Extraintestinal Manifestations Research Articles

Specific Oral Manifestations in Adults with Crohn’s Disease

Background: Oral manifestations of Crohn’s disease (CD) include non-specific lesions and specific lesions directly related to intestinal inflammation. Oral lesions that can be overlooked in CD are sometimes challenging to treat. Methods: In this retrospective single-center study, patients with CD aged over 18 years who complied with follow-up and treatment were included. Clinical definitions of specific oral lesions included pyostomatitis vegetans, glossitis with fissuring, lip swelling with fissuring, cobblestoning, and orofacial granulomatosis. Experienced dentists confirmed the specific lesions in each case. Three groups of patients were identified: those without oral lesions, those with non-specific oral lesions, and those with specific oral lesions. The groups were compared based on demographics, disease extent and behavior (based on the Montreal classification), extraintestinal involvement, biologic and steroid treatment, and the requirement of resective surgery. Results: A total of 96 patients (14.2%) with oral lesions were found among the 676 patients with CD (59.7% male, median age 38 years) who were followed for 6.83 years (IQR 0.5–29.87 years). Eight patients (1.2%, 9 lesions) had specific oral lesions, while eighty-eight patients (13%) had non-specific lesions. Orofacial granulomatosis (n = 3), cobblestoning (n = 2), glossitis with fissuring (n = 2), and lip swelling with fissuring (n = 2) were among the specific lesions. The majority of patients (75%) with specific lesions were male, and their median age was 46.5 years (range: 23–68 years). Disease localization was commonly ileocolonic (50%), and perianal disease was present in 25% of patients. Three patients were active smokers. Extraintestinal manifestations were peripheral arthritis/arthralgia (n = 7) and sacroiliitis (n = 1). All specific lesions were associated with moderate-to-severe disease. Five patients improved with biologic therapy, and two patients with immunomodulatory therapy. Conclusions: Specific oral lesions in CD were associated with active disease and improved with immunomodulators or biologic therapy. Close cooperation between gastroenterologists and dentists is essential for early diagnosis and optimal management of CD.

A real-life pediatric experience of Crohn's disease exclusion diet at disease onset and in refractory patients.

We aimed to appraise the real-life efficacy of Crohn's disease exclusion diet (CDED) coupled with partial enteral nutrition (PEN) in inducing clinical and biochemical remission at disease onset and in patients with loss of response to biologics and immunomodulators. We retrospectively gathered data of patients aged less than 18 years of age with a diagnosis of Crohn's disease (CD), who received CDED coupled with PEN at a tertiary level pediatric inflammatory bowel disease center. Sixty-six patients were identified. Forty (60.6%) started CDED plus PEN at disease onset and 26 (39.4%) received CDED with PEN as add-on therapy. Forty-six (69.7%) patients achieved clinical remission (weighted Pediatric Crohn's Disease Activity Index < 12.5) at the end of Phase 1, 44 (66.7%) normalized c-reactive protein levels (<0.5 mg/dL) and 18 (27.2%) patients normalized calprotectin levels (<150 microg/g). Nine of 19 (47.3%) of patients with clinically severe disease (defined by Physician Global Assessment) achieved clinical remission at the end of Phase I. Patients with extraintestinal manifestations had statistically lower clinical response rates to the dietary regimen (p = 0.018). Among patients who received CDED + PEN as add-on treatment, a previous successful course of Exclusive Enteral Nutrition was associated with statistically higher clinical remission rates at Week 8 (p = 0.026). Clinical response at Week 4 was an independent predictor of clinical remission and fecal calprotectin normalization at Week 8 (p = 0.002). CDED with PEN confirmed its efficacy in a real-life setting, proving to be effective also in refractory patients and those with severe disease. Early clinical response predicts clinical remission at the end of Phase 1.

