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Non-IgG4-related Multifocal Fibrosclerosis
Yosuke Kunishita1, Ryusuke Yoshimi, Mitsuhiro Takeno
1Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine, Japan.
Internal Medicine (Tokyo, Japan)
|September 2, 2016
Summary
Multifocal fibrosclerosis (MFS) can mimic IgG4-related disease (IgG4-RD) histopathologically. This case highlights non-IgG4-related MFS with features resembling IgG4-RD, despite normal IgG4 levels.
Area of Science:
- Rheumatology
- Pathology
Background:
- Multifocal fibrosclerosis (MFS) is a systemic connective tissue inflammation often associated with Immunoglobulin G4 (IgG4).
- MFS is frequently considered synonymous with IgG4-related disease (IgG4-RD).
- Exceptional cases of MFS mimicking IgG4-RD histopathologically, despite normal IgG4 levels, are rarely documented.
Observation:
- A case of non-IgG4-related MFS presented with periaortitis and parotiditis.
- Histological examination revealed findings consistent with IgG4-related disease (IgG4-RD).
Findings:
- Serum and tissue IgG4 levels were not elevated in this patient.
- The histological features mimicked IgG4-RD, but the condition was classified as non-IgG4-related MFS.
Implications:
- This case expands the understanding of MFS presentations.
- It underscores the importance of considering non-IgG4-related conditions that histopathologically resemble IgG4-RD.
- Highlights the need for careful diagnostic evaluation beyond serum IgG4 levels in suspected MFS cases.
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