Abstract: FR-PO0649
Risk of Disease Progression and Tolerance of Antifibrotic Agents in Patients with ANCA-Associated Pulmonary-Renal Syndrome with Interstitial Lung Disease
Session Information
- Glomerular Diseases: Clinical, Outcomes, and Therapeutics Research - ANCA/FSGS
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Hackett, William, The University of Manchester Faculty of Biology Medicine and Health, Manchester, England, United Kingdom
- Muralitharan, Guganeshan, The University of Manchester Faculty of Biology Medicine and Health, Manchester, England, United Kingdom
- Suhail, Taqdees, Manchester University NHS Foundation Trust, Manchester, England, United Kingdom
- Brix, Silke R., Manchester University NHS Foundation Trust, Manchester, England, United Kingdom
Background
Interstitial lung disease (ILD) is a recognised extra-renal manifestation of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and has been associated with adverse outcome in AAV. Systematic screening for ILD has not been established in renal clinical practice and the impact on disease progression and adverse events in patients with ANCA-associated glomerulonephritis has not been demonstrated.
Methods
Retrospective tertiary centre investigation of ANCA GN cohort (n=288). Longitudinal CT data review to assess ILD progression and change of phenotype over time recording treatment responses and tolerability of antifibrotic therapy.
Results
ILD was identified on chest CT in 72 of 288 patients (prevalence 25%). On longitudinal follow-up, 42 of 72 patients (58%) demonstrated ILD progression on serial CT imaging and 36% of patients evolved to a usual interstitial pneumonia (UIP) phenotype, reflecting a pattern of progressive fibrosis.
Among those with progressive ILD, 10 patients met criteria for antifibrotic therapy with nintedanib, however, all 10 experienced gastrointestinal (GI) adverse effects (70% diarrhoea; 30% nausea and vomiting). Five patients (50%) required discontinuation of nintedanib due to persistent intolerance. A further 5 patients with severe progressive ILD were established on home oxygen therapy, indicating the significant morbidity associated with AAV-ILD in this cohort.
Conclusion
This study demonstrates a substantial ILD prevalence of 25% in a UK tertiary ANCA GN cohort, highlighting that ILD is a clinically significant extra-renal manifestation of AAV that warrants systematic screening. The high rate of radiological progression (58%), evolution to a UIP phenotype (36%), and limited tolerability of antifibrotic therapy in this population highlights the need for early detection and multidisciplinary management.
These findings support the incorporation of routine HRCT chest screening and monitoring into the standard management pathway for all AAV patients.
Funding
- Government Support – Non-U.S.