Changes in Serum Oncostatin M Levels During Treatment of Inflammatory Bowel Disease

Abstract Background Inflammatory bowel diseases (IBD) consist of Crohn’s disease (CD) and ulcerative colitis (UC). They are characterized by a chronic relapsing and remitting disease course that results in intestinal symptoms but also frequently in extra-intestinal manifestations. Among potential targets and biomarkers, oncostatin M (OSM) has gained a lot of interest. OSM is a pleiotropic cytokine produced by activated T cells, monocytes, macrophages, and neutrophils. It is considered proinflammatory given its ability to promote leukocyte recruitment. Objective To evaluate the changes in serum Oncostatin M levels during treatment of inflammatory bowel disease. Patients and Methods To elucidate this aim 30 IBD patients and 30 controls were included in this study. Results The mean Oncostatin M in IBD cases before treatment was 120.13 while after treatment was 91.17 with high significant difference. There was high statistical correlation in between Fecal Calprotectin Level after treatment and Oncostatin M After Treatment. The relation between UC, CD and Oncostatin level before and after treatment shows high statistical significant difference. There was no statistical relation in between Oncostatin level (after treatemnt) and type of treatment. That there was a high statistically significant difference in between cases and control group regarding to HB, WBCs, ESR, CRP, Urea, ALT, Oncostatin M and a statistically significant difference in between cases and control group regarding to Platelets. Conclusion Oncostatin M is a promising marker for the diagnosis and follow up of IBD patients, however it has a limited predictive performance for the prediction of the response for IBD treatment.

Beyond the Gut: Extraintestinal Manifestations of Inflammatory Bowel Disease in Children and Adolescents

Beyond the Gut: Extraintestinal Manifestations of Inflammatory Bowel Disease in Children and Adolescents

Neuroimmune response and oxidative stress in the prefrontal cortex mediate seizure susceptibility in experimental colitis in male mice.

Inflammatory bowel disease (IBD) is a chronic gastrointestinal disorder. Oxidative stress and inflammatory responses have a vital role in the pathophysiology of IBD as well as seizure. IBD is associated with extraintestinal manifestations. This study aimed to explore the relationship between colitis and susceptibility to seizures, with a focus on the roles of neuroinflammation and oxidative stress in acetic acid-induced colitis in mice. Forty male Naval Medical Research Institute mice were divided into four groups: control, colitis, pentylenetetrazole (PTZ), and colitis + PTZ. Colitis was induced by intrarectal administration of acetic acid, and seizures were induced by intravenous injection of PTZ 7 days postcolitis induction. Following the measurement of latency to seizure, the mice were killed, and their colons and prefrontal cortex (PFC) were dissected. Gene expression of inflammatory markers including interleukin-1β, tumor necrosis factor-alpha, NOD-like receptor protein 3, and toll-like receptor 4, as well as total antioxidant capacity (TAC), malondialdehyde (MDA), and nitrite levels were measured in the colon and PFC. Histopathological evaluations were performed on the colon samples. Data were analyzed by t-test or one-way variance analysis. Colitis decreased latency to seizure, increased gene expression of inflammatory markers, and altered levels of MDA, nitrite, and TAC in both the colon and PFC. Simultaneous induction of colitis and seizure exacerbated the neuroimmune response and oxidative stress in the PFC and colon. Results concluded that neuroinflammation and oxidative stress in the PFC at least partially mediate the comorbid decrease in seizure latency in mice with colitis.

Inflammatory Bowel Disease-Associated Arthritis Is Associated with Concomitant Autoimmune and Inflammatory Disorders.

Extraintestinal Manifestations (EIMs) are a common and potentially debilitating complication of Inflammatory Bowel Diseases (IBD), sometimes requiring additional treatment beyond those used to control intestinal disease. IBD-associated arthritis (IAA), a form of spondyloarthritis, is associated with several factors including disease location, sex, and IBD type. However, much remains unknown about other clinical factors predicting development of EIMs. Our goal was to identify additional factors associated with IAA. Participants in the LOCATION-IBD cohort were included in this analysis. We performed univariate and multivariate analysis of demographics, clinical data, and patient-reported outcomes data. The LOCATION-IBD cohort included 182 participants with (n = 53) and without (n = 110) joint EIMs and with joint pain of unclear etiology (n = 19). In a multivariate analysis comparing those with and without joint EIMs, female sex (OR = 2.5, p = 0.014), the presence of concomitant autoimmune and inflammatory disorders (OR = 2.5, p = 0.038), and Crohn's disease (OR = 2.9, p = 0.026) were associated with the presence of joint EIMs. This analysis reveals patients with IAA are more likely to have concomitant autoimmune disorders. Further studies are needed to confirm this association, understand the mechanisms underlying the common pathogenesis of these concurrent disorders, and evaluate their impact on the treatment of IAA.

Inflammatory bowel disease training assessment of gastroenterology fellows in Saudi Arabia.

