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Study Description

The development and validation of accurate biological markers and predictors of disease activity and outcome in Polyarteritis Nodosa (PAN), a form a idiopathic vasculitis, would have a major positive impact on the clinical care of patients with this rare disease, be an important advance in the design of clinical trials and feasibility of new drug development, and provide important insight into the pathogenesis of this condition. Current measures of disease activity, including erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), while helpful in selected settings, are mostly considered highly flawed and markers whose poor correlation with disease flares makes them inadequate to help guide therapy. Similarly, ESR and CRP are clearly unhelpful as diagnostic tests for vasculitis. The current state of clinical investigation for vasculitis relies heavily upon determination of disease status solely by clinical presentation and investigator opinion. Thus, discovery and validation of more precise markers that quantifiably measure activity, predict disease course, and correlate with an important biological process, would be of great importance.

Knowledge to be gained from this study could potentially be highly important. Discovery of effective biomarkers of vasculitis and assessment of long term disease course and complications by the collection of clinical and diagnostic imaging studies proposed could lead to better care and less toxic treatment regimens. Furthermore, insight into the pathophysiology of vasculitis could be gained and this might lead to better treatments and improved outcomes in terms of reduction of vascular and treatment related complications. Ideally, excellent biomarkers reflect a sophisticated understanding of the pathophysiology of a disease.

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Study Inclusion/Exclusion Criteria

Inclusion Criteria:

An adaption of the America College of Rheumatology criteria will be used for the diagnosis of Polyarteritis Nodosa (PAN). At the time of inclusion, one major and one minor criteria or two major criteria or isolated cutaneous PAN must be met. These criteria assume the investigator has diagnosed the patient with vasculitis and these criteria are meant to help more specifically classify the patient's diagnosis.

Adapted American College of Rheumatology (ACR) criteria:

Major criteria (not explained by other causes)

  • Arteriographic abnormality
  • Presence of granulocyte or mixed leukocyte infiltrate in an arterial wall on biopsy
  • Mononeuropathy or polyneuropathy

Minor criteria (not explained by other causes)

  • Weight loss >4 kg
  • Livedo reticularis, cutaneous ulcerations, or skin nodules
  • Testicular pain or tenderness
  • Myalgias
  • Diastolic blood pressure >90mm Hg
  • Elevated BUN or serum creatinine levels
  • Ischemic abdominal pain

Isolated cutaneous Polyarteritis Nodosa

  • Biopsy-proven cutaneous PAN

Exclusion Criteria:

  • Microscopic Polyangiitis
  • Granulomatosis with Polyangiitis (Wegener's)
  • Eosinophilic granulomatosis with polyangitiis (Churg-Strauss syndrome)
  • Takayasu's arteritis
  • Giant cell arteritis
  • Cogan's Syndrome
  • Behçet's disease
  • Sarcoidosis
  • Kawasaki's disease
  • Cryoglobulinemic asculitis
  • Systemic lupus erythematosus
  • Rheumatoid arteritis
  • Mixed connective tissue disease or any overlap autoimmune syndrome
  • Presence of anti-proteinase 3 or anti-myeloperoxidase antineutrophil cytoplasmic antibodies (ANCA)
  • Glomerulonephritis
  • Alveolar hemorrhage
  • Hepatitis B, Hepatitis C, HIV infection
  • Any other infectious form of medium vessel vasculitis
  • Inability of participants (or their guardians in the case of children) to give informed consent and to sign the consent form.

Study History

This study is a project of the Vasculitis Clinical Research Consortium (VCRC), which is a consortium of investigators in 8 major North American vasculitis centers (Boston University, Cleveland Clinic, Johns Hopkins, Mayo Clinic, Mount Sinai Hospital, St. Joseph's Hospital, University of Pennsylvania, University of Pittsburgh, and University of Utah). The VCRC is funded through the National Institutes of Health as a Rare Diseases Clinical Research Network with the purpose of promoting research in vasculitis. This study was activated for enrollment on July 3, 2006.

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Study Attribution
  • Principal Investigator
    • Peter A. Merkel, MD, MPH. University of Pennsylvania, Philadelphia, PA, USA.
  • Funding Source
    • U54 AR057319. National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA.