Azathioprine and prednisone combination treatment for adult periocular and orbital xanthogranulomatous disease

Abstract

ABSTRACT.Purpose: To report the authors' experience with azathioprine and prednisone combination for adult periocular and orbital xanthogranulomatous disease. Methods: We identified 13 adults with histology-proven periocular or orbital xanthogranuloma in two tertiary referral orbital centres from 1984 to 2008. Patient records were reviewed and data collected on orbital localization, immune dysfunction, applied treatment and outcome. Results: Five patients with periocular or orbital xanthogranulomatous disease were fully treated with prednisone and azathioprine combination, which resulted in stabilization in two and regression in three. Two other patients had to discontinue azathioprine because of side-effects. Of the non-fully treated prednisone ⁄ azathioprine patients, four out of eight progressed. Conclusion: In adult periocular and orbital xanthogranuloma, combined treatment with prednisone and azathioprine yields adequate immunosuppression, often for a prolonged period of time.

Metastatic xanthogranulomatous pyelonephritis managed with immunosuppression

“…10 A case series of 13 patients with periocular and orbital xanthogranuloma demonstrated stabilization or regression of disease in five patients. 11 In this case, immunosuppression was associated with good clinical and biochemical effect.…”

“…Other forms of xanthogranulomatous disease such as juvenile xanthogranuloma and adult xanthogranuloma are a form of non‐Langerhans cell histiocytosis, most commonly involving the skin and mucosa 10 . A case series of 13 patients with periocular and orbital xanthogranuloma demonstrated stabilization or regression of disease in five patients 11 . In this case, immunosuppression was associated with good clinical and biochemical effect.…”

Atypical Presentation of Low-Grade Small Lymphocytic Lymphoma of the Lacrimal Sac

“…The treatment modalities for adult-onset asthma and periocular xanthogranuloma range from debulking surgery 7 to high-dose systemic corticosteroids in combination with low-dose radiotherapy 8 or immunosuppressive agents such as cyclophosphamide, 9 methotrexate, 10,11 or azathioprine. 12 There is no consensus on the best choice of immunosuppressive drug. Adult-onset asthma and periocular xanthogranuloma and necrobiotic xanthogranuloma 13 are associated with immune dysfunction ranging from asthma and reactive lymphadenopathy to lymphoproliferative disorders including paraproteinemia and multiple myeloma.…”

Adult Orbital Xanthogranulomas: Clinical Features and Management

“…Chlorambucil provided initial improvement but this could not be sustained (17). After treating 6 patients with AOX and 3 with AAPOX, Bijlsma et al (11) concluded that corticosteroids and azathioprine was the most effective combination. Another study reported satisfactory results with oral corticosteroids, methotrexate, and azathioprine (9).…”

“…The treatment of adult xanthogranulomatous diseases continues to be challenging and often frustrating. Several modalities including systemic and intralesional corticosteroids, cytotoxic and cytostatic agents, immunomodulators, and radiotherapy and their various combinations have been utilized with contrasting outcomes (2, 3, 8–9–10–11–12). The principal objective of this study of a case series of patients with AAPOX and AOX was to assess the efficacy of the management scheme that we consistently implemented to these patients for the past 14 years.…”