Recent advancement and complexity in the management of inflammatory bowel disease (IBD) has made it challenging for gastroenterology (GI) fellows to obtain competency and confidence in managing the complex IBD patient. We aimed to evaluate the confidence and training in IBD among GI fellows in Saudi Arabia. We conducted an electronic, voluntary, and anonymous multicenter survey study of GI fellows in Saudi Arabia, from 1/5/2023 to 1/9/2023. The survey evaluated the fellows' confidence level in IBD management, methods of training received, and the amount of additional training desired in 20 core IBD domains. GI fellows' preferred learning method was also evaluated. A total of 65 GI fellows responded to the survey. In the entire cohort, >50% of fellows reported low confidence in 7 out of 20 IBD management domains, which included 71% in managing j-pouch disorders, 67% in managing the elderly/frail patient with IBD, 60% in managing extraintestinal manifestations, 57% in recommending preventative health services, and 54% in counseling patients on small molecules. Receiving >4 IBD didactic sessions per year was significantly associated with high confidence in managing j-pouch disorders (44.4% vs 13.3%, P = 0.05) and managing the elderly/frail patient with IBD (86.7% vs 50.0%, P = 0.03). Doing an external rotation to expand IBD knowledge was associated with high confidence in managing the elderly/frail patient with IBD (100% vs 26.7%, P = 0.01). Many GI fellows lacked confidence and training in key domains of IBD management. Enhancing IBD exposure with didactics and external rotations improved fellows' confidence in specific domains.

Distinct metabolomic and lipidomic profiles in serum samples of patients with primary sclerosing cholangitis.

Identification of specific metabolome and lipidome profile of patients with primary sclerosing cholangitis (PSC) is crucial for diagnosis, targeted personalized therapy, and more accurate risk stratification. Nuclear magnetic resonance (NMR) spectroscopy revealed an altered metabolome and lipidome of 33 patients with PSC [24 patients with inflammatory bowel disease (IBD) and 9 patients without IBD] compared with 40 age-, sex-, and body mass index (BMI)-matched healthy controls (HC) as well as 64 patients with IBD and other extraintestinal manifestations (EIM) but without PSC. In particular, higher concentrations of pyruvic acid and several lipoprotein subfractions were measured in PSC in comparison to HC. Of clinical relevance, a specific amino acid and lipid profile was determined in PSC compared with IBD and other EIM. These results have the potential to improve diagnosis by differentiating PSC patients from HC and those with IBD and EIM.

Manifestations Reported by Inflammatory Bowel Disease Patients in University Hospital.

Inflammatory bowel disease (IBD) may affect organs outside the intestines, it is called extraintestinal manifestations of IBD. Data on the prevalence of mu-cocutaneous manifestations in IBD patients are very limited, therefore, the aim of this study was to assess the prevalence of skin and mucosal lesions and to determine the relationship with demographic factors, clinical features, and systemic treatment. Prospective study included 162 out-patients with IBD who were managed in the tertiary care center. Ulcerative colitis (UC) was diagnosed in 117 patients, Crohn's disease (CD) in 45. Patients completed the questionnaire containing demographic and IBD data, questions about mucocutaneous lesions (in past or present state). Overall mucocutaneous lesions were reported by 48.1% of IBD patients. Skin lesions were reported by 40.7% of patients, oral mucosal lesions were reported by 16.7%, without significant differences between sexes or IBD types. In 47 (29%) of patients, skin lesions appeared together with IBD or during the course of the disease. The most common skin lesions were psoriasis (8.0%), erythema nodosum (5.6%), pyoderma gangrenosum and acne (3.7% each). UC patients mostly reported about psoriasis (9.4%), while CD patients about erythema nodosum (11.1%). There were more frequent skin lesions in patients with more extensive UC type (p = 0.01), while no difference was noticed between different types of CD. The average duration of IBD in patients with skin lesions was similar to those without lesions (9.3±6.7 vs. 9.4±6.7 years). Mucocutaneous lesions were reported by 48.1% of inflammatory bowel disease patients. The frequency of mucocutaneous lesions does not differ significantly between UC and CD, and a longer duration of illness is not a predictive factor for the appearance of lesions. More extensive UC is related to higher frequency of skin lesions.

Juvenile polyposis syndrome in children: The impact of SMAD4 and BMPR1A mutations on clinical phenotype and polyp burden.

