Interstitial Lung Disease in Antineutrophil Cytoplasmic Antibody–Associated Vasculitis Patients

Kwon, Minkyung; Lee, Augustine S.; Mira-Avendano, Isabel; Rojas, Carlos; Grage, Rolf A.; Abril, Andy

doi:10.1097/rhu.0000000000001357

Cited by 8 publications

(11 citation statements)

References 23 publications

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“…In IPF patients, a positivity for ANCA antibodies may be observed in about 5-10% of cases [4,5]. In particular, anti-MPO antibodies have been reported in 4-36% of patients with idiopathic interstitial pneumonia, including IPF, while the occurrence of MPA has been described in a range from 1.7 to 25.7% of patients [20,21]. This group of patients might be called "pulmonary limited vasculitis" and considered a phenotypic variant of MPA, as reported by Katsumata et al [22].…”

Section: Discussionmentioning

confidence: 99%

Interstitial Lung Disease and Anti-Myeloperoxidase Antibodies: Not a Simple Association

Sebastiani

Luppi

Sambataro

et al. 2021

JCM

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Anti-neutrophil cytoplasmic antibodies (ANCA), mainly anti-myeloperoxidase (MPO) antibodies, have been frequently identified in patients with idiopathic pulmonary fibrosis (IPF). However, their role remains unclear, and only 7–23% of these patients develops clinically overt vasculitis. We aimed to investigate the clinical, serological, and radiological features and prognosis of anti-MPO-positive interstitial lung disease (ILD) patients. Fifty-eight consecutive patients firstly referred for idiopathic interstitial pneumonia and showing serological positivity of anti-MPO antibodies were retrospectively enrolled. For each patient, clinical data, lung function testing, chest high-resolution computed tomography (HRCT) pattern, and survival were recorded. Thirteen patients developed a rheumatic disease during a median follow-up of 39 months. Usual interstitial pneumonia (UIP) was the most frequent ILD pattern, significantly influencing the patients’ survival. In fact, while the 52-week survival of the overall population was 71.4 ± 7.5%, significantly higher than IPF, survivals of anti-MPO patients with UIP pattern and IPF were similar. Forced vital capacity and diffusion lung capacity for CO significantly declined in 37.7 and 41.5% of cases, respectively, while disease progression at chest HRCT was observed in 45.2%. A careful clinical history and evaluation should always be performed in ILD patients with anti-MPO antibodies to quickly identify patients who are developing a systemic rheumatic disease.

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Section: Discussionmentioning

confidence: 99%

Interstitial Lung Disease and Anti-Myeloperoxidase Antibodies: Not a Simple Association

Sebastiani

Luppi

Sambataro

et al. 2021

JCM

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“…In a series of 24 patients with AAV and ILD, it was found that pulmonary function was progressing over time. 145 In addition, ILD presence among patients with MPA appeared to decrease life expectancy by almost 50% 148 and was associated with a 4-fold higher mortality. 149 In a series of 62 French patients with AAV and ILD, the 1-, 3- and 5-year survival rates were 93.5%, 89.6% and 83.8% respectively, with older age at AAV diagnosis, alveolar hemorrhage and UIP pattern being associated with increased mortality.…”

Section: Anca-associated Vasculitis (Aav)mentioning

confidence: 99%

Natural history and screening of interstitial lung disease in systemic autoimmune rheumatic disorders

