Longitudinal Protocol for Granulomatosis With Polyangiitis (Wegener’s) and Microscopic Polyangiitis

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Brief Title

Longitudinal Protocol for Granulomatosis With Polyangiitis (Wegener's) and Microscopic Polyangiitis

Official Title

Determining Disease Activity Biomarkers in Individuals With Granulomatosis With Polyangiitis (Wegener's) and Microscopic Polyangiitis

Brief Summary

      Granulomatosis with polyangiitis (Wegener's) (GPA) and microscopic polyangiitis (MPA) are two
      rare immune system disorders that cause the inflammation of blood vessels, or vasculitis. In
      order to properly treat these diseases, it is critical that the level of disease activity can
      be determined over the course of the disease. The purpose of this study is to determine new
      biological markers, or biomarkers, that may be used to assess the severity of disease in
      people with GPA or MPA.
    

Detailed Description

      GPA and MPA are two autoimmune disorders that cause systemic vasculitis. GPA commonly affects
      the upper respiratory tract, the lungs, and the kidneys. MPA is marked by kidney
      inflammation, weight loss, skin lesions, nerve damage, and fever. Many patients with WG or
      MPA show no visible symptoms of active disease; it is known that underlying subclinical
      disease activity leads to long-term damage in these patients. Also, because it is difficult
      to monitor WG and MPA disease activity, it is difficult for clinicians to know when and how
      to treat these patients. This study will use new scientific methods to identify new
      biomarkers that can be used to monitor disease activity in GPA and MPA patients. These
      biomarkers may be used to help direct clinical care for GPA and MPA patients and assist in
      future drug development.

      Study visits will occur monthly for the first year, then every 3 months thereafter for the
      remainder of the study. Blood and urine collection will occur at every visit. A physical exam
      and medical and medication history will occur every 3 months; also, participants will be
      asked to complete several questionnaires to assess disease activity, health status, and
      tobacco, alcohol, and drug use. Participants may have additional study visits if a disease
      flare or disease-related complications occur during the study.
    


Study Type

Observational


Primary Outcome

Discover biomarkers in GPA/MPA capable of measuring disease activity and response to treatment.

Secondary Outcome

 Measure the predictive value of biomarkers for clinical outcome in GPA/MPA

Condition

Granulomatosis With Polyangiitis



Publications

* Includes publications given by the data provider as well as publications identified by National Clinical Trials Identifier (NCT ID) in Medline.

Recruitment Information



Estimated Enrollment

1200

Start Date

April 2006

Completion Date

September 2022

Primary Completion Date

September 2022

Eligibility Criteria

        Inclusion Criteria:

          -  Diagnosis of GPA or MPA. Widely accepted diagnostic criteria, as opposed to
             classification criteria or definitions, have not been developed for GPA and MPA.

          -  For diagnosis of GPA, meets at least 2 of the following 5 modified American College of
             Rheumatology (ACR) criteria:

               1. Nasal or oral inflammation with oral ulcers or nasal discharge with pus or blood

               2. Abnormal chest radiograph with nodules, fixed infiltrates, or cavities

               3. Urinary sediment with microhematuria or red cell casts

               4. Granulomatous inflammation within the wall of an artery or in the perivascular
                  area on biopsy

               5. Antineutrophil cytoplasmic antibody (ANCA) positive by enzyme immunoassay for
                  either PR3- or MPO-ANCA

          -  For diagnosis of MPA, meets the Chapel Hill Consensus Conference Definition for MPA:

               1. Necrotizing vasculitis, with few or no immune deposits, that affects small
                  vessels (i.e., capillaries, venules, arterioles)

               2. Necrotizing arteritis involving small- and medium-sized arteries may be present

               3. Necrotizing glomerulonephritis is very common

               4. Pulmonary capillaritis often occurs

          -  Parent or guardian willing to provide informed consent, if applicable

        Exclusion Criteria:

          -  Simultaneous diagnoses of both GPA and MPA

          -  Granulomatosis with polyangiitis (Churg-Strauss)

          -  Takayasu's arteritis

          -  Giant cell arteritis

          -  Polyarteritis nodosa

          -  Cogan's syndrome

          -  Behcet's disease

          -  Sarcoidosis

          -  Kawasaki disease

          -  Tuberculosis or any atypical mycobacterial infections

          -  Deep fungal infections

          -  Lymphoma, lymphomatoid granulomatosis, or any other type of cancer that mimics
             anti-neutrophil cytoplasmic antibody-associated vasculitis (AAVs)

          -  Cryoglobulinemic vasculitis

          -  Systemic lupus erythematosus

          -  Rheumatoid arthritis

          -  Mixed connective tissue disease or any overlapping autoimmune syndrome
      

Gender

All

Ages

N/A - N/A

Accepts Healthy Volunteers

No

Contacts

Peter A. Merkel, MD, MPH, , 

Location Countries

Canada

Location Countries

Canada

Administrative Informations


NCT ID

NCT00315393

Organization ID

VCRC5505

Secondary IDs

U54AR057319

Responsible Party

Principal Investigator

Study Sponsor

University of Pennsylvania

Collaborators

 Office of Rare Diseases (ORD)

Study Sponsor

Peter A. Merkel, MD, MPH, Study Chair, University of Pennsylvania


Verification Date

May 2021