Patient case (clinical description): A 61-year-old man comes to the emergency department complaining of an acute vision disturbance. He had an episode of vision disturbance in the right eye that occurred suddenly and resolved spontaneously in 15 minutes. He also has right jaw pain while chewing.  He also complains of fatigue and hip muscle aches over the last several months.  The patient has a history of mild hyperlipidemia that has been controlled by diet and lifestyle modifications.  On examination, his blood pressure is 130/70 mm Hg and pulse is 66/min.  Neurological examination is unremarkable.  Visual examination is also normal. ESR is 103 mm/h. Temporal artery biopsy shows multinuclear giant cells and internal elastic membrane fragmentation.

Patient's own account: I’m a 61-year-old man and I came to the ER because I had a sudden episode where I couldn’t see out of my right eye. It only lasted about 15 minutes and went away on its own but it really scared me. I’ve also been having pain in my right jaw when I chew. For the past few months I’ve felt really tired and noticed my hips ache a lot. I have a history of mild cholesterol issues but I’ve been managing that with my diet and lifestyle. When the doctors checked me my blood pressure and pulse were normal and my vision and neurological exams didn’t show anything wrong. However they ran some tests and said my ESR levels were very high and a biopsy of my temporal artery showed some inflammation and damage.

Clinical trial: One-Time DNA Study for Vasculitis

Eligibility criteria:
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

        Inclusion Criteria:

        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)

        Inclusion Criteria:

        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

        Inclusion Criteria:

        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

                  Inclusion Criteria:

                  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.

                  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
No condition on gender to be admitted to the trial.
No healthy subjects accepted to join the trial.
Subject must be at least 7 Years old.


Eligibility (NLI label): Entailment