A constitutional disease-causing variant (DCV) in the SMAD4 or BMPR1A genes is present in 40%-60% of patients with juvenile polyposis syndrome (JPS). The aim of this study was to characterize the clinical course and polyp burden in children with DCV-positive JPS compared to DCV-negative JPS. Demographic, clinical, genetic, and endoscopic data of children with JPS were compiled from eight international centers in the ESPHGAN/NASPGHAN polyposis working group. A total of 124 children with JPS were included: 69 (56%) DCV-negative and 55 (44%) DCV-positive (53% SMAD4 and 47% BMPR1A) with a median (interquartile range [IQR]) follow-up of 4 (2.8-6.4) years. DCV-positive children were diagnosed at an older age compared to DCV-negative children [12 (8-15.7) years vs. 5 (4-7) years, respectively, p < 0.001], had a higher frequency of family history of polyposis syndromes (50.9% vs. 1.4%, p < 0.001), experienced a greater frequency of extraintestinal manifestations (27.3% vs. 5.8%, p < 0.001), and underwent more gastrointestinal surgeries (16.4% vs. 1.4%, p = 0.002). The incidence rate ratio for the development of new colonic polyps was 6.15 (95% confidence interval 3.93-9.63, p < 0.001) in the DCV-positive group compared to the DCV-negative group, with an average of 12.2 versus 2 new polyps for every year of follow-up. There was no difference in the burden of polyps between patients with SMAD4 and BMPR1A mutations. This largest international cohort of pediatric JPS revealed that DCV-positive and DCV-negative children exhibit distinct clinical phenotype. These findings suggest a potential need of differentiated surveillance strategies based upon mutation status.

Association between inflammatory bowel disease and primary sclerosing cholangitis: Insights from bibliometric analysis.

Crohn's disease and ulcerative colitis are forms of inflammatory bowel disease affecting approximately 1% of the population. Their typical features include chronic diarrhea, abdominal pain, and weight loss. Extraintestinal manifestations may coincide with or precede the diagnosis of these diseases. Primary sclerosing cholangitis is one such extraintestinal manifestation. Although many papers on this field have been published, bibliometric analysis still needs to be conducted. This article summarizes the current research progress through a bibliometric study, provides an overview of the research status in this field, and analyzes recent research trends. Publications on inflammatory bowel disease and primary sclerosing cholangitis from January 1, 2008, to August 31, 2023, were extracted from the Web of Science Core Collection. VOSviewer and CiteSpace were used to perform a bibliometric and visual study. There are 1499 relevant articles, and the number of articles in this field has been relatively stable in recent years. The results indicate that Karlson TH from the University of Oslo has the highest cumulative number of publications. The institution with the highest publication output is the Mayo Clinic, and the United States leads in article production, occupying a dominant position. Keyword analysis reveals 4079 keywords, with primary sclerosing cholangitis, inflammatory bowel disease, and ulcerative colitis being the most frequently occurring keywords. Research on the association between inflammatory bowel disease and primary sclerosing cholangitis is steadily advancing, with the United States leading in publication output globally. China needs to invest more in research in this area, and collaboration among institutions should be strengthened. The research hotspots revolve around the association between inflammatory bowel disease and primary sclerosing cholangitis, gut microbiota, and other fields.

#1852 When ‘pebbles’ affect the kidney: nephrolithiasis and chronic renal failure in Crohn's disease. The experience of a 3rd level centre

Abstract Background and Aims Nephrolithiasis is the most common expression of kidney involvement in chronic inflammatory bowel disease (IBD), especially in Crohn's disease (CD). Stone formation favoring factors are hyperoxaluria, dehydration, acidosis, common conditions in CD patient, who present a little higher incidence of renal stones, made of calcium oxalate, than the normal population. Only few studies focused on the relationship between CD activity (defined by CDAI score) and the development of nephrolithiasis and possible subsequent development of chronic renal failure (CRF). Materials and Method We conducted a retrospective study on a cohort of 1236 patients affected by CD referring to our Centre of IBD- Sant'Orsola between 2014 and 2021. We identified two cohorts from this population: 118 patients with nephrolithiasis and 118 patients without nephrolithiasis. Among these two groups, we identified variable clinical manifestations of CD and their relation with CRF and lithiasis. Results The prevalence of nephrolithiasis in all CD patients was 10.3%, a little higher than in the general population. The results of the analysis of patient and disease variables in the two groups are shown in Table 1. The mean age at diagnosis of CD was higher in the group without nephrolithiasis, so the treatment was started later. The presence of perianal disease, extraintestinal manifestations (MEI), like ankylosing spondylitis (APS), biliary tract involvement, or previous surgery, and presence of ileostomy correlate with the formation of stones in univariate regression analysis (Table 2). Perianal disease and MEI also maintained significance in multivariate analysis. Conclusion Our data suggest that a higher degree of disease activity, defined by the presence of perianal disease (a variable on CDAI score) and the presence of MEI, correlates with higher risk of lithiasis. It is advisable to start early the study of nephrolithiasis in these patients to adopt preventive strategies against formation and progression of renal stones. Future studies are required for a better definition of this deep relationship.

