IgG4-related disease in the nervous system

Area of Science:

• Neurology
• Immunology
• Rheumatology

Background:

• Immunoglobulin G4-related disease (IgG4-RD) is a recently identified multisystemic condition.
• While diverse, IgG4-RD exhibits recognizable disease patterns.
• Neurological involvement is uncommon, often presenting with orbital, pituitary, or dural inflammation.

Purpose of the Study:

• To review the neurological manifestations of IgG4-RD.
• To emphasize the significance of early clinical suspicion for prompt diagnosis and management.
• To present a treatment algorithm for neurological IgG4-RD.

Main Methods:

• Literature review of IgG4-related disease with neurological involvement.
• Analysis of distinct patterns of neurological presentation.
• Development of a management strategy and treatment algorithm.

Main Results:

• Neurological manifestations of IgG4-RD, though rare, include specific patterns like orbital disease, hypophysitis, and pachymeningitis.
• IgG4-RD affecting the nervous system typically shows a high response to treatment.
• Prompt diagnosis and management are crucial to prevent irreversible neurological damage.

Conclusions:

• A high index of clinical suspicion is vital for diagnosing IgG4-RD affecting the nervous system.
• Timely intervention can prevent significant neurological dysfunction and tissue damage.
• The proposed treatment algorithm offers guidance for managing neurological IgG4-RD.