One-Time DNA Study for Vasculitis

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 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)
• •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.