Background IgG-related disease (IgG4RD) is a systemic lymphoproliferative disorder characterized by tissue infiltration with IgG4-secreting plasma cells, occasionally leading to organ dysfunction. The gold standard of IgG4RD diagnosis is based on the pathological finding, but in practical, tissue sampling could be often unavailable.
Objectives To determine the state of diagnosis and therapy for IgG4RD in our hospital.
Methods Since April 2011, we have prospectively followed up patients with IgG4RD who were diagnosed mostly by the physical and image findings highly specific for IgG4RD and serum IgG4 levels of >135mg/dL.
Results At present, 18 patients enrolled in the study. Their mean age at presentation was 66.7 years (range: 39 – 85 years) and male : female ratio was 12 : 6. Organs involvement at diagnosis were salivary glands in 8 cases, lymph nodes in 5 cases, thyroid glands in 4 cases, pancreas or retroperitoneum in 3 cases, aorta, kidneys, salivary glands, bile tracts in 2 cases, and lungs or joints in one case. 8 patients have been treated with glucocorticoids (GC) therapy for their retroperitoneal lesions (3 cases), renal dysfunction (3 cases), pancreatitis, periaortitis, and others, and in 6 of them, azathioprine (AZA) was added later because of insufficient GC efficacy and/or its adverse effects. They responded well to the treatment, and 8 other untreated patients have not yet developed any organ dysfunction.
Conclusions These results indicate that IgG4RD principally shows a benign clinical course and GC therapy thus should be indicated for patients with progression or risk of organ damage. In our experience, AZA may be useful for patients GC-resistant or intolerant patients.
Disclosure of Interest None Declared
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