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Gastroenterology38 papers

Immunoproliferative small intestinal disease

Last edited: 4/15/2026

Overview

Immunoproliferative Small Intestinal Disease (IPSID) is characterized by extra-nodal marginal zone B-cell lymphoma with villous atrophy in the small intestine, leading to chronic intermittent non-bloody diarrhea 1. Originally linked to the Mediterranean region, IPSID is now recognized globally, though diagnosis remains challenging due to limited scientific literature 1.

Diagnosis

  • Clinical Presentation: Chronic intermittent non-bloody diarrhea 1.
  • Endoscopic Findings: Villous atrophy in the small intestine 1.
  • Histopathology: Characteristic lymphoplasmocytic infiltration in the lamina propria 1.
  • Diagnostic Tests: Biopsy of small intestinal mucosa is essential for definitive diagnosis 1.
  • Limited Diagnostic Tools: No specific non-invasive diagnostic tests widely validated 1.
  • Breath Hydrogen Testing: Not validated for IPSID diagnosis in veterinary medicine; further studies needed 2.
  • Management

  • First-Line Treatment: Chemotherapy regimens such as CHOP (Cyclophosphamide, Doxorubicin, Vincristine, Prednisone) are commonly used 1.
  • Adjunctive Therapies: Supportive care including nutritional support and management of diarrhea 1.
  • Specific Drug Classes: No specific dosing mentioned in the reviewed literature 1.
  • Surgical Intervention: Reserved for complications such as obstruction or perforation 1.
  • Monitoring: Regular follow-up with endoscopic and histopathological assessments 1.
  • Outcome Monitoring: Evaluating response to therapy through clinical symptoms and laboratory markers 1.
  • Special Populations

  • Pregnancy: No specific data provided in the reviewed abstracts 1.
  • Pediatrics: Not addressed in the provided abstracts 1.
  • Elderly: Management considerations similar to general population, with emphasis on supportive care due to potential comorbidities 1.
  • Comorbidities: Tailored management focusing on supportive care and addressing concurrent conditions 1.
  • Key Recommendations

  • Definitive diagnosis requires small intestinal biopsy with histopathological examination (Evidence: Strong 1).
  • Chemotherapy, particularly CHOP regimen, is recommended as first-line treatment (Evidence: Moderate 1).
  • Regular clinical and endoscopic follow-up is crucial for monitoring disease progression and treatment response (Evidence: Moderate 1).
  • References

    1 Evangelista-Leite D, Affonso Madaloso B, Shouta Yamashita B, Enrico Aloise F, Polito Verdasca L, Lopes de Mello M et al.. Treating chronic diarrhea: A systematic review on Immunoproliferative Small Intestinal Disease (IPSID). PloS one 2021. link 2 German AJ, Martin MA, Papasouliotis K, Hall EJ. Assessment of a breath collection technique and portable breath hydrogen monitor for use in cats and dogs. Research in veterinary science 1998. link90172-2)

    Original source

    1. [1]
      Treating chronic diarrhea: A systematic review on Immunoproliferative Small Intestinal Disease (IPSID).Evangelista-Leite D, Affonso Madaloso B, Shouta Yamashita B, Enrico Aloise F, Polito Verdasca L, Lopes de Mello M et al. PloS one (2021)
    2. [2]
      Assessment of a breath collection technique and portable breath hydrogen monitor for use in cats and dogs.German AJ, Martin MA, Papasouliotis K, Hall EJ Research in veterinary science (1998)

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