Vasculitis, Emergency Medicine

Basics

Description

Injury to the walls of blood vessels from inflammation:
- Ischemia and necrosis
- Aneurysms and hemorrhage
- Immunopathologic mechanisms:
  - Deposition of circulating antigen " “antibody complex and complement fixation
  - Cell-mediated hypersensitivity
  - Granulomatous tissue reaction from persistent inflammation and formation of epithelioid giant cells
The vasculitides represent a wide group of disorders:
- Multisystem disease with constitutional symptoms and inflammatory lab indices
- Secondary to another disorder or trigger, or primary if vasculitis is the principal feature and the cause is unknown
- Multiple factors determine presentation:
  - The size of the affected blood vessels
  - The specific distribution, severity, and duration of the inflammation
  - Degree of permeability or occlusion of the affected vessels
1 out of 2,000 adults has some form of vasculitis

Etiology

Classification is evolving and is increasingly based on presence or absence of antineutrophil cytoplasmic antibodies (ANCA).
Traditional classification is based on vessel size.
Large vessel vasculitides:
- Temporal (giant cell) arteritis:
  - Granulomatous arteritis of the aorta and its major branches often involving the temporal artery
  - Patients >50 yr
- Takayasu arteritis:
  - Granulomatous inflammation of the aorta and its major branches
  - Usually occurs in patients <50 yr
Medium vessel vasculitides:
- Polyarteritis nodosa (PAN):
  - Small- and medium-sized arteritis
  - Common distribution includes vessels supplying the muscles, joints, intestines, nerves, kidneys, and skin
  - Most common in middle age
- Kawasaki disease (mucocutaneous lymph node syndrome):
  - Coronary arteries are often involved and involves large-, medium-, and small-sized arteries
  - Usually occurs in children
- Isolated CNS vasculitis
Small vessel vasculitides:
- Granulomatosis with polyangiitis (Wegener granulomatosis):
  - Necrotizing vasculitis affecting small- to medium-sized vessels
  - Granulomatous inflammation of the upper and lower respiratory tract and glomerulonephritis
- Microscopic polyangiitis:
  - Necrotizing affecting small vessels
  - Glomerulonephritis is very common.
  - Pulmonary capillaritis often occurs.
- Churg " “Strauss syndrome (allergic granulomatosis):
  - Small- and medium-sized arteries
  - Mainly lungs, GI, and nerves
  - Can also involve heart, skin, and kidney
- Henoch " “Sch ƒ ¶nlein purpura:
  - Most patients <20 yr
- Buerger disease (thromboangiitis obliterans):
- Hypersensitivity vasculitis:
  - Recurring inflammation and thrombosis of small and medium arteries and veins of the hands and feet
  - Typically between 20 and 40 yr and male
- Secondary vasculitides:
  - Bacterial infections:
    - Streptococcal, tuberculous, staphylococcal, Lyme disease, leprosy
  - Viral infections:
    - Hepatitis B or C, CMV, HSV, HIV
  - Rickettsial infections
  - Drug related
  - Connective tissue disease:
    - Systemic lupus erythematosus
    - Rheumatoid arthritis
    - Beh ƒ §et disease
  - Malignancy:
    - Hairy cell leukemia
    - Lymphoma
  - Mixed cryoglobulinemia
  - Goodpasture syndrome
  - Serum sickness

Diagnosis

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:
  - Angina, abdominal angina, claudication, jaw claudication
- Neurologic ischemia:
  - Headache, TIA, stroke, visual and sensorineural hearing loss, hallucinations, neuropathy, vision loss
- Renal ischemia:
  - Severe or resistant HTN
- 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

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

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
CBC, ESR, CRP, urinalysis, BUN, creatinine

Diagnosis 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

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

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

Treatment

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
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.

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

Follow-Up

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 the definitive diagnosis and treatment.
Consult appropriate specialties based on the severity of the end-organ damage.

Followup 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. ‚

Pearls and Pitfalls

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.

Additional Reading

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.

Codes

ICD9

446.0 Polyarteritis nodosa
446.5 Giant cell arteritis
447.6 Arteritis, unspecified
443.1 Thromboangiitis obliterans [Buergers disease]

ICD10

I77.6 Arteritis, unspecified
M30.0 Polyarteritis nodosa
M31.6 Other giant cell arteritis
I73.1 Thromboangiitis obliterans [Buergers disease]
M31.4 Aortic arch syndrome [Takayasu]

SNOMED

31996006 Vasculitis (disorder)
400130008 Temporal arteritis (disorder)
155441006 Polyarteritis nodosa (disorder)
52403007 Thromboangiitis obliterans (disorder)
11791001 Necrotizing vasculitis (disorder)
359789008 Takayasus disease (disorder)
70933002 Aortitis (disorder)

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