One-Time DNA Study for Vasculitis

Individuals with giant cell arteritis, Takayasu's arteritis, polyarteritis nodosa, granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis, and Churg-Strauss syndrome. Enrollment will be sequential and patients will have disease in various stages and of different duration.

Inclusion Criteria:

1. Diagnostic criteria for Giant Cell Arteritis Age at disease onset >50 years (required)

1. New onset or new type of localized pain in the head
2. Temporal artery abnormality (i.e. temporal artery tenderness to palpation or decreased pulsation, unrelated to arteriosclerosis of cervical arteries)
3. ESR of >40mm in the first hour by the Westergren method
4. 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)
5. Large Vessel Vasculitis (LVV) by angiogram or biopsy not explained by something else

2. Diagnostic criteria for Takayasu's Arteritis

1. Age at disease onset <50 years
2. Claudication of extremities
3. Decreased brachial artery pulse (one or both arteries)
4. Blood pressure difference of >10mm Hg between the arms
5. Bruit over subclavian arteries or aorta
6. Arteriogram abnormalities compatible with TAK (includes conventional dye angiography or MR angiography or CT angiography)

3. Diagnostic criteria for Polyarteritis Nodosa Major criteria (not explained by other causes) felt by investigator to be due to vasculitis

1. Arteriographic abnormality
2. Presence of granulocyte or mixed leukocyte infiltrate in an arterial wall on biopsy
3. Mononeuropathy or polyneuropathy

Minor criteria (not explained by other causes) felt by investigator to be due to vasculitis

1. Weight loss > 4 kg
2. Livedo reticularis, cutaneous ulcerations, or skin nodules
3. Testicular pain or tenderness
4. Myalgias
5. Diastolic blood pressure > 90 mm Hg
6. Elevated BUN or serum creatinine levels
7. Ischemic abdominal pain

Isolated cutaneous Polyarteritis Nodosa 1. Biopsy-proven cutaneous PAN

4. Diagnostic criteria for Granulomatosis with Polyangiitis (Wegener's) (GPA) and Microscopic Polyangitis (MPA)

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

• For diagnosis of GPA meets at least 2 of the following 5 modified 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

     5. Diagnostic criteria for Churg-Strauss Syndrome

     1. Asthma
     2. Peak peripheral blood eosinophilia of >10% of total WBC
     3. Peripheral neuropathy attributable to vasculitis
     4. Transient pulmonary infiltrates on chest imaging studies
     5. Paranasal sinus abnormalities or nasal polyposis
     6. Eosinophilic inflammation on tissue biopsy

     If patients have 4 of the above 6 criteria but lack clearcut documentation of small vessel vasculitis, they are also eligible for enrollment.

     General 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