para />
Renal involvement is often clinically silent. Routine serum creatinine and urinalysis with microscopy are needed to identify underlying glomerulonephritis.
Initial tests to exclude alternate diagnosis and guide appropriate therapy
Routine tests
Specific serology
Antinuclear antibodies (ANA)
Rheumatoid factor (RF)
Rapid plasma reagin/venereal disease reaction level (RPR/VDRL)
RMSF titer
Lyme titer
Complement levels C3, C4
ANCA
Antiproteinase 3 antibodies (anti-PR3)
Antimyeloperoxidase antibodies (anti-MPO)
Hepatitis screen for B and C
Cryoglobulin
Anti-GBM titer
HIV
Serum and urine protein electrophoresis
Miscellaneous
Drug screen
ESR
C-reactive protein
Creatine kinase (CK)
Blood culture
ECG
CXR, CT scan, MRI, and arteriography may be required to delineate extent of organs involved.
Diagnostic Procedures/Other
- Electromyography with nerve conduction can document neuropathy and target nerve for biopsy.
- Biopsy of affected site confirms diagnosis (e.g., temporal artery, sural nerve, renal biopsy).
- If biopsy is not practical, angiography may be diagnostic for large- and medium-vessel vasculitides.
- Bronchoscopy may be required to differentiate pulmonary infection from potentially life-threatening hemorrhagic vasculitis in patients with hemoptysis.
Test Interpretation
Blood vessel biopsy shows immune cell infiltration into vessel wall layers with varying degrees of necrosis and granuloma formation, depending on the type of vasculitis
TREATMENT
GENERAL MEASURES
- Discontinue offending drug (hypersensitivity vasculitis)
- Simple observation for mild cases of IgA vasculitis
- ANCA-associated vasculitis has 2-phase treatment: initial induction followed by maintenance (steady tapering of corticosteroids with immunosuppressants or immunomodulators)
MEDICATION
First Line
Corticosteroids are initial anti-inflammatory of choice.
Second Line
Cytotoxic medications, immunomodulatory, or biologic agents (e.g., cyclophosphamide (2)[B],(3,5)[A] methotrexate (4,5)[A], azathioprine (4,5)[A], leflunomide (5)[A], mycophenolate mofetil (2)[B],(5)[A], and rituximab (3,5)[A], are often required in combination with corticosteroids for rapidly progressive vasculitis with significant organ involvement or inadequate response to corticosteroids.
ISSUES FOR REFERRAL
- Rheumatology referral for complicated cases where newer or more toxic treatments are required
- Nephrology referral for persistent hematuria or proteinuria, rising creatinine, or a positive ANCA titer
- Pulmonary referral for persistent pulmonary infiltrate unresponsive to antibiotic therapy or if gross hemoptysis
ADDITIONAL THERAPIES
- IVIG and aspirin for KD, but corticosteroids are contraindicated.
- Plasma exchange appears to improve recovery of patients with severe acute renal failure secondary to vasculitis and pulmonary hemorrhage (5)[A].
SURGERY/OTHER PROCEDURES
Rarely, corrective surgery is required to repair tissue damage as a result of aggressive vasculitis.
INPATIENT CONSIDERATIONS
Admission Criteria/Initial Stabilization
- Hemoptysis, acute renal failure, intestinal ischemia, any organ-threatening symptoms or signs, and/or need for biopsy
- Initial therapy is guided by the organ system involved.
- If pulmonary hemorrhage is present, life-saving measures may include mechanical ventilation, plasmapheresis, and immunosuppression.
- If acute renal failure is present, attend to electrolyte and fluid balance and consider plasma exchange and immunosuppression.
- If signs of intestinal ischemia are present, make NPO and consider plasmapheresis, immunosuppression, and parenteral nutrition.
Discharge Criteria
Stabilization or resolution of potential life-threatening symptoms
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
If significant coronary artery disease is involved in KD, moderate activity restriction may be of benefit.
Patient Monitoring
Frequent clinical follow-up supported by patient self-monitoring to identify disease relapse
DIET
Alter diets for patients with renal involvement or hyperglycemia/dyslipidemia
PROGNOSIS
Prognosis is good for patients with vasculitis and limited organ involvement. Relapsing courses, renal, intestinal, or extensive lung involvement have a poorer prognosis.
