Anti-neutrophil Cytoplasmic Antibody in Interstitial Lung Disease.

Study Overview
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Brief Summary
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The purpose of this study is to investigate the clinical features and long-term outcome of anti-neutrophil cytoplasmic antibody (ANCA)-positive interstitial lung disease (ILD) and assess the difference between microscopic polyangiitis (MPA) associated ILD and isolated ANCA-positive idiopathic interstitial pneumonia.

Detailed Description
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ILD patients with serum ANCA-positivity were enrolled in this study. Baseline data collection: Demographics information (age, gender) Clinical course Clinical symptoms and signs (cough, dyspnea, fever, fatigue, crackling, clubbing fingers, mechanics hand) Laboratory findings (blood and urine routine, liver and renal function tests, erythrocyte sedimentation rate, C reactive protein) Serologic tests (ANA , Rheumatoid factor , Anti-cyclic citrullinated peptide (CCP), Anti-dsDNA, Anti-Ro (SS-A), Anti-La (SS-B), Anti-Smith, Anti-Scl-70, Anti-Jo-1) Systemic manifestation if diagnosed as systemic vasculitis Pulmonary function tests (ventilation and diffusion capacity test) Chest high-resolution computed tomography (HRCT) Treatment: treatment will be given according to the experience of each pulmonologist. Follow-up: Patients were followed up at least once a year and basic laboratory tests, serologic autoantibodies, pulmonary function test and chest HRCT were evaluated routinely Follow-up end point was April 2019. Outcome

Official Title
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Observational Study of Clinical Features and Outcome of Interstitial Lung Disease With Anti-neutrophil Cytoplasmic Antibody in Chinese Patients.

Conditions
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Interstitial Lung Disease, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Males and females Aged from 18 to 85 years with informed consent Have a diagnosis of ILD based on clinical symptoms and radiologic features, with or without histopathologic results Have available ANCA testing results during the first visit and follow-up period Exclusion Criteria: Connective tissue disease associated ILD ILD induced by drug, environment, or occupational exposure Hypersensitivity pneumonitis and sarcoidosis

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 85 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Death from all cause | Death from all cause | From baseline until the date of death from all cause, up to 5 years | 
| Number of patients who underwent lung transplantation | Number of patients who underwent lung transplantation | From baseline until the date of transplantation, up to 5 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Interstitial Lung Disease, Anti-Neutrophil Cytoplasmic Antibody, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis