Request Demo

Last update 08 May 2025

Immunoproliferative Small Intestinal Disease

Last update 08 May 2025

Basic Info

Synonyms

ALPHA CHAIN DISEASE, ALPHA HCD, ALPHA HEAVY CHAIN DISEASE

+ [62]

Introduction

A condition that is caused by HYPERPLASIA of LYMPHOCYTES in the small intestine (INTESTINE, SMALL) and the mesenteric LYMPH NODES. These lymphocytes produce an anomalous alpha heavy chain protein. Generally, these IPSID patients have either concurrent LYMPHOMA or develop lymphoma within a few years. The disease was first described in the Mediterranean region and is characterized by malabsorption; WEIGHT LOSS; DIARRHEA; and STEATORRHEA.

Clinical Trials associated with Immunoproliferative Small Intestinal Disease

NCT06462352

/ RecruitingNot ApplicableIIT

Multicenter Study Comparing the Artificial Intelligence-Based Navicam Endoscopic Capsule With the Conventional Pillcam Endoscopic Capsule in Small Bowel Pathology

Since its introduction in 2001, small bowel capsule endoscopy has been pivotal in diagnosing small bowel pathology due to its minimally invasive nature and high diagnostic accuracy. However, the technology has limitations, including prolonged reading times and the need for specialized endoscopists. The Navicam endoscopic capsule, leveraging artificial intelligence (AI) with ProScan™ for automated reading, promises to address these limitations by reducing reading times and enhancing diagnostic efficiency.
This study aims to assess the diagnostic concordance and to compare the efficiency of the AI-based Navicam capsule with the conventional Pillcam SB3 in the exploration of the small bowel.

Start Date27 May 2024

Sponsor / Collaborator

Hospital Clínic de Barcelona

NCT05450393

/ WithdrawnNot Applicable

Prospective Collection of PillCamTM SB3 Capsule Endoscopy and Device-Assisted Enteroscopy Procedures Data for Development of an Artificial Intelligence Software System to Apply to Endoscopy Procedures (GPS Study)

A prospective, multicenter, post-market, minimal risk, observational study designed to collect PillCamTM SB3 capsule endoscopy (CE) procedures and subsequent device-assisted enteroscopy (DAE) procedures data (double balloon enteroscopy (DBE), single balloon enteroscopy (SBE) and spiral enteroscopy (SE)) in medical centers in the United States (US).

Start Date01 May 2024

Sponsor / Collaborator-

NCT05470946

/ Unknown statusNot ApplicableIIT

Evaluation of the Diagnostic Role of Enteroscopy in Small Intestinal Diseases at Al-Rajhi University Hospital

To identify the role of enteroscopy in diagnosis and treatment of small intestinal diseases.

Start Date01 Aug 2022

Sponsor / Collaborator

Assiut University

100 Clinical Results associated with Immunoproliferative Small Intestinal Disease

100 Translational Medicine associated with Immunoproliferative Small Intestinal Disease

0 Patents (Medical) associated with Immunoproliferative Small Intestinal Disease

568

Literatures (Medical) associated with Immunoproliferative Small Intestinal Disease

01 Mar 2025·Journal of Dairy Science

Inheritance of BLIRD disorder affects Holstein production performance and longevity

Article

Author: Null, D J ; VanRaden, P M ; Nicolazzi, E L ; Al-Khudhair, A

Improved methods previously developed for tracking new mutations within existing haplotypes for cholesterol deficiency (HCD) and muscle weakness (HMW) now also were applied to track the bovine lymphocyte intestinal retention defect (BLIRD) discovered in France. Gene tests were available in US data for HCD and HMW but not yet for BLIRD. Haplotype status for 3 million genotyped animals that also had US phenotypes were used to compare recessive effects of BLIRD homozygotes with French estimates. Heifer livability was 97.6% for normal calves with no copies of the haplotype (code 0) but averaged 88.8% for 178 homozygotes (code 2) and 94.1% for 2,029 uncertain homozygotes (code 4) with corresponding estimates of -8.6% and -3.3% from an animal model. Haplotype carriers verified by pedigree (code 1) or uncertain carriers (code 3) were not affected. Yield trait effects for 412 code 2 homozygotes were -1,799 kg milk, -63 kg fat, and -55 kg protein with a cost of -$1,206 using lifetime net merit values; other traits not yet studied may increase that cost. Mating a BLIRD carrier randomly to a population with 8.9% allele frequency would cause an economic loss of $1,653 * 0.089 / 2 = $74 because half of the progeny would inherit the carrier's normal allele. Those losses should already be reflected in evaluations which average the merit across normal, carrier, and homozygous daughters. Genomic predictions do not fully track those losses because new mutations are poorly correlated with nearby markers. However, US adjustments for future inbreeding automatically reduce evaluations of popular ancestors by more than the cost of these individual defects. Gene tests are needed for new mutations within common haplotypes because tracking can be difficult even with accurate pedigrees.

13 Jun 2024·Cureus

Treatment-Resistant Immunoproliferative Small Intestinal Disease (IPSID) Leading to Lymphoma

Article

Author: Nasir, Umair M ; Elaskandrany, Menna-Allah ; Wang, Weizheng ; Ismail, Mohamed ; Kapila, Rajendra

Immunoproliferative small intestinal disease (IPSID) is a distinct variant of mucosa-associated lymphoid tissue (MALT) lymphoma, often linked to chronic Campylobacter jejuni infection. Characterized as an extra-nodal marginal zone B-cell lymphoma, IPSID predominantly affects the proximal small intestine. It features lymphoplasmacytic infiltration and deposition of monotypic α-heavy chains in the lamina propria, leading to blunted intestinal villi, malabsorption, and protein-losing enteropathy. IPSID's clinical spectrum ranges from lymphoid infiltration to malignant diffuse large B-cell lymphoma. Similar to MALT lymphoma, early-stage IPSID can be resolved with antibiotic therapy. This case study documents a 50-year-old Nigerian woman presenting with recurrent watery diarrhea, abdominal pain, and weight loss, unresponsive to antibiotics. A 50-year-old female immigrant from Nigeria presented with recurrent watery diarrhea, abdominal pain, and significant weight loss, all refractory to antibiotic treatment. Initial diagnostic investigations revealed a positive Campylobacter stool antigen, mesenteric lymphadenopathy on CT and gallium scans, and diffuse mucosal lymphoplasmacytic infiltration with villi flattening on small bowel biopsies. An octreotide scan identified a reactive mesenteric lymph node, confirmed by surgical biopsy as reactive lymphadenitis. The patient was diagnosed with IPSID and commenced antibiotic therapy, which initially resolved her symptoms. However, she experienced frequent recurrences requiring multiple hospitalizations and repeated courses of intravenous antibiotics. Eventually, the disease progressed to lymphoma, necessitating chemotherapy initiation. This case underscores the diagnostic complexities of IPSID, particularly in distinguishing it from other causes of mesenteric lymphadenopathy. It also highlights the challenges in preventing disease progression from a benign to a malignant state despite appropriate antibiotic treatment. Given IPSID's prevalence in endemic regions, it should be considered in differential diagnoses for similar presentations. Continuous monitoring is crucial to evaluate therapeutic response and mitigate the risk of progression to lymphoma. IPSID presents a significant diagnostic and therapeutic challenge. This case exemplifies the necessity for heightened clinical awareness, especially in patients from endemic regions, and the importance of rigorous monitoring to prevent malignant transformation. Further research is warranted to elucidate the mechanisms behind IPSID progression in certain patients despite repeated antibiotic interventions.

01 Oct 2023·Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology

Falsely elevated anti-tissue transglutaminase antibodies in patients with immunoproliferative small intestinal diseases: A case series.

Article

Author: Banyal, Vikas ; Singh, Alka ; Das, Prasenjit ; Agarwal, Ashish ; Makharia, Govind ; Bagchi, Soumita ; Ahuja, Vineet ; Mohta, Srikant

Immunoproliferative small intestinal disease (IPSID) is an uncommon disease of the small intestine. There is a similarity in the clinical presentations of enteropathic diseases, including celiac disease, tropical sprue, IPSID, and Whipple's disease. A differentiation between them is based on the use of a highly specific serological test for celiac disease and specific histological characteristics. We found that IgA-anti-tissue transglutaminase antibody (IgA-tTG Ab) is falsely elevated in a subset of patients with IPSID. The levels of IgA-tTG Ab fall with the treatment of IPSID. The healthcare professional should be aware of the conditions that lead to a false-positive anti-tTG Ab. Intestinal mucosal biopsies even in the presence of anti-tTG Ab should be done in endemic regions as they provide an opportunity for making a diagnosis of alternative and uncommon diseases before the diagnosis of celiac disease.

Analysis

Perform a panoramic analysis of this field.

view full example data

AI Agents Built for Biopharma Breakthroughs

Accelerate discovery. Empower decisions. Transform outcomes.

Get started for free today!

Accelerate Strategic R&D decision making with Synapse, PatSnap’s AI-powered Connected Innovation Intelligence Platform Built for Life Sciences Professionals.

Start your data trial now!

Synapse data is also accessible to external entities via APIs or data packages. Empower better decisions with the latest in pharmaceutical intelligence.

Bio

Bio Sequences Search & Analysis

Chemical

Chemical Structures Search & Analysis

Immunoproliferative Small Intestinal Disease

Basic Info

Related

Analysis