Clinical Trials

Inclusion Criteria

• For Giant Cell Arteritis
  • age at disease onset >50 years (required)
  • New onset or new type of localized pain in the head
  • Temporal artery abnormality (i.e. temporal artery tenderness to palpation or decreased pulsation, unrelated to arteriosclerosis of cervical arteries)
  • ESR of >40mm in the first hour by the Westergren method
  • Abnormal artery biopsy (i.e. temporal artery biopsy showing vasculitis characterized by a predominance of mononuclear cell infiltration or granulomatous inflammation, usually with multinucleated giant cells)
  • Large Vessel Vasculitis (LVV) by angiogram or biopsy not explained by something else
• For Takayasu's Arteritis
  • Age at disease onset <50 years
  • Claudication of extremities
  • Decreased brachial artery pulse (one or both arteries)
  • Blood pressure difference of >10mm Hg between the arms
  • Bruit over subclavian arteries or aorta
  • Arteriogram abnormalities compatible with TAK (includes conventional dye angiography or MR angiography or CT angiography)
• For Polyarteritis Nodosa Major (not explained by other causes, and felt by investigator to be due to vasculitis)
  • 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, and felt by investigator to be due to vasculitis)
  • Weight loss > 4 kg
  • Livedo reticularis, cutaneous ulcerations, or skin nodules
  • Testicular pain or tenderness
  • Myalgias
  • Diastolic blood pressure > 90 mm Hg
  • Elevated BUN or serum creatinine levels
  • Ischemic abdominal pain
• Isolated cutaneous Polyarteritis Nodosa 
  • Biopsy-proven cutaneous PAN
• For Granulomatosis with Polyangiitis (Wegener's) (GPA) and Microscopic Polyangitis (MPA)(Widely accepted diagnostic criteria, as opposed to classification criteria or definitions, have not been developed for GPA & MPA).
  • For diagnosis of MPA, meets the Chapel Hill Consensus Conference Definition for MPA:
    • Necrotizing vasculitis, with few or no immune deposits, that affects small vessels (i.e., capillaries, venules, arterioles)
    • Necrotizing arteritis involving small- and medium-sized arteries may be present
    • Necrotizing glomerulonephritis is very common
    • Pulmonary capillaritis often occurs
    • Asthma
    • Peak peripheral blood eosinophilia of >10% of total WBC
    • Peripheral neuropathy attributable to vasculitis
    • Transient pulmonary infiltrates on chest imaging studies
    • Paranasal sinus abnormalities or nasal polyposis
    • Eosinophilic inflammation on tissue biopsy
  • For diagnosis of GPA meets at least 2 of the following 5 modified ACR criteria:
    • Nasal or oral inflammation with oral ulcers or nasal discharge with pus or blood
    • Abnormal chest radiograph with nodules, fixed infiltrates, or cavities
    • Urinary sediment with microhematuria or red cell casts
    • Granulomatous inflammation within the wall of an artery or in the perivascular area on biopsy
    • Antineutrophil cytoplasmic antibody (ANCA) positive by enzyme immunoassay for either PR3- or MPO-ANCA
  • For Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)
  • If patients have 4 of the above 6 criteria but lack clearcut documentation of small vessel vasculitis, they are also eligible for enrollment.

Exclusion Criteria

• Inability to give informed consent and to sign the consent form
• Enrolled in VCRC protocols 5502, 5503, 5504, 5505, 5506, 5522, or 5523
• Unwilling to provide blood for DNA collection