Diagnostic Pitfalls and Treatment Challenges in Interstitial Pneumonia With Autoimmune Features: Comment on the Article by Wilfong et al

Novikov, Pavel; Shchegoleva, E.; Akulkina, Larisa; Bulanov, Nikolai; Vinogradova, Ekaterina; Moiseev, Sergey

doi:10.1002/art.40783

Cited by 10 publications

(4 citation statements)

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“…This is especially relevant given that “anti-Ro (SS-A)” is part of the serologic domain for interstitial pneumonia with autoimmune features [33]. This classification is increasingly used in research and clinical practice for undifferentiated ILD [36, 37]. Deliberate anti-Ro52 testing may therefore help to phenotype patients with previously unclassifiable ILD.…”

Section: Discussionmentioning

confidence: 99%

Presentations and outcomes of interstitial lung disease and the anti-Ro52 autoantibody

et al. 2019

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BackgroundDistinct clinical presentations of interstitial lung disease (ILD) with the myositis-specific antibodies, including anti-synthetase antibodies, are well-recognized. However, the association between ILD and the myositis-associated antibodies, including anti-Ro52, is less established. Our objectives were to compare presenting phenotypes of patients with anti-Ro52 alone versus in combination with myositis-specific autoantibodies and to identify predictors of disease progression or death.MethodsWe performed a retrospective cohort study of 73 adults with ILD and a positive anti-Ro52 antibody. We report clinical features, treatment, and outcomes.ResultsThe majority of patients with ILD and anti-Ro52 had no established connective tissue disease (78%), and one-third had no rheumatologic symptoms. Thirteen patients (17.8%) required ICU admission for respiratory failure, with 84.6% all-cause mortality. Of the 73 subjects, 85.7% had a negative SS-A, and 49.3% met criteria for idiopathic pneumonia with autoimmune features (IPAF). The 50 patients with anti-Ro52 alone were indistinguishable from patients with anti-Ro52 plus a myositis-specific autoantibody. ICU admission was associated with poor outcomes (HR 12.97, 95% CI 5.07–34.0, p < 0.0001), whereas rheumatologic symptoms or ANA > = 1:320 were associated with better outcomes (HR 0.4, 95% CI 0.16–0.97, p = 0.04, and HR 0.29, 95% CI 0.09–0.81, p = 0.03, respectively).ConclusionsPresentations of ILD with the anti-Ro52 antibody are heterogeneous, and outcomes are similar when compared to anti-Ro52 plus myositis-specific antibodies. Testing for anti-Ro52 may help to phenotype unclassifiable ILD patients, particularly as part of the serologic criteria for IPAF. Further research is needed to investigate treatment of ILD in the setting of anti-Ro52 positivity.

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Section: Discussionmentioning

confidence: 99%

Presentations and outcomes of interstitial lung disease and the anti-Ro52 autoantibody

et al. 2019

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“…Testing for ANCA is typically not performed as part of the diagnostic work-up of IIP and has also not been included in the research criteria for interstitial pneumonia with autoimmune features (IPAF) due to its association with the vasculitides, rather than the CTD-ILD spectra of disorders [68]. Given the ill-defined criteria for CTD as well as the fine line between CTD and systemic vasculitides, this approach has been disputed by experts [69,70]. ANCA positivity in patients with IIP can have prognostic implications including the risk of future development of AAV, as well as potential therapeutic implications including consideration of immunomodulating agents when appropriate.…”

Section: Laboratory Findingsmentioning

confidence: 99%

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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“…и/или выявляются указанные выше аутоантитела, однако не может быть установлен диагноз определенного системного заболевания соединительной ткани в связи с тем, что они не удовлетворяют необходимым классификационным критериям [27]. АНЦА не были включены в серологический домен критериев ИПАП, так как, по мнению экспертов, васкулиты не относятся к системным заболеваниям соединительной ткани и должны рассматриваться отдельно [18,27], хотя эта точка зрения представляется спорной [28].…”

Section: анца-ассоциированные интерстициальные заболевания легких и и...unclassified

ANCA-associated interstitial lung disease and interstitial pneumonia with autoimmune features

Skvortsov,

Akulkina,

Novikov

et al. 2024

CPT

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Diagnostic Pitfalls and Treatment Challenges in Interstitial Pneumonia With Autoimmune Features: Comment on the Article by Wilfong et al

Cited by 10 publications

References 9 publications

Presentations and outcomes of interstitial lung disease and the anti-Ro52 autoantibody

Presentations and outcomes of interstitial lung disease and the anti-Ro52 autoantibody

Antineutrophil cytoplasmic antibody-associated interstitial lung disease: a review

ANCA-associated interstitial lung disease and interstitial pneumonia with autoimmune features

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