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.
ILD patients with serum ANCA-positivity were enrolled in this study. 1. Baseline data collection: 1. Demographics information (age, gender) 2. Clinical course 3. Clinical symptoms and signs (cough, dyspnea, fever, fatigue, crackling, clubbing fingers, mechanics hand) 4. Laboratory findings (blood and urine routine, liver and renal function tests, erythrocyte sedimentation rate, C reactive protein) 5. 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) 6. Systemic manifestation if diagnosed as systemic vasculitis 7. Pulmonary function tests (ventilation and diffusion capacity test) 8. Chest high-resolution computed tomography (HRCT) 2. Treatment: treatment will be given according to the experience of each pulmonologist. 3. Follow-up: 1. Patients were followed up at least once a year and basic laboratory tests, serologic autoantibodies, pulmonary function test and chest HRCT were evaluated routinely 2. Follow-up end point was April 2019. 4. Outcome
Study Type
OBSERVATIONAL
Enrollment
80
Peking Union Medical College Hospital
Beijing, Beijing Municipality, China
Death From All Causes
Death from all causes Retrieved from medical records.
Time frame: 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 Retrieved from medical records.
Time frame: From baseline until the date of transplantation, up to 5 years
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