Vasculitis in Adults Testing Algorithm

Vasculitis in Adults Testing Click here for topics associated with this algorithm

Abbreviations INDICATIONS FOR TESTING Unexplained systemic illness with organ involvement including ≥1 of the ANA Anti-Nuclear Antibody following ANCA Anti-Neutrophil Cytoplasmic Antibodies • Upper airway (chronic rhinosinusitis) + Keratitis CRP C-Reactive Protein • Renal (microscopic hematuria or glomerulonephritis) + Vestibuloauditory signs and symptoms MPO Antimyeloperoxidase • Pulmonary (pulmonary infiltrates, dyspnea, cough, hemoptysis) PR3 Antiproteinase 3 • Dermatologic (palpable purpura, urticaria) UA Urinalysis • Neurologic (mononeuritis multiplex) Angiography demonstrates aortitis Must rule out infection, neoplasm, drug effect

NOTE: Classification is primarily based on Cogan syndrome predominant vessels involved.

Confirms presence of ANA + Connective tissue disease/rheumatic Recurrent oral ulceration plus 2 of the or disease following: HIV + To determine systems involved, or • Genital ulceration or ORDER UA - HIV • Eye lesions Treponema • UA CRP + or • Skin lesions pallidum + • CRP ANCA - • Pathergy test positive Syphilis • Urea nitrogen/creatinine Pulmonary • ANCA symptoms - • ANA HIV - • Syphilis screening Treponema Behçet syndrome UA +/- • HIV testing pallidum - CRP + ORDER • CBC with platelet count and ANA - Biopsy of involved site differential + Blood pressure differential between upper extremities + Hypertension, young female ORDER Positive Negative Biopsy of involved site UA - UA + Angiography demonstrates aneurysmal CRP - CRP + formation, irregular vessel walls, and Secondary Primary disease occlusion in aorta vasculitis without vasculitis

Takayasu arteritis ORDER (aortic arch syndrome) Vasculitis demonstrated Biopsy of involved site

No vasculitis + Headache demonstrated +/- Jaw claudication Vasculitis likely +/- Vision symptoms associated with other + Painful temporal artery to palpation disease diagnosis +/- Polymyalgia rheumatica Consider other etiologies for > 50 yrs old systemic illness (other infection, autoimmune) Biopsy of temporal artery demonstrates medium and large artery vasculitis ANCA - ANCA + with giant cell formation

Giant cell arteritis (Temporal arteritis) +/- Pulmonary signs - Pulmonary signs and and symptoms symptoms No palpable purpura Palpable purpura present

ORDER ORDER Skin biopsy of involved area Antimyeloperoxidase Antiproteinase 3 Glomerular Basement predominates (MPO) predominates Membrane Antibody, IgG by (PR3) Multiplex Bead Assay and IFA Demonstrates Demonstrates inflammatory IgA+ infiltrates and immunoglobulin and C3 complement deposition Negative Positive deposition • Pulmonary signs and symptoms, including a Pulmonary and No pulmonary history of asthma upper airway IgA vasculitis ORDER signs/symptoms If strong Renal biopsy required to • Mononeuritis multiplex signs/symptoms (Henoch Schőnlein Cryoglobulin, suspicion confirm antiglomerular • Eosinophilia purpura) Qualitative exists, order basement membrane disease a biopsy (Goodpasture syndrome)

ORDER ORDER ORDER Cryoglobulins present Biopsy of involved site Biopsy of involved site Biopsy of involved site Severe widespread Demonstrates crescentic small and medium glomerulonephritis or Vasculitis present on biopsy necrotizing linear IgG deposits in vasculitis Demonstrates Demonstrates the GBM Demonstrates small and medium artery, small artery and No Yes small artery, capillary and venule vein necrotizing capillary and vasculitis, with vasculitis with granulomatous reactions venule vasculitis Polyarteritis nodosa Goodpasture Rule out underlying Cryoglobulinemic granulomas a and eosinophilia (PAN) syndrome diseases such as hepatitis vasculitis B or C, SLE, Sjögren

Eosinophilic Granulomatosis Microscopic Negative Positive granulomatosis with with polyangiitis polyangiitis polyangiitis (formerly (formerly Churg-Strauss Wegener aUp to 30% of patients with Goodpasture syndrome Essential Cryoglobulinemia secondary syndrome) granulomatosis) may also be ANCA+ cryoglobulinemia to underlying disease