Abstract:

Objective To explore the clinical features and treatment of immunoglobulin G4 (IgG4)-related diseases characterized by increased immunoglobulin E (IgE) level and eosinophilic count with multiple lymphadenopathy. Methods The clinical data, diagnosis and treatment process of a patient with IgG4-related disease characterized by increased IgE level and eosinophilic count with multiple lymphadenopathy were retrospective analyzed, and related literatures were reviewed. Results A 24-year-old male patient was admitted with the enlargement of bilateral inguinal lymph nodes for more than 2 years. The clinical manifestations, IgG4 test and pathology results suggested the diagnosis of IgG4-related disease. The therapeutic regimen was 20 mg prednisone orally once a day and 5 mg Tofacitinib citrate tablets orally twice a day. The dosage was adjusted according to the blood test and ultrasound results, and the symptoms of the patient were significantly improved at the final follow-up visit. Conclusion IgG4-related diseases are often characterized by significantly elevated serum IgG4 levels and lump-like lesions. Increased IgE level and eosinophil count are rarely observed in cases of IgG4-related diseases, although their important roles have been recently identified. Glucocorticoids are the first-line drugs, and targeted drugs like Tofacitinib may have potential therapeutic effects on IgG4-related diseases, which expand the new idea of the treatment.

Key words: immunoglobulin G4-related disease, immunoglobulin E, eosinophilia, lymph nodes