and treatment. During evaluation, an expanding list of other possible causes should be considered, even after surgery, as subsequent management may be affected. Free perforation may not only complicate an established intestinal disorder, but also a new acute process (e.g., caused by different infectious agents) or a longstanding and unrecognized disorder (e.g., congenital, metabolic and vascular causes). Moreover, new endoscopic therapeutic and medical therapies, including use of emerging novel biological agents, have been complicated by intestinal perforation. Recent studies also support the hypothesis that perforation of the small intestine may be genetically-based with different mutations causing altered connective tissue structure, synthesis and repair.

Collagenous sprue (CS) is a pattern of small-bowel injury characterized histologically by marked villous blunting, intraepithelial lymphocytes, and thickened sub-epithelial collagen table. Clinically, patients present with diarrhea, abdominal pain, malabsorption, and weight loss. Gluten intolerance is the most common cause of villous blunting in the duodenum; however, in a recent case series by the Mayo Clinic, it has been reported that olmesartan can have a similar effect. In this case report, a 62-year-old female with a history of hypothyroidism and hypertension managed for several years with olmesartan presented with abdominal pain, weight loss, and nausea. Despite compliance to a gluten-free diet, the patient's symptoms worsened, losing 20 pounds in 3 wk. Endoscopy showed thickening, scalloping, and mosaiform changes of the duodenal mucosa. The biopsy showed CS characterized by complete villous atrophy, lymphocytosis, and thickened sub-epithelial collagen table. After 2 mo cessation of olmesartan, the patient's symptoms improved, and follow-up endoscopy was normal with complete villous regeneration. These findings suggest that olmesartan was a contributing factor in the etiology of this patient's CS. Clinicians should be aware of the possibility of drug-induced CS and potential reversibility after discontinuation of medication.

Celiac disease is a gluten-dependent intestinal disorder that appears to be associated with several clinical conditions. Some involve the luminal mucosa of the stomach and intestinal tract and may, occasionally, complicate the course of celiac disease. Collagenous colitis has been associated with celiac disease and may lead to chronic diarrhea. Conversely, some of these clinical disorders that involve the luminal mucosa of the stomach and intestine may represent the initial clinical presentation of celiac disease. These disorders should be considered in patients with celiac disease who develop recurrent or refractory symptoms despite adherence to a strict gluten-free diet. Detection of collagenous disorders that affect the luminal mucosa of the stomach or intestinal tract may result in recognition of underlying celiac disease.