A concomitant case of pathologically proven IgG4-related disease and ANCA-associated vasculitis: case report. Issue 1 (2nd January 2018)

• Record Type: Journal Article
• Authors: Nakayama, Yoichi; Yoshifuji, Hajime; Mori, Masato; Kuramoto, Nobuo; Murakami, Kosaku; Nakashima, Ran; Imura, Yoshitaka; Ohmura, Koichiro; Handa, Tomohiro; Miyagawa-Hayashino, Aya; Yokoi, Hideki; Mimori, Tsuneyo
• Is Part Of: Modern rheumatology case reports. Volume 2:Issue 1(2018)
• Page Start: 84
• Page End: 91
• Publication Date: 2018-01-02
• Subjects: IgG4-related disease -- IgG4-related lung disease -- ANCA-associated vasculitis -- microscopic polyangiitis -- glomerulonephritis; Rheumatology -- Periodicals; 616.723005
• DOI: 10.1080/24725625.2017.1408551
• Languages: English
• ISSNs: 2472-5625

Abstract

Abstract: A 63-year-old male visited our hospital after bilateral apical lung masses were detected on medical check-up chest X-ray. Histopathology of the resected mass revealed storiform fibrosis with an increased number of immunoglobulin G4 (IgG4)-positive plasma cells, compatible with IgG4-related disease (IgG4-RD). The patient was subsequently followed up without treatment for 3 years. Later, at age 66, he revisited our hospital because of scleritis, proteinuria, and myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) positivity (179 U/ml). A renal biopsy revealed pauci-immune necrotising crescentic glomerulonephritis. After high-dose prednisolone (PSL) was started, the patient's scleritis subsided and his MPO-ANCA level and proteinuria were decreased. Subclass-based indirect immunofluorescence revealed that the patient's serum was positive for IgG1-type ANCA but negative for IgG4-type ANCA. Including the present case, there have been nine reported cases involving both biopsy-proven IgG4-RD and ANCA-associated vasculitis (AAV). In all these concomitant cases of IgG4-RD and AAV, PSL and immunosuppressants were used to control AAV. Deliberate care should be taken to distinguish between IgG4-RD and AAV at diagnosis as they have overlapping features and can, though very rarely, occur concomitantly.