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Supervised Machine Learning for Semi-Quantification of Extracellular DNA in Glomerulonephritis
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Highlights from the breakout session: other forms of vasculitis
Tanaz A Kermani1, Kenneth J Warrington2
1Division of Rheumatology, University of California Los Angeles, Los Angeles, CA, USA.
Rheumatology (Oxford, England)
|March 12, 2025
Summary
Methotrexate effectively treats retroperitoneal fibrosis in IgG4-related disease (IgG4-RD). Rituximab shows promise for IgA Vasculitis but has lower remission rates in IgA Nephropathy.
Area of Science:
- Rheumatology
- Nephrology
- Immunology
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a complex fibroinflammatory condition.
- Understanding therapeutic efficacy and diagnostic criteria for IgG4-RD and related vasculitides is crucial.
Purpose of the Study:
- To evaluate methotrexate for retroperitoneal fibrosis in IgG4-RD.
- To assess the histopathologic scoring system of the ACR/EULAR Classification criteria for IgG4-RD.
- To evaluate rituximab's efficacy in adult-onset IgA Vasculitis (IgAV) and crescentic IgA Nephropathy (cIgAN).
Main Methods:
- Prospective multicentre randomized clinical trial of methotrexate with glucocorticoids for retroperitoneal fibrosis.
- Evaluation of ACR/EULAR Classification criteria for IgG4-RD using histopathologic scoring.
- Retrospective analysis of rituximab treatment in adult-onset IgAV and cIgAN.
Main Results:
- Methotrexate combined with glucocorticoids induced remission and reduced prednisone dosage in retroperitoneal fibrosis.
- The ACR/EULAR criteria effectively distinguished between confirmed and suspected IgG4-RD.
- Rituximab achieved a high renal response rate in IgAV, but lower remission rates and poorer renal outcomes were observed in cIgAN.
Conclusions:
- Methotrexate is an effective therapeutic agent for IgG4-related retroperitoneal fibrosis.
- The ACR/EULAR Classification criteria are valuable for diagnosing IgG4-RD.
- Rituximab demonstrates differential efficacy in IgAV and cIgAN, warranting further investigation for cIgAN treatment.
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