Rosen & Barkin's 5-Minute Emergency Medicine Consult (759 page)

• 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:
    
    • Levsamisole (as a cocaine adulterant), phenytoin, carbamazepine, isoniazid, methimazole, minocycline, penicillamine, propylthiouracil, sulfasalazine
      

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
    

• History and physical exam
  
• CBC, ESR, CRP, urinalysis, BUN, creatinine
  

• CBC:
  
  • Leukocytosis
    
  • Eosinophilia
    
  • Anemia
    
• Creatinine
  
• LFT
  
• CRP
  
• ESR
  
• ANA
  
• ANCA
  
• Complement
  
• CPK
  
• Urinalysis:
  
  • Proteinuria and hematuria
    

• 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
  

• EKG:
  
  • Pericarditis, conduction disturbances
    
• Endoscopy, sigmoidoscopy, and colonoscopy for GI tract involvement
  
• Tissue biopsy
  

• 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
  

TREATMENT

Stabilization of cerebrovascular complications

• 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
  
• PAN: Steroids, cyclophosphamide
  
• Takayasu arteritis: Corticosteroids, methotrexate, azathioprine, cyclophosphamide
  
• Wegner granulomatosis: Corticosteroids:
  
  • Cyclophosphamide, azathioprine may be substituted
    
  • Plasma exchange may be helpful in severe disease.
    

• Azathioprine: 2 mg/kg/d PO
  
• Cyclophosphamide:
  
  • IV: 0.5–1 mg/m
    ²
    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
  

FOLLOW-UP

• Patients with evidence of severe disease and end-organ dysfunction should be admitted.
  
• Consult for procedures to revascularize ischemic organs.
  

Less-symptomatic patients without evidence of end-organ involvement

• Any patient suspected of vasculitis and being managed as an outpatient should be referred as soon as possible to a rheumatologist for the definitive diagnosis and treatment.
  
• Consult appropriate specialties based on the severity of the end-organ damage.
  

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.

• Drug therapy may be toxic; do not prescribe without specialist consultation.
  
• Patients may be immunosuppressed and at risk for opportunistic pathogens.
  
• Do not miss subacute bacterial endocarditis as a mimic of vasculitis.
  
• Temporal (giant cell) arteritis does not occur before age 50 yr.
  
• Nodular lesions are the skin changes most likely to yield a diagnosis of vasculitis.
  

• Langford CA. Vasculitis.
  J Allerg Clin Immunol.
  2010;125(2 suppl 2):S216–S225.
  
• Lapraik C, Watts R, Bacon P, et al. BSR and BHPR guidelines for the management of adults with ANCA associated vasculitis.
  Rheumatology (Oxford).
  2007;46(10):1615–1616.
  
• Mukhtyar C, Guillevin L, Cid MC, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis.
  Ann Rheum Dis.
  2009;68(3):310–317.
  
• Newburger JW, Takahashi M, Gerber MA, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: A statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association.
  Pediatrics.
  2004;114(6):1708–1733.
  
• Semple D, Keogh J, Forni L, et al. Clinical review: Vasculitis on the intensive care unit-part 1: Diagnosis.
  Crit Care
  . 2005;9(1):92–97.
  
• Semple D, Keogh J, Forni L, et al. Clinical review: Vasculitis on the intensive care unit-part 2: Treatment and prognosis.
  Crit Care
  . 2005;9(2):193–197.
  

See Also (Topic, Algorithm, Electronic Media Element)

• Erythema Nodosum
  
• Henoch–Schönlein Purpura
  
• Hepatitis
  
• Reiter Syndrome
  
• Systemic Lupus Erythematosus
  

CODES

• 446.0 Polyarteritis nodosa
  
• 446.5 Giant cell arteritis
  
• 447.6 Arteritis, unspecified
  

BASICS

• Inadequacy of the venous valves that causes impaired venous drainage leading to edema of the lower extremities.
  
• A chronic condition of lower extremity vascular incompetence.
  
• Normal blood flow in the venous system is unidirectional from the superficial veins to the deep veins.
  
• Unidirectional flow is maintained by contraction of leg muscles and by valves within the veins.
  
• Damage to the valves, e.g. following DVT, causes them to become rigid and they lose their ability to prevent retrograde blood flow properly.
  
• Decreased venous return from lower extremities causes increased pressure and distention of the veins, which in turn causes separation of the valve leaflets.
  
• Increased pressure transmitted into the dermal microcirculation results in extravasation of macromolecules and red blood cells causing inflammatory injury resulting in ulcer formation, skin changes, and poor ulcer healing.