Periostin: a promising biomarker in nonspecific orbital inflammation and orbit-involving IgG4 disease

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PURPOSE: To demonstrate crisp, specific immunohistochemistry (IHC) for periostin and document its utility as a biomarker for nonspecific orbital inflammation (NSOI) and immunoglobulin G4-related orbital disease (IgG4-RD). METHODS: A multi-institutional retrospective study of both NSOI and IgG4-RD patients. Demographics, clinical disease location, and severity were recorded. Routine histologic sections of affected NSOI and IgG4-RD tissue and fibrocollagenous control tissue were interrogated with an anti-periostin antibody. RESULTS: Ten NSOI patients had orbital manifestations of variable severity involving the lacrimal gland, extraocular muscle, and diffuse or focal orbital tissue. 12 IgG4-RD disease patients had involvement of the lacrimal gland, multifocal orbital disease, or extra-orbital extension. Periostin IHC showed strong, diffuse positivity restricted to pathological fibrotic zones, sparing inflammatory and epithelial elements. This was observed in both the NSOI and IgG4 cases. CONCLUSION: Periostin is highly expressed in zones of pathologic fibrosis, indicating its potential role in NSOI and IgG4 disease pathogenesis and as a disease biomarker.

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