Background/Purpose: IgG4-related disease (IgG4-RD) is a recently recognized disease entity that is characterized by tumefactive and hyperplastic lesions in various organs including the lacrimal and salivary glands, pancreas and kidneys. Because patients with IgG4-RD have elevated serum IgG4 levels and characteristic histopathological features including dense infiltration of IgG4-positive plasma cells and storiform fibrosis, such lesions are assumed to have a common etiology and pathogenesis. Organ dysfunction is rarely severe in patients with IgG4-RD at diagnosis and responsiveness to corticosteroid therapy is frequently positive. Although the prognosis of IgG4-RD seems favorable, no long-term follow-up study has been reported. Data regarding the prognosis of IgG4-RD is essential when considering the introduction of intensive immunosuppressive therapy. We describe the clinical characteristics and prognosis of patients who have had IgG4-RD for ≥10 years.

Methods: This retrospective study at Sapporo Medical University Hospital analyzed clinical data at onset, involved organs during the clinical course and prognosis of 15 patients (5 men, 10 women) who had lived with IgG4-RD for ≥10 years. They were basically diagnosed according to the diagnostic criteria for IgG4-related Mikulicz’s disease determined by the Japanese Society for Sjögren’s syndrome.

Results: The average age at onset was 59 years and the average disease duration was 158 months. The lacrimal and salivary glands were the most frequently involved organs at onset in 12 of the 15 patients. Other initially involved organs included the mammary glands, pancreas and optic nerve. Only the lacrimal and salivary glands were affected in 6 patients during the clinical course (Mikulicz type). The others had multiple lesions (systemic type) of the retroperitoneum (n = 4), pancreas (n = 3), kidney (n = 2), lung (n = 2), liver (n = 1) and pituitary gland (n = 1). However, other lesions developed within 5 years in most patients with the systemic type and autoimmune pancreatitis developed in 1 patient 19 years after onset. The average levels of serum IgG4 at diagnosis in the Mikulicz and systemic types were 703 and 1,111 mg/dL, respectively. All patients initially responded well to corticosteroid therapy. Two patients required additional immunosuppressants due to recurrence during corticosteroid tapering. None of the patients died, progressed to end-stage organ failure or developed malignant lymphoma during the clinical course.

Conclusion: Patients with IgG4-RD presenting with lacrimal and salivary lesions at onset often developed other involved lesions within 5 years. However, only the lacrimal and salivary glands remained affected in a third of the patients over a period of about 10 years. Because the prognosis in terms of both life and function for patients with IgG4-RD is favorable when supported by corticosteroid therapy, careful consideration is required before introducing intensive immunosuppressive therapy.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/long-term-follow-up-of-igg4-related-diseases-presenting-with-lacrimal-and-salivary-gland-involvement/