A case of proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA) positive/IgG4-related lung disease

Background Antineutrophil cytoplasmic antibody (ANCA)‐associated vasculitis (AAV) and immunoglobulin G4‐related disease (IgG4‐RD) have some common features. The co‐occurrence/concurrence of AAV and IgG4‐RD was recently published by the collaborative European Vasculitis Study Group. First, we aimed to investigate ANCA positivity of our IgG4‐RD cohort. Second, a literature review of co‐occurrence/concurrence of AAV and IgG4‐RD was done. Methods Data of 62 patients with IgG4‐RD in Hacettepe Vasculitis Center Database were used. Patient dataset was designed to include demographic data, clinical characteristics, imaging and IgG4‐RD, AAV and ANCA test results. At the next step, we performed a systematic literature review in PUBMED database covering the time period from 1976 until April 2018. Relevant publications were searched using these MeSH terms ‘‘IgG4‐related disease and Anti‐Neutrophil Cytoplasmic Antibody‐Associated Vasculitis’’, “IgG4‐related disease and Eosinophilic Granulomatosis with Polyangiitis”, “IgG4‐related disease and Microscopic Polyangiitis” and “IgG4‐related disease and Granulomatosis with Polyangiitis”. Results Three (10.3%) of 29 patients had low titer ANCA positivity. These three patients didn't have any findings of vasculitis and no granuloma was seen in biopsy. In the literature review, we found 17 cases had features of both IgG4‐RD and AAV. These cases were re‐evaluated according to the Comprehensive Diagnostic Criteria for IgG4‐RD. ANCA were positive in 15 of 17 patients (88%). Conclusion None of our IgG4‐RD patients overlapped with AAV. Only two patients in the literature review seemed to be fully compatible with both diseases. Even though AAV and IgG4‐RD share similar clinical features, we think this might be a co‐occurrence instead of a histopathological link.

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IgG4:IgG RNA ratio differentiates active disease from remission in granulomatosis with polyangiitis: a new disease activity marker? A cross-sectional and longitudinal study

Al-Soudi¹,

Doorenspleet²,

Esveldt³

et al. 2019

Arthritis Res Ther

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ObjectivesAn important limitation in granulomatosis with polyangiitis (GPA) is the lack of disease activity markers. Immunoglobulin G4-positive (IgG4+) B cells and plasma cells are implicated in the pathogenesis of GPA. We hypothesized that the presence of these cells in peripheral blood could serve as disease activity parameter in GPA.MethodsWe included 35 proteinase 3-antineutrophil cytoplasmic antibodies-positive patients with GPA in a cross-sectional study. Active disease was defined as Birmingham Vasculitis Activity Score (BVAS) ≥ 3 (n = 15), remission as BVAS of 0 (n = 17), and low disease activity (LDA) as BVAS of 1–2 and clinical remission (n = 3). Healthy subjects (n = 10), patients with systemic lupus erythematosus (n = 24), and patients with rheumatoid arthritis (n = 19) functioned as control subjects. An additional longitudinal study was performed in ten patients with GPA. Using a validated qPCR test, we measured the IgG4:IgG RNA ratio in all groups and compared the results with known biomarkers.ResultsThe median qPCR score was higher in active GPA (21.4; IQR 12.1–29.6) than in remission/LDA (3.3; IQR 1.6–5.6) (Mann-Whitney U test, p < 0.0001) and outperformed other known disease activity parameters in detecting activity. A cutoff qPCR score of 11.2% differentiated active disease from remission/LDA accurately (AUC 0.993). The qPCR test correlated well with the BVAS (Spearman r = 0.77, p < 0.0001). In the longitudinal study, a decrease in BVAS correlated with qPCR score reduction (paired t test, p < 0.05).ConclusionsThe IgG4:IgG RNA ratio in GPA accurately distinguishes active disease from remission and correlates well with disease activity in these single-center studies. If these results are confirmed in larger longitudinal studies, this test might help to steer treatment decisions in patients with GPA.Electronic supplementary materialThe online version of this article (10.1186/s13075-018-1806-6) contains supplementary material, which is available to authorized users.

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A case of proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA) positive/IgG4-related lung disease

Cited by 5 publications

References 23 publications

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Granulomatosis with polyangiitis in a patient with a thoracic vertebral lesion: a case report

Do ANCA‐associated vasculitides and IgG4‐related disease really overlap or not?

IgG4:IgG RNA ratio differentiates active disease from remission in granulomatosis with polyangiitis: a new disease activity marker? A cross-sectional and longitudinal study

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