Anti-neutrophil cytoplasmic antibody-associated vasculitis overlap immunoglobulin G4-related diseases: a 4 case series and review of literature / 中华风湿病学杂志

ABSTRACT

Objective:To summarize the characteristics and treatment outcomes of immunoglobulin G4-related disease (IgG4-RD) overlapped with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV).Methods:The clinical data of four patients with AAV overlaped with IgG4-RD from Zhongshan Hospital of Fudan University from August 2018 to July 2019 were collected and the related literature were reviewed.Results:Four patients were included, in which two were diagnosed with IgG-RD and granulomatosis with polyangiitis (GPA), one was probable IgG4-RD and microscopic polyangiitis (MPA), and one was probable IgG4-RD and GPA. All patients were female, with an average age of (42±12) (26-56) years, and disease duration was (7±4) (4-13) months. The manifestations were ocular inflammatory pseudotumor, sinusitis, otitis media, mastoiditis, parotitis, meningitis, lung and kidney involvement. After treatment with glucocorticoid and immunosuppressants (including cyclophosphamide, methotrexate, azathioprine, leflunomide), 2 patients failed to achieve remission, and 2 patients relapsed 8-15 months after treatment. One patient was treated withglucocorticoid pulse therapy combined with rituximab and one was treated with glucocorticoid combined with methotrexate and rituximab, and the patient was relieved.Conclusion:AAV and IgG4-RD may be a new overlap syndrome. Hypertrophic meningitis, orbital mass, chronic periaortic inflammation and interstitial glomerulonephritis are reported in the literature. The pathological changes of orbit, nasopharynx, parotid gland and lung are common. Glucocorticoids and immunosuppressive agents have poor treatment response, which indicates that AAV is refractory when combined with elevated IgG4. The induced remission rate is low, and easy to relapse. Glucocorticoid pulse therapy and rituximab treatment are effective.