Interstitial lung disease in ANCA-associated vasculitis: another step forward

Sebastiani, Marco; Manfredi, Andreina; Faverio, Paola; Salvarani, Carlo; Luppi, Fabrizio

doi:10.55563/clinexprheumatol/tpm0on

Cited by 15 publications

(25 citation statements)

References 7 publications

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“…[27][28][29] Therefore, ILD itself could induce MPO-ANCA production as a result of neutrophil destruction during the chronic inflammation process, perhaps explaining the appearance of ANCA after the onset of ILD. 30 Thus, we do not consider ILD as a priori disease manifestation of MPA but rather a pre-existing comorbidity, and antifibrotic drugs can be considered a reasonable treatment option for fibrotic ILD patients with ANCA positivity.…”

Section: Discussionmentioning

confidence: 99%

Clinical significance of acute exacerbation in interstitial lung disease with antineutrophil cytoplasmic antibody: an indicator of poor prognosis

Zhang

Cen

et al. 2022

Ther Adv Respir Dis

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Background: The association between interstitial lung disease (ILD) and antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) has been increasingly recognized in recent years. The clinical features and prognostic differences between AAV-associated ILD and isolated ANCA-positive idiopathic interstitial pneumonias (IIPs) remain unclear. The purpose of this study was to determine the clinical significance and prognosis of ANCA-positive ILD to further guide clinical management. Methods: This study retrospectively reviewed the data of 379 ILD patients with available ANCA results and ultimately analysed 49 ANCA-positive patients. AAV diagnosis was based on the 2012 revised Chapel Hill Consensus Conference (CHCC) criteria, and 33 of 49 patients were diagnosed with microscopic polyangiitis (MPA). The baseline clinical information and laboratory parameters were collected and analysed at each patient’s initial diagnosis. Results: Among 49 ANCA-positive ILD patients, the high-resolution computed tomography (HRCT) pattern was mainly usual interstitial pneumonia (UIP) (59.18%), followed by nonspecific interstitial pneumonia (NSIP) (26.53%). The C-reactive protein (CRP) level (43.89± 40.61 versus 18.74± 20.05, p = 0.028) and erythrocyte sedimentation rate (ESR) (71.97± 42.73 versus 40.69± 28.46, p = 0.011) were significantly higher in the MPA-ILD group than in the ANCA-IIP group. Haemoglobin (113.09 ± 24.47 versus 132.19± 13.34, p = 0.006) and albumin (32.95± 5.84 versus 36.52± 3.94, p = 0.032) levels were significantly lower. Survival was shorter among MPA-ILD patients than among ANCA-IIP patients [hazard ratio (HR) 3.38, 95% confidence interval (CI) 1.32–8.67, p = 0.040]. In the multivariable Cox analysis, a diagnosis of MPA (HR 3.91, 95% CI 1.07–14.08, p = 0.038) and acute exacerbation (AE) of ILD (HR 9.43, 95% CI 2.89–30.30, p < 0.001) were significantly independently associated with shorter survival in ANCA-positive ILD patients, and the NSIP pattern (HR 0.07, 95% CI 0.01–0.41, p = 0.003) was independently associated with prolonged survival. Conclusion: ANCA-ILD patients mostly have myeloperoxidase (MPO)-ANCA positivity and an MPA diagnosis. Survival was shorter among MPA-ILD patients than among ANCA-IIP patients. Respiratory failure and AE were associated with poorer prognosis. Early antifibrotic treatment may be a reasonable treatment option in fibrotic ILD patients with ANCA positivity.

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Section: Discussionmentioning

confidence: 99%

Clinical significance of acute exacerbation in interstitial lung disease with antineutrophil cytoplasmic antibody: an indicator of poor prognosis

Zhang

Cen

et al. 2022

Ther Adv Respir Dis

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“…Clinical significance of ILD among patients with AAV has recently gained increasing attention. AAV-related ILD (AAV-ILD) is identified in up to 45% of patients with MPA and 23% of GPA [2], which is particularly common in the Asian population [9]. The prevalence of ILD is about 26-66% in Asian studies, while only 3-36% from Western publications [10][11][12][13][14][15][16].…”

Section: Introductionmentioning

confidence: 99%

“…The prevalence of ILD is about 26-66% in Asian studies, while only 3-36% from Western publications [10][11][12][13][14][15][16]. Usual interstitial pneumonia (UIP) is the most common pattern, described in almost half of the patients [9]. Nonspecific interstitial pneumonia (NSIP) ranks second, being described in less than a third of cases [17].…”

Section: Introductionmentioning

confidence: 99%

Patterns of Interstitial Lung Disease and Prognosis in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

