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  • Study identifies distinctive pathophysiology of ocular adnexal IgG4-related lymphoplasmacytic infiltrative disorder

    By Kelvin Kam-lung Chong, MBChB, MRCS, FCOphth, FHKAM
    Oculoplastics/Orbit

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    Given the recent surge in interest in using IgG4 positivity to evaluate orbital disorders, the authors sought to determine the clinicopathological characteristics of patients with ocular adnexal infiltration of IgG4-positive plasma cells.

    They prospectively classified 24 patients with ocular adnexal lymphoplasmacytic infiltrative lesions, including those with sclerosing inflammation and reactive lymphoid hyperplasia, based on their IgG4-related status - IgG4 related, indeterminate or unrelated - using two criteria.

    Patients with an elevated serum level of IgG4 of 135 mg/dL or greater and an IgG4:IgG ratio of infiltrating plasma cells of 30 percent or greater were classified as IgG4-related; 10 patients met this criteria. Five patients who met one but not both criteria were considered "indeterminate," and nine patients who did not meet either criteria were classified as IgG4-unrelated.

    Patients in the IgG4-related group more frequently had bilateral ocular adnexal lesions (P = 0.02), allergic diseases (P = 0.01) and elevated IgE serum levels (P = 0.01). Additionally, three patients in the IgG4-related group had polyclonal hypergammaglobulinemia, six had systemic lymphadenopathy or salivary gland enlargement and one developed autoimmune pancreatitis. Patients in the IgG4-unrelated group did not have these abnormalities.

    The authors conclude that ocular adnexal IgG4-related lymphoplasmacytic infiltrative disorder has different patterns of tissue involvement and systemic disease associations and possibly different prognoses than IgG4-unrelated lesions.

    Whether treatment response to irradiation or recurrence upon steroid cessation and future transformation to ocular adnexal lymphoma occur more frequently in the IgG4-related group still needs to be verified with larger numbers of patients and longer follow-up. The presence of IgG4-related features in serum and biopsy may reflect systemic immunological imbalance or activation, the underlying pathophysiology of which is still under investigation.