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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

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[IgG4-related sclerosing disease].

I A Kazantsev, S V Lishchuk

Arkhiv Patologii

|August 23, 2011

Summary

Immunoglobulin G4-related sclerosing disease (IgG4-RSD) is a systemic condition involving IgG4-positive plasma cell infiltration. Early consideration of IgG4-RSD aids in differential diagnosis, preventing unnecessary surgeries.

Area of Science:

Immunology
Pathology
Rheumatology

Context:

IgG4-related sclerosing disease (IgG4-RSD) is a multi-organ systemic disorder.
Characterized by IgG4-positive plasma cells and T lymphocytes infiltrating various organs.
Pathological hallmarks include tissue fibrosis and obliterative phlebitis.

Purpose:

To describe the clinical and pathological features of IgG4-related sclerosing disease.
To highlight the diverse organ manifestations and elevated serum IgG4 levels.
To emphasize the importance of considering IgG4-RSD in differential diagnosis.

Summary:

IgG4-RSD presents with a wide range of clinical manifestations, including autoimmune pancreatitis, sclerosing cholangitis, and interstitial nephritis.
Significantly elevated serum IgG4 levels are a key diagnostic indicator.

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The condition predominantly affects elderly men and shows a positive response to steroid therapy.

Impact:

Accurate diagnosis of IgG4-RSD can prevent misdiagnosis and unnecessary surgical interventions.
Understanding IgG4-RSD is crucial for effective patient management and treatment strategies.
Highlights the need for differential diagnosis to include IgG4-RSD when suspecting malignancy.