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  4. Case report: Clinical highlights and radiological classification of IgG4-related spinal pachymeningitis: A rare case series and updated review of the literature

Case report: Clinical highlights and radiological classification of IgG4-related spinal pachymeningitis: A rare case series and updated review of the literature

Frontiers in Oncology, 2023 · DOI: 10.3389/fonc.2022.1035056 · Published: January 10, 2023

RheumatologyImmunologyNeurology

Simple Explanation

This study focuses on IgG4-related spinal pachymeningitis (IgG4-RSP), a rare condition where the spinal cord's protective layers thicken due to an immune response. The research presents three new cases and reviews existing literature to understand the disease's clinical features, radiological manifestations, treatment approaches, and patient outcomes. The paper aims to improve the diagnosis and treatment of IgG4-RSP by analyzing radiological findings and lesion locations.

Study Duration
Not specified
Participants
45 patients (3 case reports from the center and 42 cases from literature review)
Evidence Level
Case Report and Literature Review

Key Findings

  • 1
    Progressive worsening of neurological impairment was observed in nearly half of the patients (48.9%).
  • 2
    The thoracic and cervical spine were the most frequently involved segments.
  • 3
    Early surgical decompression, corticosteroid treatment, and steroid-sparing agents offered significant therapeutic advantages.

Research Summary

The study reports three cases of IgG4-RSP and reviews 45 cases total, highlighting the disease's clinical presentations, radiological manifestations, treatment, and prognosis. Key findings include the progressive nature of neurological symptoms in many patients and the frequent involvement of the thoracic and cervical spine. The research suggests that early surgical intervention combined with immunosuppressive therapy and long-term maintenance with corticosteroids and steroid-sparing agents improves patient outcomes.

Practical Implications

Early Diagnosis

Recognizing progressive neurological symptoms and characteristic MRI features can lead to earlier diagnosis of IgG4-RSP.

Treatment Strategy

Combining surgical decompression with immunosuppressive therapy improves outcomes.

Long-term Management

Long-term maintenance therapy with corticosteroids and steroid-sparing agents can reduce recurrence.

Study Limitations

  • 1
    Subjectivity in literature review.
  • 2
    Lack of quality assessment of included studies.
  • 3
    Small sample size limited statistical analysis.

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