Dataset Information

Systemic sclerosis associated interstitial lung disease: a conceptual framework for subclinical, clinical and progressive disease.

ABSTRACT:

Objectives

To establish a framework by which experts define disease subsets in systemic sclerosis associated interstitial lung disease (SSc-ILD).

Methods

A conceptual framework for subclinical, clinical and progressive ILD was provided to 83 experts, asking them to use the framework and classify actual SSc-ILD patients. Each patient profile was designed to be classified by at least four experts in terms of severity and risk of progression at baseline; progression was based on 1-year follow-up data. A consensus was reached if ≥75% of experts agreed. Experts provided information on which items were important in determining classification.

Results

Forty-four experts (53%) completed the survey. Consensus was achieved on the dimensions of severity (75%, 60 of 80 profiles), risk of progression (71%, 57 of 80 profiles) and progressive ILD (60%, 24 of 40 profiles). For profiles achieving consensus, most were classified as clinical ILD (92%), low risk (54%) and stable (71%). Severity and disease progression overlapped in terms of framework items that were most influential in classifying patients (forced vital capacity, extent of lung involvement on high resolution chest CT [HRCT]); risk of progression was influenced primarily by disease duration.

Conclusions

Using our proposed conceptual framework, international experts were able to achieve a consensus on classifying SSc-ILD patients along the dimensions of disease severity, risk of progression and progression over time. Experts rely on similar items when classifying disease severity and progression: a combination of spirometry and gas exchange and quantitative HRCT.

SUBMITTER: Roofeh D

PROVIDER: S-EPMC10152284 | biostudies-literature | 2023 May

REPOSITORIES: biostudies-literature

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Publications

Systemic sclerosis associated interstitial lung disease: a conceptual framework for subclinical, clinical and progressive disease.

Roofeh David D Brown Kevin K KK Kazerooni Ella A EA Tashkin Donald D Assassi Shervin S Martinez Fernando F Wells Athol U AU Raghu Ganesh G Denton Christopher P CP Chung Lorinda L Hoffmann-Vold Anna-Maria AM Distler Oliver O Johannson Kerri A KA Allanore Yannick Y Matteson Eric L EL Kawano-Dourado Leticia L Pauling John D JD Seibold James R JR Volkmann Elizabeth R ER Walsh Simon L F SLF Oddis Chester V CV White Eric S ES Barratt Shaney L SL Bernstein Elana J EJ Domsic Robyn T RT Dellaripa Paul F PF Conway Richard R Rosas Ivan I Bhatt Nitin N Hsu Vivien V Ingegnoli Francesca F Kahaleh Bashar B Garcha Puneet P Gupta Nishant N Khanna Surabhi S Korsten Peter P Lin Celia C Mathai Stephen C SC Strand Vibeke V Doyle Tracy J TJ Steen Virginia V Zoz Donald F DF Ovalles-Bonilla Juan J Rodriguez-Pinto Ignasi I Shenoy Padmanabha D PD Lewandoski Andrew A Belloli Elizabeth E Lescoat Alain A Nagaraja Vivek V Ye Wen W Huang Suiyuan S Maher Toby T Khanna Dinesh D

Rheumatology (Oxford, England) 20230501 5

<h4>Objectives</h4>To establish a framework by which experts define disease subsets in systemic sclerosis associated interstitial lung disease (SSc-ILD).<h4>Methods</h4>A conceptual framework for subclinical, clinical and progressive ILD was provided to 83 experts, asking them to use the framework and classify actual SSc-ILD patients. Each patient profile was designed to be classified by at least four experts in terms of severity and risk of progression at baseline; progression was based on 1-ye ...[more]

PMID: 36173318

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Project description:Background Interstitial lung disease (ILD) is the most common cause of death in patients with systemic sclerosis (SSc). Prognostic biomarkers are needed to identify SSc-ILD patients at risk for progressive pulmonary fibrosis. This study investigates autoantibodies measured in bronchoalveolar lavage (BAL) fluid and in serum in reference to the clinical disease course of SSc-ILD. Methods Fifteen patients with new onset SSc-ILD underwent bronchoscopy. Autoantibody levels were analyzed using addressable laser bead immunoassay from BAL fluid and the serum. In a separate longitudinal cohort of 43 patients with early SSc-ILD, autoantibodies in serum were measured at baseline and pulmonary function tests were performed at least 2 times over the course of at least 2 or more years. Linear mixed effect models were created to investigate the relationship between specific autoantibodies and progression of SSc-ILD. Finally, lung tissue from healthy controls and from subjects with SSc was analyzed for the presence of the Ro52 antigen using immunohistochemistry. Results Among SSc-ILD patients who were positive for anti-Ro52 (N = 5), 3 (60%) had enrichment of anti-Ro52 in BAL fluid at a ratio exceeding 50x. In the longitudinal cohort, 10/43 patients (23%) were anti-Ro52 positive and 16/43 (37%) were anti-scl-70 positive. Presence of anti-Scl-70 was associated with a lower vital capacity (VC) at baseline (-12.6% predicted VC [%pVC]; 95%CI: -25.0, -0.29; p = 0.045), but was not significantly associated with loss of lung function over time (-1.07%pVC/year; 95%CI: -2.86, 0.71; p = 0.230). The presence of anti-Ro52 was significantly associated with the loss of lung function over time (-2.41%pVC/year; 95% CI: -4.28, -0.54; p = 0.013). Rate of loss of lung function increased linearly with increasing anti-Ro52 antibody levels (-0.03%pVC per arbitrary units/mL and year; 95%CI: -0.05, -0.02; p < 0.001). Immunohistochemical staining localized the Ro52 antigen to alveolar M2 macrophages in peripheral lung tissue both in subjects with and without SSc. Conclusions This study suggests that antibodies targeting Ro52 are enriched in the lungs of patients with new-onset SSc-ILD, linking Ro52 autoimmunity to the pulmonary pathology of SSc. Clinical and immunohistochemical data corroborates these findings and suggest that anti-Ro52 may serve as a potential biomarker of progressive SSc-ILD. Supplementary Information The online version contains supplementary material available at 10.1186/s13075-023-03141-4.

Dataset Information

Systemic sclerosis associated interstitial lung disease: a conceptual framework for subclinical, clinical and progressive disease.

Objectives

Methods

Results

Conclusions

Publications

Systemic sclerosis associated interstitial lung disease: a conceptual framework for subclinical, clinical and progressive disease.

Similar Datasets

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