COMPLICATIONS
- Persistent organ dysfunction may be the result of the disease, medications, or inflammation/scarring in the more serious forms of vasculitis.
- Early morbidity/mortality is due to active vasculitic disease; delayed morbidity/mortality may also be secondary to complications of chronic therapy with cytotoxic medications.
REFERENCES
11 Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Arthritis Rheum. 2013;65(1):1 " 11.22 Appel GB, Contreras G, Dooley MA, et al. Mycophenolate mofetil versus cyclophosphamide for induction treatment of lupus nephritis. J Am Soc Nephrol. 2009;20(5):1103 " 1112.33 Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010;363(3):221 " 232.44 Walters GD, Willis NS, Craig JC. Interventions for renal vasculitis in adults. A systematic review. BMC Nephrol. 2010;11:12.55 Bosch X, Guilabert A, Espinosa G, et al. Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review. JAMA. 2007;298(6):655 " 669.
ADDITIONAL READING
- Bertsias G, Ioannidis JP, Boletis J, et al. EULAR recommendations for the management of systemic lupus erythematosus. Report of a Task Force of the EULAR Standing Committee for International Clinical Studies Including Therapeutics. Ann Rheum Dis. 2008;67(2):195 " 205.
- Gatto M, Iaccarino L, Canova M, et al. Pregnancy and vasculitis: a systematic review of the literature. Autoimmun Rev. 2012;11(6 " 7):A447 " A459.
- Lee YH, Choi SJ, Ji JD, et al. Associations between the angiotensin-converting enzyme insertion/deletion polymorphism and susceptibility to vasculitis: a meta-analysis. J Renin Angiotensin Aldosterone Syst. 2012;13(1):196 " 201.
- National Heart Lung and Blood Institute Diseases and Conditions Index: Vasculitis. www.nhlbi.nih.gov/health/dci/Diseases/vas/vas_whatis.html
- Vasculitis Foundation: www.vasculitisfoundation.org/
CODES
ICD10
- I77.6 Arteritis, unspecified
- M31.30 Wegener 's granulomatosis without renal involvement
- M30.0 Polyarteritis nodosa
- M31.6 Other giant cell arteritis
- M31.5 Giant cell arteritis with polymyalgia rheumatica
- M31.4 Aortic arch syndrome [Takayasu]
- M31.31 Wegener 's granulomatosis with renal involvement
- M30.3 Mucocutaneous lymph node syndrome [Kawasaki]
- M30.1 Polyarteritis with lung involvement [Churg-Strauss]
- D89.1 Cryoglobulinemia
- D69.0 Allergic purpura
- M31.0 Hypersensitivity angiitis
ICD9
- 447.6 Arteritis, unspecified
- 446.4 Wegener 's granulomatosis
- 446.0 Polyarteritis nodosa
- 446.5 Giant cell arteritis
- 287.0 Allergic purpura
- 273.2 Other paraproteinemias
- 446.7 Takayasu 's disease
- 446.20 Hypersensitivity angiitis, unspecified
- 446.1 Acute febrile mucocutaneous lymph node syndrome [MCLS]
SNOMED
- 31996006 Vasculitis (disorder)
- 195353004 Wegener 's granulomatosis (disorder)
- 155441006 Polyarteritis nodosa (disorder)
- 414341000 giant cell arteritis (disorder)
- 82275008 Allergic granulomatosis angiitis (disorder)
- 75053002 Acute febrile mucocutaneous lymph node syndrome (disorder)
- 359789008 Takayasu 's disease (disorder)
- 191306005 Henoch-Schonlein purpura (disorder)
- 190815001 Cryoglobulinemic vasculitis
- 60555002 Hypersensitivity angiitis (disorder)
CLINICAL PEARLS
- Suspect vasculitis in patients with a petechial rash, palpable purpura, glomerulonephritis, pulmonary-renal syndrome, intestinal ischemia, or mononeuritis multiplex.
- Exclude silent renal involvement by routinely obtaining serum creatinine and urinalysis with microscopy.
- Vasculitis has "skip " lesions, which may complicate diagnostic biopsy.
- In patients with vasculitis, look for a primary underlying process such as infection, thrombosis, or malignancy.