EP483 - IGg4-related Disease in the Rectum, A Rare Entity.

ePoster Presenter(s)

• 

  Maria P. Gomez, R.M1010233863

  Medical Doctor
  Clinica los Nogales
  Bogotá D.C, Cundinamarca, Colombia

  All of the relevant financial relationships listed below have been mitigated.
  This individual has no financial relationships with ineligible companies.

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Author(s)

• MG

  Mauricio Gonzalez, n/a

  Coloproctólogo
  Hospital San José Sociedad Bogotá de Cirugía
  BOGOTA, Distrito Capital de Bogota, Colombia

  All of the relevant financial relationships listed below have been mitigated.
  This individual has no financial relationships with ineligible companies.
• LM

  Luis Martinez, .

  Coloprotologo
  Clinica los Nogales
  Bogota, Cundinamarca, Colombia

  Disclosure information not submitted.

Senior Author(s)

• NN

  Nelson Niño, n/a

  Coloproctologo
  FUCS
  Bogota, Distrito Capital de Bogota, Colombia

  All of the relevant financial relationships listed below have been mitigated.
  This individual has no financial relationships with ineligible companies.

Author(s)

• DG

  Daniel Gonzalez, ED.D, MED

  Sr Instructional Designer
  The University of Texas MD Anderson Cancer Center
  Houston, Texas, United States

  Disclosure information not submitted.
• AO

  Andrés Olaya, n/a

  Fellow Coloproctología
  FUCS
  BOGOTA, Distrito Capital de Bogota, Colombia

  Disclosure information not submitted.
• LT

  Luis Torres, .

  Fellowship Coloproctology
  Clinica los Nogales
  Bogota, Cundinamarca, Colombia

  Disclosure information not submitted.
• DM

  David Mayo, n/a

  Fellow coloproctología
  FUCS
  BOGOTA, Distrito Capital de Bogota, Colombia

  Disclosure information not submitted.

Purpose/Background: IgG4-related disease was described as a different and specific condition at the beginning of the century, having scarce evidence on its etiology and pathophysiology. It is underdiagnosed mainly because of this, being frequently confused with cancer, infection or other autoimmune diseases. Little is known about its epidemiology, with some reporting higher prevalence in male patients. This diagnosis was achieved with the correlation of clinical findings and histopathological results, as well as in the not-so-frequent elevated serum IgG4.

Methods/Interventions: An advanced literature search was carried out in databases, PubMed and Cochrane with articles published in the last 20 years with the following MeSH terms “IgG4 related disease” “rectum” “lung” “pneumonia” “lymphoplasmacytic infiltrate”. We present the case of a 56-year-old patient with abdominal pain associated with proctalgia with a finding on pelvis MRI with striation of the perirectal fat, neovascularization and the presence of masses that compromised the perirectal and presacral space. After a non-definitive EUS-guided biopsy, a laparoscopic mesorectal excision was performed, with histopathological evidence of accumulations of IGg4 cells greater than 40 x field which confirms the diagnosis of IgG4-related disease.

Results/Outcomes: The patient required surgical management with a laparoscopic mesorectal excision of affected tissue. Intraoperative findings described a marked thickening of the visceral and parietal peritoneum, edema and 2-5 cm lymphadenopathy in the mesorectum. Postoperative complications significantly extended the ICU stay mainly due to pulmonary edema ultimately attributed to possible infiltration by IgG4. After biopsy confirmation of the disease steroid and immunomodulator use reverted clinical deterioration allowing for extubation and eventually out-patient management

Conclusion/Discussion: Immunoglobulin G4-related disease is a fibroinflammatory process which can affect multiple organs, causing tumor-like swelling regardless of the plasma level of IgG4. Apart from multiorgan involvement, clinical suspicion is key, especially when other diagnoses have been ruled out. The most accurate test of ER-IgG4 is based on a thorough clinical history, adequate examination, selected laboratory tests and previously described typical findings in histopathology. When confirming the diagnosis the use of corticosteroids usually allows for a manageable disease.

Figure 1. Gross feature of IgG4 related disease of rectum
photograph of biopsy specimen

Figure 2. findings of imaging study of IgG4 related disease of rectum
T1- weighted MRI, mass involving anterior wall of rectum