Zhou

et al. 2023

Respiration

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Background: Interstitial lung disease (ILD) is a common pulmonary manifestation of antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Objectives: We aimed to clarify the clinical predictors of mortality in a cohort of patients with AAV-related ILD (AAV-ILD). Method: We retrospectively identified AAV-ILD patients seen at Peking University First Hospital from January 2010 to June 2020 and manually screened for study inclusion. Baseline computed tomography (CT) images were further classified as nonspecific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), organizing pneumonia (OP), and unclassified ILD. Disease characteristics and other pulmonary findings including pulmonary function test and bronchoalveolar lavage (BAL) were also evaluated. Multivariable Cox regression analysis was performed to identify clinical predictors of mortality. Results: The cohort included 204 patients with AAV-ILD, 152 had UIP on CT (AAV-UIP), 39 had NSIP on CT (AAV-NSIP), 3 had OP, and 10 had unclassified ILD. Microscopic polyangiitis was more prevalent in patients with UIP, while granulomatosis with polyangiitis was more common in the NSIP and OP groups, and eosinophilic granulomatosis with polyangiitis was more frequent in patients with unclassified ILD. ILD diagnosis before AAV was more common in patients with either UIP or NSIP patterns. During the median follow-up of 40 months, 44 (21.6%) patients died. One- and 5-year overall survival rates were 88.2% (95% CI, 83.7–92.7%) and 81.0% (95% CI, 74.9–87.1%) for the entire cohort. Patients with UIP patterns had the worst prognosis, while those with NSIP patterns had the best long-term outcome. Specifically, patients with UIP patterns had an approximately 5-fold risk of death compared to those with NSIP. After controlling for potential confounding factors, we observed that each 10% increase in the BAL fluid neutrophil percentage was associated with nearly a 20% increased risk of death (HR 1.195, 95% CI 1.018–1.404). Conclusions: Clinical characteristics and survival differ between subgroups defined by CT patterns. BAL fluid neutrophilia is an independent predictor of mortality among AAV-ILD patients, and therefore, the clinical utility of BAL at the time of AAV diagnosis should be considered.

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“…found that the mortality was increased to two to four times in AAV patients with interstitial lung disease, especially in those with MPA. [ 40 ] Pulmonary involvement is uncommon in PAN. [ 41 ] Therefore, it was not surprising that pulmonary involvement was not related to poor outcomes in the rFFS generating cohort, in which about one-third of the patients were with PAN.…”

Section: Discussionmentioning

confidence: 99%

Development and internal validation of a model to predict long-term survival of ANCA associated vasculitis

Chen

Tian

et al. 2023

Rheumatology and Immunology Research

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Objectives Risk stratification and prognosis prediction are critical for appropriate management of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). Herein, we aim to develop and internally validate a prediction model specifically for long-term survival of patients with AAV. Methods We thoroughly reviewed the medical charts of patients with AAV admitted to Peking Union Medical College Hospital from January 1999 to July 2019. The Least Absolute Shrinkage and Selection Operator method and the COX proportional hazard regression was used to develop the prediction model. The Harrell's concordance index (C-index), calibration curves and Brier scores were calculated to evaluate the model performance. The model was internally validated by bootstrap resampling methods. Results A total of 653 patients were included in the study, including 303 patients with microscopic polyangiitis, 245 patients with granulomatosis with polyangiitis and 105 patients with eosinophilic granulomatosis with polyangiitis, respectively. During a median follow-up of 33 months (interquartile range 15–60 months), 120 deaths occurred. Age at admission, chest and cardiovascular involvement, serum creatinine grade, hemoglobin levels at baseline and AAV sub-types were selected as predictive parameters in the final model. The optimism-corrected C-index and integrated Brier score of our prediction model were 0.728 and 0.109. The calibration plots showed fine agreement between observed and predicted probability of all-cause death. The decision curve analysis (DCA) showed that in a wide range of threshold probabilities, our prediction model had higher net benefits compared with the revised five factor score (rFFSand) and the birmingham vasculitis activity score (BVAS) system. Conclusion Our model performs well in predicting outcomes of AAV patients. Patients with moderate-to-high probability of death should be followed closely and personalized monitoring plan should be scheduled.

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Interstitial lung disease in ANCA-associated vasculitis: another step forward

Cited by 15 publications

References 7 publications

Clinical significance of acute exacerbation in interstitial lung disease with antineutrophil cytoplasmic antibody: an indicator of poor prognosis

Clinical significance of acute exacerbation in interstitial lung disease with antineutrophil cytoplasmic antibody: an indicator of poor prognosis

Patterns of Interstitial Lung Disease and Prognosis in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

Development and internal validation of a model to predict long-term survival of ANCA associated vasculitis

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