Challenges in IBD Research 2024: Preclinical Human IBD Mechanisms.

Preclinical human inflammatory bowel disease (IBD) mechanisms is one of 5 focus areas of the Challenges in IBD Research 2024 document, which also includes environmental triggers, novel technologies, precision medicine, and pragmatic clinical research. Herein, we provide a comprehensive overview of current gaps in inflammatory bowel diseases research that relate to preclinical research and deliver actionable approaches to address them with a focus on how these gaps can lead to advancements in IBD interception, remission, and restoration. The document is the result of multidisciplinary input from scientists, clinicians, patients, and funders and represents a valuable resource for patient-centric research prioritization. This preclinical human IBD mechanisms section identifies major research gaps whose investigation will elucidate pathways and mechanisms that can be targeted to address unmet medical needs in IBD. Research gaps were identified in the following areas: genetics, risk alleles, and epigenetics; the microbiome; cell states and interactions; barrier function; IBD complications (specifically fibrosis and stricturing); and extraintestinal manifestations. To address these gaps, we share specific opportunities for investigation for basic and translational scientists and identify priority actions.

Predictors of Complicated Disease Course in Children andAdults With Ulcerative Colitis: A Nationwide Study From the epi-IIRN.

Data on predictors of complicated ulcerative colitis (UC) course from unselected populations cohorts are scarce. We aimed to utilize a nationwide cohort to explore predictors at diagnosis of disease course in children and adults with UC. Data of patients diagnosed with UC since 2005 were retrieved from the nationwide epi-IIRN cohort. Complicated disease course was defined as colectomy, steroid-dependency, or the need for biologic drugs. Hierarchical clustering categorized disease severity at diagnosis based on complete blood count, albumin, C-reactive protein and erythrocyte sedimentation rate (ESR), analyzed together. A total of 13 471 patients with UC (1427 [11%] pediatric-onset) including 103 212 person-years of follow-up were included. Complicated disease course was recorded in 2829 (21%) patients: 1052 (7.9%) escalated to biologics, 1357 (10%) experienced steroid-dependency, and 420 (3.1%) underwent colectomy. Probabilities of complicated disease course at 1 and 5 years from diagnosis were higher in pediatric-onset (11% and 32%, respectively) than adult-onset disease (4% and 16%; P < .001). In a Cox multivariate model, complicated course was predicted by induction therapy with steroids (hazard ratio [HR], 1.5; 95% CI, 1.2-2.0), extraintestinal manifestations (HR, 1.3; 95% CI, 1.03-1.5) and the disease severity clusters of blood tests (HR, 1.8; 95% CI, 1.01-3.1), while induction therapy with enemas (HR, 0.6; 95% CI, 0.5-0.7) and older age (HR, 0.99; 95% CI, 0.98-0.99) were associated with noncomplicated course. In this nationwide cohort, the probability of complicated disease course during the first 5 years from diagnosis was 32% in pediatric-onset and 16% in adults with UC and was associated with more severe clusters of routinely collected laboratory tests, younger age at diagnosis, extraintestinal manifestations, and type of induction therapy.

