Signs and Symptoms
- Systemic complaints are common early in the presentation of vasculitis, before vascular-related complications occur:
- Fever, fatigue, weight loss, diffuse aches and pains
- Signs of arterial insufficiency:
- Ischemic pain:
- Neurologic ischemia:
- Headache, TIA, stroke, visual and sensorineural hearing loss, hallucinations, neuropathy, vision loss
- Renal ischemia:
- Dermatologic ischemia:
- Classic skin findings include palpable purpura
- Nodular lesions, ulcers, livedo reticularis, and digital ischemia may also be seen
- Oligoarthritis
- Ocular ischemia:
- Diplopia, retinal hemorrhages, scleritis, and episcleritis
- Respiratory tract:
- Sinusitis, epistaxis, nasal and oral ulcerations, strawberry tongue
- GI ischemia:
- Hematochezia, melena, hematemesis, peritonitis, hepatitis
- Cardiac:
- Coronary artery aneurysms, myocarditis, pericarditis, valvular disease, CHF
- Mucosal: Strawberry gingivitis, oral ulcers
History
- Suspect vasculitis with general systems and signs of arterial insufficiency:
- Claudication, angina, abdominal angina, or TIA, in a young patient
- Prolonged systemic illness with multiorgan dysfunction
- History of glomerulonephritis, peripheral neuropathy, or autoimmune disease
- Diagnostic clues to the etiology:
- Age, gender, ethnicity, travel history
- Specific complaints that suggest the size of the involved vessel and organs
- Recent infections
- Connective tissue disorders
- Medications that may cause vasculitis:
Physical Exam
Classify vasculitis:
- Large arteries:
- Diminished pulses and bruits over several large arteries
- BP discrepancy >10 mm Hg between left and right limbs
- Pulse discrepancy >30 mm Hg between the left and right limbs
- Cool extremities due to claudication and ulceration
- Medium and small arteries:
- Palpable purpura (nodules, ulcers, livedo papules)
- Skin ulcers
- Digital ischemia
Essential Workup
- History and physical exam:
- Chewing gum test for jaw claudication
- CBC, ESR, CRP, urinalysis, BUN, creatinine
Diagnostic Tests & Interpretation
Lab
- CBC:
- Leukocytosis
- Eosinophilia
- Anemia
- Creatinine
- LFT
- CRP
- ESR
- ANA
- ANCA
- Complement
- CPK
- Urinalysis:
- Proteinuria and hematuria
Imaging
- CXR:
- PAN usually has a nonspecific patchy alveolar infiltration
- CT scan:
- Sinus CT for suspected granulomatosis with polyangiitis (Wegener)
- CTA:
- Coronary artery aneurysms in Kawasaki
- Echocardiography:
- Coronary artery aneurysms in Kawasaki
- MRI and MRA:
- Positron emission tomography (PET) scan for suspected Takayasu and Kawasaki
- ECG:
- Indications: Suspected Takayasu and Kawasaki
- US:
- Temporal artery US for suspected giant cell arteritis
- Use pretest probability in interpretation of results
- Arteriography
Diagnostic Procedures/Surgery
- ECG:
- Pericarditis, conduction disturbances
- Endoscopy, sigmoidoscopy, and colonoscopy for GI tract involvement:
Differential Diagnosis
- Endocarditis
- Adverse drug reaction
- Viral infections (e.g., enterovirus)
- Scarlet fever
- Staphylococcal scalded-skin syndrome
- Toxic shock syndrome
- Stevens-Johnson syndrome
- Rocky Mountain spotted fever
- Leptospirosis
- Antiphospholipid antibody syndrome
- Disseminated intravascular coagulation
- Cholesterol emboli
- Calciphylaxis
Initial Stabilization/Therapy
Stabilization of cerebrovascular complications
ED Treatment/Procedures
- Treatment for vasculitis is determined by the underlying cause or the specific disease and is best initiated by rheumatology
- Kawasaki: Aspirin, IVIG
- Giant cell arteritis: Corticosteroids, tocilizumab
- Eosinophilic granulomatosis with polyangiitis: Mepolizumab
- Behçet syndrome: Apremilast
- ANCA-associated vasculitis: Rituximab
- PAN: Steroids, cyclophosphamide
- Takayasu arteritis: Corticosteroids, methotrexate, azathioprine, cyclophosphamide
- Wegner granulomatosis:
- Corticosteroids
- Cyclophosphamide,
- Azathioprine
- Plasma exchange for severe disease
Medication
- Azathioprine: 2 mg/kg/d PO
- Cyclophosphamide:
- IV: 0.5-1 mg/m2 body surface area
- PO: 2 mg/kg/d (up to 4 mg/kg) (peds: dose as per consultant)
- IVIG: 1-2 g/kg IV
- Methylprednisolone: 0.25-1 mg/d IV
- Methotrexate: 7.5-15 mg/wk PO
- Prednisolone: 1 mg/kg/d PO
- Prednisone: 40-60 mg/d (peds: 1-2 mg/kg/d) PO
Disposition
Admission Criteria
- Patients with evidence of severe disease and end-organ dysfunction should be admitted
- Consult for procedures to revascularize ischemic organs
Discharge Criteria
Less-symptomatic patients without evidence of end-organ involvement
Issues for Referral
- Any patient suspected of vasculitis and being managed as an outpatient should be referred as soon as possible to a rheumatologist for definitive diagnosis and treatment
- Consult appropriate specialties based on the severity of end-organ damage
Follow-up Recommendations
Stress the need for close follow-up with general symptoms to confirm the diagnosis and initiate therapy that will be life-saving on a long-term basis
- ButtgereitF, DejacoC, MattesonEL, et al. Polymyalgia rheumatica and giant cell arteritis: A systematic review . JAMA. 2016;315(22):2442-2458.
- McCrindleBW, RowleyAH, NewburgerJW, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: A scientific statement for health professionals from the American Heart Association . Circulation. 2017;135(17):e927-e999.
- OzenS. The changing face of polyarteritis nodosa and necrotizing vasculitis . Nat Rev Rheumatol. 2017;13(6):381-386.
- Weyand CM, GoronzyJJ. Clinical practice. Giant-cell arteritis and polymyalgia rheumatica . N Engl J Med. 2014;371(1):50-57.
- YatesM, WattsRA, BajemaIM, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis . Ann Rheum Dis. 2016;75(9):1583-1594.
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