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Chronic Bowel Disorders: Introduction

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Inflammatory Bowel Disease III: Crohn's Disease

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Related Experiment Videos

[From 'malignancy' to IgG4-related systemic disease].

Andrea B Kramer1, Helma R Lebbink, Marcory C R F van Dijk

  • 1Universitair Medisch Centrum Groningen, Afd. Nefrologie, Groningen, the Netherlands. a.b.kramer@int.umcg.nl

Nederlands Tijdschrift Voor Geneeskunde
|November 24, 2011
PubMed
Summary

IgG4-related systemic disease presents with diverse symptoms, often mimicking cancer. Early diagnosis via IgG4 levels and plasma cell infiltration ensures effective glucocorticoid treatment and good prognosis.

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Area of Science:

  • Immunology
  • Rheumatology
  • Pathology

Background:

  • IgG4-related systemic disease (IgG4-RD) is an emerging condition characterized by multi-organ involvement.
  • Common manifestations include retroperitoneal fibrosis and autoimmune pancreatitis.
  • IgG4-RD can present with symptoms mimicking malignancy, leading to diagnostic challenges.

Observation:

  • Three elderly patients (71, 83, 70 years) presented with malaise, fatigue, and swellings suggestive of malignancy.
  • Histopathology revealed plasma cell infiltration in the swellings.
  • Elevated serum IgG4 levels confirmed the diagnosis.

Findings:

  • Diagnosis of IgG4-RD is supported by elevated serum protein and IgG4 levels.
  • Presence of IgG4-positive plasma cell infiltrates is a key diagnostic marker.
  • All presented patients showed a positive response to glucocorticoid therapy.

Implications:

  • IgG4-RD should be considered in the differential diagnosis for patients with malaise, elevated protein levels, and multi-organ involvement.
  • Prompt diagnosis can prevent unnecessary surgical interventions for suspected malignancies.
  • Effective treatment with glucocorticoids leads to favorable patient outcomes and prognosis.