Panagopoulos

Goules

Hoffmann-Vold

et al. 2021

Therapeutic Advances in Musculoskeletal

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Interstitial lung disease (ILD) is a relatively frequent manifestation of systemic autoimmune rheumatic disorders (SARDs), including systemic sclerosis (SSc), rheumatoid arthritis (RA), idiopathic inflammatory myopathies (IIM), systemic lupus erythematosus (SLE), primary Sjögren’s syndrome (pSS), and anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis. Interstitial pneumonia with autoimmune features (IPAF) has been proposed to describe patients with ILD who have clinical or serological findings compatible with SARDs but they are not sufficient for a definite diagnosis. ILD may present with different patterns among patients with SARDs, but most commonly as nonspecific interstitial pneumonia (NSIP), with the exception of RA and ANCA vasculitis that more often present with usual interstitial pneumonia (UIP). The natural history of ILD is quite variable, even among patients with the same SARD. It may present with subclinical features following a slow progressively course or with acute manifestations and clinically significant rapid progression leading to severe deterioration of pulmonary function and respiratory failure. The radiographic pattern of ILD, the extent of the disease, the baseline pulmonary function, the pulmonary function deterioration rate over time and clinical variables related to the primary SARD, such as age, sex and the clinical phenotype, are considered prognostic factors for SARDs-ILD associated with adverse outcomes and increased mortality. Different modalities can be employed for ILD detection including clinical evaluation, pulmonary function tests, high resolution computed tomography and novel techniques such as lung ultrasound and serum biomarkers. ILD may determine the clinical outcome of SARDs, since it is associated with significant morbidity and mortality and therefore screening of patients with SARDs for ILD is of great clinical importance.

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“…Similar to other fibrotic interstitial pneumonias, both isolated ANCA-IIP and AAV-ILD are associated with a restrictive pattern with reduced total lung capacity, forced vital capacity (FVC) and diffusing capacity for carbon monoxide, though co-existing airflow obstruction can occasionally be observed in AAV-ILD [7,8,11,15,16,18,21,26,27,31]. FVC and diffusing capacity have been shown to decline over time [11,26,31].…”

Section: Pulmonary Function Testing and Bal Cellular Findingsmentioning

confidence: 99%

“…Commonly described radiographic imaging patterns of ANCA/AAV-ILD include ground glass opacities, reticulation, interlobular septal thickening, consolidation, nodular pattern and honeycombing [10,15,18,26,27,30,31,32,73]. Airway lesions have also been reported and include bronchiolitis, bronchial wall thickening and bronchiectasis [71,73].…”

Section: Imagingmentioning

confidence: 99%

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Antineutrophil cytoplasmic antibody-associated interstitial lung disease: a review

Kadura

Raghu

2021

Eur Respir Rev

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Over the past three decades, an increasing number of publications have reported the association between interstitial lung disease (ILD) and anti-neutrophil cytoplasmic antibody (ANCA) or ANCA-associated vasculitis (AAV). With this increased awareness, we have reviewed the literature to date and provide an update in this narrative review. The vast majority of cases of ILD have been shown to be in the setting of positive anti-myeloperoxidase antibody and can be present in up to 45% of patients of microscopic polyangiitis, though cases of ILD associated with proteinase 3 ANCA have rarely been reported. Pulmonary fibrosis and ANCA positivity can occur with or without systemic involvement. The pathogenetic mechanisms establishing the relationship between ANCA and the development of pulmonary fibrosis remain unclear. Histologic and radiographic features of ANCA-ILD most commonly reveal usual interstitial pneumonia or non-specific interstitial pneumonia patterns, though other atypical features such as bronchiolitis have been described. ILD in the setting of AAV has been associated with worse outcomes, and thus early identification and treatment in these patients is appropriate. We advocate that ANCA antibody testing be performed as a baseline evaluation in patients presenting with idiopathic interstitial pneumonia. Suggested treatment of ANCA-ILD includes immunosuppression and/or antifibrotic agents, though supporting data and clinical trials to substantiate use of these therapies are needed.

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Interstitial Lung Disease in Antineutrophil Cytoplasmic Antibody–Associated Vasculitis Patients

Abstract: Supplemental digital content is available in the text.

Cited by 8 publications

References 23 publications

Interstitial Lung Disease and Anti-Myeloperoxidase Antibodies: Not a Simple Association

Interstitial Lung Disease and Anti-Myeloperoxidase Antibodies: Not a Simple Association

Natural history and screening of interstitial lung disease in systemic autoimmune rheumatic disorders

Antineutrophil cytoplasmic antibody-associated interstitial lung disease: a review

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