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

Chinese Academy of Medical Sciences & Peking Union Medical College

Status: Unknown

Conditions: Granulomatosis With Polyangiitis; ANCA-associated Vasculitis; EosinphilicGranulomatosis With Polyangiitis; Microscopic Polyangiitis

Study type: Observational

Funder types: Other

Identifiers

NCT02126098

PUMCH-PULMONARY DISEASES

2011BA11B17 (Other Grant/Funding Number)

Details and patient eligibility

About

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.

Full 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).

Enrollment: 100 estimated patients

Sex: All

Ages: 18 to 75 years old

Volunteers: No Healthy Volunteers

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

Trial design

100 participants in 1 patient group

Adults who diagosed ANCA-vasculitis

Description:

Patients with Wegener's granulomatosis or microscopic polyangiitis were eligible to participate in the study if they had 1. Positive serum assays for proteinase 3-ANCA or myeloperoxidase-ANCA 2. 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)