One-Time DNA Study for Vasculitis

Inclusion Criteria47

• \. Diagnostic 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
• \. Diagnostic criteria 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)
• \. Diagnostic criteria for Polyarteritis Nodosa Major criteria (not explained by other causes) 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) 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 1. Biopsy-proven cutaneous PAN
• \. 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:
• 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 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
• \. Diagnostic criteria for Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)
• 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
• If patients have 4 of the above 6 criteria but lack clearcut documentation of small vessel vasculitis, they are also eligible for enrollment.