Acute Kidney Injury in Inflammatory Bowel Disease Patients: A Nationwide Comparative Analysis

Rationale & ObjectiveAbout 25-40% of patients with inflammatory bowel diseases (IBD) may have extra-intestinal manifestations, mainly involving the liver, skin, and joints. Kidney involvement in patients with IBD has been reported, but there are no estimates of its prevalence in population-based studies in the US. We compared the frequency of AKI among hospitalizations with IBD to hospitalizations with collagen vascular diseases and hospitalizations without IBD or collagen vascular diseases. Study DesignRetrospective, population-based cohort study Setting and ParticipantsHealthcare Cost and Utilization Project (HCUP)--Nationwide Inpatient Sample (NIS) database. ExposureTK OutcomesAcute kidney injury (AKI) and AKI requiring dialysis. Analytical ApproachRegression models were used to compare occurrence of AKI among groups. Inverse probability of treatment weighting (IPTW) applied to balance groups on covariates. ResultsThe final sample comprised of 5,735,804 hospitalizations, including 57,121 with IBD, 159,930 with collagen vascular diseases and 5,518,753 without IBD or collagen vascular diseases. AKI was observed in 13%, 15%, and 12.2% of hospitalizations with IBD, collagen vascular diseases, and the general population, respectively. When adjusting for demographic, hospital, and clinical characteristics using IPTW, hospitalizations with IBD had higher odds of being diagnosed with AKI compared to both the collagen vascular diseases (OR, 1.32; 95% CI, 1.27-1.38) and general population (OR 1.27, 95% CI, 1.23-1.31), and also had higher odds of being diagnosed with AKI requiring dialysis compared to collagen vascular diseases (OR, 1.59; 95% CI 1.31, 1.94) and general population (OR,1.45; 95% CI 1.25, 1.68). LimitationsCross-sectional analysis, underreporting of ICD codes, and analyses relevant to in-hospital stays only. ConclusionsThe prevalence and risk of AKI among hospitalization with IBD is greater than that of the hospitalization in the collagen vascular diseases and the general population cohort. Coexisting kidney disease should be considered among patients with a known diagnosis of IBD.

Liver Disease Associated with Inflammatory Bowel Disease

Inflammatory Bowel Disease (IBD) is a chronic condition characterized by persistent inflammation of the gastrointestinal tract, comprising ulcerative colitis (UC) and Crohn’s disease (CD). While the current global incidence of IBD exceeds 0.3% in Western nations, Indonesia reports a comparatively lower prevalence however with projections indicate a potential rise in incidence from 2020 to 2050. Hepatobiliary manifestation is one of the most common extraintestinal manifestations of IBD. Some of the liver disease associated with IBD had similar background immune response, inflammatory reaction, similar risk factor, or due to the treatment of IBD. Primary sclerosing cholangitis (PSC) has close association with IBD and both affect the prognostic and disease progression of each other. Non-alcoholic fatty liver disease (NAFLD) shares common metabolic risk factors. Several drugs used as the treatment of IBD might cause hepatic injury especially due to the long treatment duration of IBD. Hepatitis B reactivation is another concerning event found after prolonged used of immunosuppressive drugs of IBD. Therefore, close monitoring of liver function test periodically is a mandatory test to screen liver disease in IBD patients. Keyword: Inflammatory bowel disease, liver disease, autoimmune hepatitis, drug-induced liver injury

Unveiling the Potential of JAK Inhibitors in Inflammatory Bowel Disease

Background: Janus kinase (JAK) inhibitors represent a novel class of oral therapies showing efficacy in treating ulcerative colitis (UC) and Crohn’s disease (CD), challenging conventional treatment paradigms. Summary: This review provides an overview of the potential novel uses of JAK inhibitors, focusing on their current approved indications and exploring possibilities beyond these indications. Tofacitinib and filgotinib are approved for UC, while upadacitinib is approved for both UC and CD. Additionally, their potential in acute severe UC, as steroid alternatives, and in managing fistulizing CD or extraintestinal manifestations are discussed. Key Message: JAK inhibitors play an important role in IBD (inflammatory bowel disease) treatment; however, clinicians must balance their promising efficacy with safety concerns. Individualized care and vigilance are essential for optimizing therapeutic benefits while mitigating potential adverse effects. Further research is necessary to clarify their efficacy, safety, and potential applications.

