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.

Study Overview

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

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).

• Study Type: Observational
• Enrollment (Anticipated): 100

Contacts and Locations

Study Locations

• China
  • Beijing
    • Beijing, Beijing, China, 100730
      • Peking Union Medical College Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 75 years (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

Patients who newly diagnosed ANCA-vasculitis

Description

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

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort

Adults who diagosed ANCA-vasculitis; 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)

Other Outcome Measures

Outcome Measure	Measure Description	Time Frame
Adverse events	deaths (from all causes); malignant conditions; grade 2 or higher leukopenia or thrombocytopenia; grade 3 or higher infections; drug induced cystitis; venous thromboembolic events; stroke; hospitalizations	during the period of observation (30months)

Collaborators and Investigators

• Sponsor: Peking Union Medical College Hospital
• Investigators: Study Chair: Juhong Shi, M.D, Peking Union Medical College Hospital

Study record dates

Study Major Dates

• Study Start: May 1, 2014
• Primary Completion (Anticipated): November 1, 2017
• Study Completion (Anticipated): December 1, 2017

Study Registration Dates

• First Submitted: April 27, 2014
• First Submitted That Met QC Criteria: April 27, 2014
• First Posted (Estimate): April 29, 2014

Study Record Updates

• Last Update Posted (Estimate): April 29, 2014
• Last Update Submitted That Met QC Criteria: April 27, 2014
• Last Verified: April 1, 2014

More Information

Terms related to this study

• Keywords: Granulomatosis with polyangiitis; ANCA-associated vasculitis; Microscopic polyangiitis; Wegner's granulomatosis; Eosinphilic granulomatosis with polyangiitis
• Additional Relevant MeSH Terms: Cardiovascular Diseases; Vascular Diseases; Cerebrovascular Disorders; Brain Diseases; Central Nervous System Diseases; Nervous System Diseases; Respiratory Tract Diseases; Immune System Diseases; Autoimmune Diseases; Lung Diseases; Lung Diseases, Interstitial; Cerebral Small Vessel Diseases; Vasculitis; Granulomatosis with Polyangiitis; Microscopic Polyangiitis; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Systemic Vasculitis
• Other Study ID Numbers: PUMCH-PULMONARY DISEASES; 2011BA11B17 (Other Grant/Funding Number: 2011BA11B17the Ministry of Science and Technology of China)