[Collagenous sprue in a patient with severe diarrhoea, malnutrition and acute renal failure].

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 Enteritis is Unlikely a Form of Aggressive Celiac Disease Despite Sharing HLA-DQ2/DQ8 Genotypes.

Collagenous enteritis is an uncommon small intestinal injury pattern with unclear pathogenesis. While it has been speculated that collagenous enteritis represents a form of refractory celiac disease, recent clinical studies suggest a potential link to exposure to the antihypertensive medication olmesartan. Here we hypothesized that the pathogenesis of collagenous enteritis involves both genetic and environmental factors. All subjects with biopsy-proven collagenous enteritis diagnosed between 2002 and 2015 were identified from 2 tertiary care medical centers. Human leukocyte antigen (HLA)-DQ genotyping was performed by polymerase chain reaction on archived tissue. Celiac disease serology, past medical history, medications, smoking history, demographics, histology, clinical management, and follow-up were recorded. A total of 32 subjects were included. In contrast to celiac disease, subjects with collagenous enteritis were mostly elderly (median age at diagnosis, 69 y; range, 33 to 84 y). Seventy percent of collagenous enteritis subjects harbored celiac disease susceptibility alleles HLA-DQ2/DQ8; however, only 1 subject had elevated serum levels of celiac disease-associated autoantibodies while on a gluten-containing diet. Furthermore, 56% of subjects were taking nonsteroidal anti-inflammatory drugs, 36% proton-pump inhibitors, 28% statins, and 32% olmesartan at the time of diagnosis. Discontinuation of olmesartan and treatments with steroids and/or gluten-free diet resulted in symptomatic and histologic improvement. Neither lymphoma nor collagenous enteritis-related death was seen in this cohort. Therefore, while collagenous enteritis shares similar HLA genotypes with celiac disease, the difference in demographics, the lack of celiac disease-associated autoantibodies, and potential link to medications as environmental triggers suggest the 2 entities are likely distinct in pathogenesis.

Collagenous sprue cross-sectional imaging: a comparative blinded study.

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.

Comparison of clinical features, treatment, and outcomes of collagenous sprue, celiac disease, and collagenous colitis.

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.

Enteropatía asociada a olmesartán con atrofia vellositaria en paciente con genotipo HLA-DQ2 y anticuerpos-antinucleares positivos / Sprue-like enteropathy associated with olmesartan in a patient with villous atrophy, HLA-DQ2 genotype and antinuclear antibodies

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Adding Water to the Mill: Olmesartan-Induced Collagenous Sprue-A Case Report and Brief Literature Review.

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.

Duodenal Villous Atrophy in a TTG-Negative Patient Taking Olmesartan: A Case Report and Review of the Literature.

Olmesartan, an angiotensin II receptor antagonist used to treat hypertension, is associated with few adverse effects. Here, a case of severe sprue-like enteropathy and acute kidney injury is described in a 68-year-old male taking olmesartan for 3-4 years. He presented to hospital with a five-week history of diarrhea, vomiting, and a 20 lb weight loss. Anti-TTG was negative with a normal IgA. Biopsies of the distal duodenum and duodenal cap revealed marked blunting of the villi with near complete villous atrophy of the biopsies from the bulb. There was an increase in intraepithelial lymphocytes as well as neutrophils in the surface epithelium. The patient's diarrhea improved upon discontinuation of olmesartan and he returned to his previous weight. Repeat endoscopy four months later demonstrated complete resolution of inflammatory change with normal villous architecture. Long-term olmesartan use is associated with severe sprue-like enteropathy. The mechanism of intestinal injury is unknown. Duodenal biopsy results may mimic other enteropathies such as celiac disease. Physicians should consider medications as potential etiologies of enteropathy.

Collagenous enterocolitis manifesting as watery diarrhoea and iron-deficiency anaemia.

A 56-year-old Caucasian woman presented with epigastric pain, watery diarrhoea, bloating and flatulence following treatment with duloxetine and venlafaxine for anxiety and depression. Abdominal examination was benign. Blood work revealed haemoglobin of 96 g/L (115-160 g/L), iron 6 µmol/L (10-33 µmol/L), transferrin saturation 0.08 (0.20-0.55), ferritin 26 µg/L (15-180 µg/L), albumin 46 g/L (35-50 g/L), pre-albumin 293 mg/L (170-370 mg/L), total IgA 2.64 g/L (0.78-3.58 g/L) and anti-tTG IgA 5 units (<20 units). Faecal occult blood tests were 3/3 positive and stool cultures were negative. CT enterography was normal. Colonic biopsy revealed collagenous colitis, while duodenal biopsy showed collagenous sprue with blunted to completely flattened villi and markedly thickened subepithelial collagen table entrapping capillaries and lymphocytes. The patient started a gluten-free diet, loperamide and ferrous gluconate. Her symptoms resolved and a faecal immunochemical test performed 6 months later was negative.

Celiac disease, collagenous sprue and microscopic colitis in IBD. Observations from a population-based cohort of IBD (ICURE).

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.

An elderly lady with significant weight loss.

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.

Collagenous sprue in a 3-month-old infant.

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.

Atrofia vellositaria sin enfermedad celíaca: ¿un nuevo síndrome o más confusión? / Non celiac villous atrophy: More confusion or a new syndrome?

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A rare cause of protein losing enteropathy: collagenous sprue.

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.

Esprúe colágeno: ¿entidad secundaria o independiente de la enfermedad celíaca? / Collagenous sprue: secondary or independent from celiac disease?

El esprúe colágeno es una entidad poco prevalente que cursa con diarrea persistente con pérdida de peso y malabsorción por afectación del intestino delgado, principalmente duodeno y yeyuno proximal, necesitando para el diagnóstico la presencia de una clínica y una histología compatible con atrofia y depósito subepitelial de colágeno. Su etiología no es totalmente conocida, aunque su origen más propuesto es el autoinmune, ya que está ampliamente relacionada con la enfermedad celíaca e incluso se ha propuesto que se trate de una evolución de celiaquía refractaria a dieta sin gluten. En relación a esta incertidumbre presentamos el caso de una paciente con diarrea malabsortiva e importante repercusión clínica por esprúe colágeno, la cual tuvo una buena respuesta a corticoides orales (prednisona), pero hubo que añadir azatioprina. Además mejoró inicialmente con nutrición parenteral central domiciliaria...

Collagenous sprue is a rare disease that goes with persistent diarrhea, weight loss and bad absortion, because it affects the small intestine, mainly duodenum and proximal jejunum. Diagnosis is made by having clinical signs and histological proof of atrophy and subepitelial deposit of collagenous material. Its etiology is not known completely, it is proposed that the origin is autoimmune because its relationship with celiac disease. Also there is a proposal that is a celiac evolution to gluten free diet. Is because this is not clear that we present a case of a patient with bad absorptive diarrhea and a clinical expression of collagenous sprue, that had a great clinical response to corticosteroids (prednisone) but we had to add azatioprine. Also, initially improved with home parenteral nutrition center...