Vasculitis in Adults Testing

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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 + Recurrent oral ulceration plus 2 of the
Connective tissue disease/rheumatic
or following:
disease To determine systems involved,
or
HIV + • Genital ulceration
or ORDER UA - • Eye lesions
HIV • UA CRP +
or
Treponema • 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 -
ORDER
CRP + • 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
predominates (MPO)
Membrane Antibody, IgG by (PR3)
Multiplex Bead Assay and IFA Demonstrates Demonstrates inflammatory
IgA+ infiltrates and
C3 immunoglobulin and
deposition complement deposition
Negative Positive • 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
basement membrane disease purpura) Qualitative
exists, order
biopsy (Goodpasture syndrome) a

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 small and medium artery, Demonstrates
No Yes small artery and
capillary and venule small artery,
vein necrotizing
vasculitis, with capillary and
vasculitis with
Rule out underlying granulomatous reactions venule vasculitis
Polyarteritis nodosa Goodpasture Cryoglobulinemic granulomas
(PAN) syndromea diseases such as hepatitis vasculitis
and eosinophilia
B or C, SLE, Sjögren

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

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