ANCA-associated Vasculitis, EosinphilicGranulomatosis With Polyangiitis, Granulomatosis With Polyangiitis, Microscopic Polyangiitis
Conditions
Keywords
ANCA-associated vasculitis, Granulomatosis with polyangiitis, Microscopic polyangiitis, Wegner's granulomatosis, Eosinphilic granulomatosis with polyangiitis
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 1. Established diagnosis Clinical findings Laboratory workup Tissue biopsy Angiogram where appropriate 2. Evaluating respiratory system Chest HRCT Pulmonary function test Bronchoscopy exam(BALF, TBB) Lung biopsy(needle biopsy, VAST) 3. screening underlying damage to other system Paranasal sinus, vision & audition Nervous system Kidney Gastrointestinal tract Heart & vessel Skin Hematology Muscle, bone & joint 4. Treatment 5. Prognosis Evaluation 1. Study visits occurred at baseline; at weeks 4; and at 2, 4, 6,12,18,24,30 months. 2. Disease activity was measured on the basis of the BVAS/WG and the physician's global assessment. 3. 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). 4. 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. 5. Serial serum samples were tested for proteinase 3-ANCA and myeloperoxidase-ANCA by means of a direct enzyme-linked immunosorbent assay (ELISA).
Interventions
None listed
Sponsors
Peking Union Medical College Hospital
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to 75 Years
Healthy volunteers
No
Inclusion criteria
* Males and Females * Aged from 18-75 years with informed consent * Patients with ANCA-vasculitis 1. Wegener's granulomatosis, microscopic polyangiitis or CSS 2. positive serum assays for proteinase 3-ANCA or myeloperoxidase-ANCA 3. 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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Completely Remission | 6 months | BVAS/WG of 0 and successful completion of the prednisone taper at 6 months. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Disease flare | during the period of observation(30months) | Disease flare 1. an increase in the BVAS/WG of 1 point or more. 2. 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. |
Other
| Measure | Time frame | Description |
|---|---|---|
| Adverse events | during the period of observation (30months) | 1. deaths (from all causes) 2. malignant conditions 3. grade 2 or higher leukopenia or thrombocytopenia 4. grade 3 or higher infections 5. drug induced cystitis 6. venous thromboembolic events 7. stroke 8. hospitalizations |
Countries
China
Contacts
Primary ContactJuhong Shi, M.D
Backup ContactLing Qin, M.D
Outcome results
None listed