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An In Vitro Model for the Study of Cellular Pathophysiology in Globoid Cell Leukodystrophy
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IgG4-related disease.

Elisabeth Palazzo1, Clémence Palazzo2, Maxime Palazzo3

  • 1Service de rhumatologie, hôpital Bichat, Paris, France.

Joint Bone Spine
|July 16, 2013
PubMed
Summary
This summary is machine-generated.

Immunoglobulin G4-related disease (IgG4-RD) is a rare condition affecting multiple organs, often presenting as pseudotumors. While glucocorticoids are effective, relapses may necessitate immunomodulating agents like rituximab.

Keywords:
ArthritisAutoimmune pancreatitisIgG4-related diseaseRituximab

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Area of Science:

  • Rheumatology
  • Immunology
  • Pathology

Background:

  • IgG4-related disease (IgG4-RD) is an emerging clinicopathological entity previously described under various names based on affected organs.
  • It can affect virtually any organ system, commonly involving the pancreas, bile ducts, salivary glands, and retroperitoneum.
  • The condition is rare, predominantly affecting men over 50, with limited incidence data in Western countries.

Purpose of the Study:

  • To provide a comprehensive overview of IgG4-related disease.
  • To discuss its varied clinical presentations, pathological hallmarks, and diagnostic considerations.
  • To outline current therapeutic strategies and emerging treatments.

Main Methods:

  • Review of existing literature on IgG4-related disease.
  • Analysis of clinical, pathological, and immunological features.
  • Evaluation of treatment outcomes and response to therapies.

Main Results:

  • IgG4-RD presents heterogeneously, often with organ enlargement mimicking tumors.
  • Pathological findings include IgG4-positive plasma cell infiltration, storiform fibrosis, and obliterative endarteritis.
  • Elevated serum IgG4 levels (often >1.35 g/L) are frequently observed, though not diagnostic alone.
  • Glucocorticoids are the mainstay of initial treatment, but relapses are common.

Conclusions:

  • IgG4-related disease is a distinct fibroinflammatory condition, not an autoimmune disease.
  • Early diagnosis and prompt treatment with glucocorticoids can improve outcomes.
  • Immunomodulatory agents, including rituximab, are valuable for managing relapsing or refractory cases.