The problem of excessive hair loss as a signal for the diagnosis of coeliac disease – A family case

Celiac disease is an autoimmune disorder in which the small intestine is damaged after ingesting gluten. Baldness often co-occurs with various systemic diseases including autoimmune diseases. In addition, alopecia can result from nutritional deficiencies. In the case of celiac disease, we are mainly talking about deficiencies of iron, fat-soluble vitamins (A,D,E,K), vitamin B12 and folic acid. Telogenetic alopecia (TE) is diffuse and reversible alopecia, and the causes of this condition can be nutritional deficiencies and impaired absorption. Celiac disease has been linked to alopecia areata (AA). We describe the case of a mother and daughter with a history of hair loss, which represented the only incipient (mother’s case) and subsequent (daughter’s case) extraintestinal manifestation of celiac disease.

Portal vein thrombosis as extraintestinal complications of Crohn’s disease: a case report and review of literature

IntroductionThrombotic events are more than twice as common in inflammatory bowel disease patients as in the general population. We report an interesting and rare case of portal vein thrombosis as a venous thromboembolic event in the context of extraintestinal manifestations of Crohn’s disease. We also conducted a literature review on portal vein thrombosis associated with inflammatory bowel disease, with the following concepts: inflammatory bowel diseases, ulcerative colitis, Crohn’s disease, portal vein, and thrombosis.Case presentationA 24-year-old Syrian female with active chronic Crohn’s disease was diagnosed 11 years ago and classified as A1L3B1P according to the Montreal classification. She had no prior surgical history. Her previous medications included azathioprine and prednisolone. Her Crohn’s disease activity index was 390 points. Gastroduodenoscopy revealed grade I esophageal varices, a complication of portal hypertension. Meanwhile, a colonoscopy revealed several deep ulcers in the sigmoid, rectum, and descending colon. An investigation of portal vein hypertension revealed portal vein thrombosis. We used corticosteroids to induce remission, followed by tapering; additionally she received ustekinumab to induce and maintain remission. She began on low-molecular-weight heparin for 1 week, warfarin for 3 months, and then apixaban, a novel oral anticoagulant, after excluding antiphospholipid syndrome. Primary prophylaxis for esophageal varices was not required. After 1 year, she achieved clinical, biochemical, and endoscopic remission. Despite 1 year of treatment, a computed tomography scan revealed no improvement in portal vein recanalization.ConclusionPortal vein thrombosis is a rare and poorly defined complication of inflammatory bowel disease. It is usually exacerbated by inflammatory bowel disease. The symptoms are nonspecific and may mimic a flare-up of inflammatory bowel disease, making the diagnosis difficult. Portal vein Doppler ultrasound for hospital-admitted inflammatory bowel disease patients may contribute to the diagnosis and management of this complication.

The impact of vedolizumab therapy on extraintestinal manifestations in patients with inflammatory bowel disease: A systematic review and meta-analysis.

Extraintestinal manifestations (EIMs) pose a significant threat in inflammatory bowel disease (IBD) patients. Vedolizumab (VDZ) primarily affects the gastrointestinal tract. However, its impact on EIMs remains uncertain. Therefore, we conducted this meta-analysis to examine the effects of VDZ on EIMs during treatment. Relevant studies were identified by conducting thorough searches across electronic databases, including PubMed, Ovid Embase, Medline, and Cochrane CENTRAL. Primary outcomes focused on the proportion of patients with resolution for pre-existing EIMs in IBD patients receiving VDZ. Secondary outcomes included the proportion of patients with EIM exacerbations and new onset EIMs during VDZ treatment. Our meta-analysis encompassed 21 studies. The proportion of patients with resolution of pre-existing EIMs in VDZ-treated IBD patients was 39% (150/386; 95% confidence interval [CI] 0.31-0.48). The proportion of patients with EIM exacerbations occurred at a rate of 28% (113/376; 95% CI 0.05-0.50), while new onset EIMs had a rate of 15% (397/2541; 95% CI 0.10-0.20). Subgroup analysis revealed a 40% (136/337) proportion of patients with resolution for articular-related EIMs and a 50% (9/18) rate for erythema nodosum. Exacerbation rates for arthritis/arthralgia, erythema nodosum/pyoderma gangrenosum, and aphthous stomatitis during VDZ use were 28% (102/328), 18% (7/38), and 11% (3/28), respectively. The incidence rate of newly developed EIMs during treatment was 11% (564/4839) for articular-related EIMs, with other EIMs below 2%. VDZ demonstrates efficacy in skin-related EIMs like erythema nodosum and joint-related EIMs including arthritis, arthralgia, spondyloarthritis, and peripheral joint diseases. Some joint and skin-related EIMs may experience exacerbation during VDZ therapy.