Immunoproliferative Small Intestinal Disease (IPSID)

  • – Generally occurs in 2nd and 3rd decade
  • – Associated with low socioeconomic status
  • – High prevalence in SIBO patients
  • – Early lesions are responsive to antibiotic therapy
  • – Associated with Campylobacter jejuni
  • – Associated with production of unusual heavy chain IgA, called a heavy chain which is secreted by plasma cells and is detected in various body fluids
  • – Analogous to Hp associated gastric MALT lymphoma
  •    – B cells of small intestine is stimulated by jejuni to proliferate

 

  • Gross Pathology of IPSID:
  •   – Lesions are generally confined to the proximal small intestine
  •   – Mesenteric lymph nodes are enlarged in early lesions, with preserved architecture, as the disease progress lymph nodes may appear dysplastic
  •   – Some patients have thickening of mucosal folds only and others have a generalized thickening of the bowel wall
  •    – Discrete mass
  •    – Nodule
  •    – Polypoid lesion
  • Clinical Features:
  • Chronic Diarrhea : Initially intermittent, later voluminous and foul smelling as malabsorption develops
  • – Colicky abdominal pain
  • – Anorexia
  • – Significant weight loss
  • Physical findings of IPSID:
  •      – Features of malnutrition
  •      – Digital clubbing
  •       – Peripheral edema
  •       – Ascites
  •       – Hepatosplenomegaly
  •       – Abdominal mass
  •       – Peripheral lymphadenopathy

 

  • Investigations:

 Endoscopic findings of IPSID

  • – Thickened mucosal folds
  • – Nodule
  • – Ulcer
  • – Evidence of submucosal infiltration
  • – Intestine is immobile, non distensible due to submucosal involvement

Barium study of IPSID

  • – Diffuse dilatation of the duodenum, jejunum and proximal ileum
  • – Thickened mucosal fold

CBC

  • – Anemia (due to B vitamin deficiency)
  • – Low circulating lymphocyte
  • – High ESR
  1. Immunoglobulins

–  Low /undetectable IgA

IgG/IgA low or high

             Stool examination

  • Giardia lamblia infestation

** C.jejuni on intestinal biopsy PCR

 

Small Intestine Biopsy

  • – Dense mucosal/ submucosal infiltration of Lymphoplasmacytic cell/ Atypical plasma cell that extends continuously throughout the length of small bowel
  • – Infiltrates broadens villi, Shorten and separate crypt

Serum Protein Electrophoresis:

  • – A heavy chain
  • Salem pathological staging of IPSID:

Treatment of IPSID:

  • Early disease (Salem stage 0):
  •       – Tetracycline alone or for 6 months
  •       – Metronidazole + Ampicillin
  • Chemotherapy:
  •       – For advanced disease
  •      – Who do not significantly achieve complete remission by 12 months

 

 

 

 


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