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

Cohort Study of Chinese Patients With Pulmonary Vasculitis

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.

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).

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