Observation Study of Clinical Manifestation and Outcome in Chinese Patients With Pulmonary Vasculitis

Study Overview
=================
Brief Summary
-----------------
The purpose of this study is to observe the clinical manifestation, Lab findings including chest CT scans, pathological findings and outcomes in chinese patients with pulminary vasculitis.

Detailed Description
-----------------
Presentation of patient with suspected vasculitis Established diagnosis Clinical findings Laboratory workup Tissue biopsy Angiogram where appropriate Evaluating respiratory system Chest HRCT Pulmonary function test Bronchoscopy exam(BALF, TBB) Lung biopsy(needle biopsy, VAST) screening underlying damage to other system Paranasal sinus, vision & audition Nervous system Kidney Gastrointestinal tract Heart & vessel Skin Hematology Muscle, bone & joint Treatment Prognosis Evaluation Study visits occurred at baseline; at weeks 4; and at 2, 4, 6,12,18,24,30 months. Disease activity was measured on the basis of the BVAS/WG and the physician's global assessment. Damage related to disease or treatment was scored according to the Vasculitis Damage Index (scores for this index range from 0 to 64, with higher scores in- dicating more severe damage). Health related quality of life was scored with the use of the Med- ical Outcomes Study 36-Item Short-Form Health Survey (SF-36).Scores on this scale range from 0 to 100, with higher scores indicating better health. Serial serum samples were tested for proteinase 3-ANCA and myeloperoxidase-ANCA by means of a direct enzyme-linked immunosorbent assay (ELISA).

Official Title
-----------------
Cohort Study of Chinese Patients With Pulmonary Vasculitis

Conditions
-----------------
ANCA-associated Vasculitis, Granulomatosis With Polyangiitis, Microscopic Polyangiitis, EosinphilicGranulomatosis With Polyangiitis

Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: Males and Females Aged from 18-75 years with informed consent Patients with ANCA-vasculitis Wegener's granulomatosis, microscopic polyangiitis or CSS positive serum assays for proteinase 3-ANCA or myeloperoxidase-ANCA manifestations of activity disease, and Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG) of 3 or more (scores range from 0 to 63, with higher scores indicating more active disease) Exclusion Criteria: Females planning to bear a child recently or with childbearing potential Secondary vasculitis, cancer,infective disease or drug induced vasculitis

Ages Eligible for Study
-----------------
Minimum Age: 18 Years
Maximum Age: 75 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Adults who diagosed ANCA-vasculitis<br>Patients with Wegener's granulomatosis or microscopic polyangiitis were eligible to participate in the study if they had Positive serum assays for proteinase 3-ANCA or myeloperoxidase-ANCA manifestations of severe disease,11 and a Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG) of 3 or more (scores range from 0 to 63, with higher scores indicating more active disease) | |

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Completely Remission | BVAS/WG of 0 and successful completion of the prednisone taper at 6 months. | 6 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Disease flare | Disease flare an increase in the BVAS/WG of 1 point or more. Patients were classified as having early treatment failure if at 1 month their BVAS/WG had not decreased by at least 1 point or a new manifestation of disease had emerged. | during the period of observation(30months) | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
ANCA-associated vasculitis, Granulomatosis with polyangiitis, Microscopic polyangiitis, Wegner's granulomatosis, Eosinphilic granulomatosis with polyangiitis