An inflammatory disorder of blood vessels
Highly variable, depending on the particular syndrome
Annual incidence in adults (unless otherwise specified)
A combination of genetic susceptibility and environmental exposure likely triggers onset.
Early identification is the key to prevent irreversible organ damage in severe forms of systemic vasculitis.
Hepatitis C (cryoglobulinemic vasculitis), hepatitis B (PAN), cytomegalovirus (CMV), Epstein-Barr virus (EBV), HIV (viral-/retroviral-associated vasculitis), SLE, rheumatoid arthritis (RA), Sjögren syndrome, mixed connective tissue disease (MCTD), dermatomyositis, ankylosing spondylitis, Behçet disease, relapsing polychondritis (CTD-associated vasculitis), respiratory tract methicillin-resistant Staphylococcus aureus (MRSA) in GPA, levamisole-adulterated cocaine, medications: propylthiouracil, methimazole, hydralazine, minocycline, levamisole-tainted cocaine
Renal involvement is often clinically silent. Routine serum creatinine and urinalysis with microscopy are needed to identify underlying glomerulonephritis.
Blood vessel biopsy shows immune cell infiltration into vessel wall layers with varying degrees of necrosis and granuloma formation, depending on the type.
Corticosteroids are initial anti-inflammatory of choice.
Cytotoxic medications, immunomodulatory, or biologic agents (e.g., cyclophosphamide (1)[B],(2)[A], methotrexate (3)[A], azathioprine (3)[A], leflunomide (3)[A], mycophenolate mofetil (1)[B], and rituximab (2)[A]) are often required in combination with corticosteroids for rapidly progressive vasculitis with significant organ involvement or inadequate response to corticosteroids. Rituximab (2)[A] is the first FDA-approved treatment for GPA and MPA. Tocilizumab (4)[A] is the first FDA-approved treatment for GCA. Mepolizumab (5)[A] is the first FDA-approved treatment for EGPA.
Rarely, corrective surgery is required to repair tissue damage as a result of aggressive vasculitis.
If significant coronary artery disease is involved in KD, moderate activity restriction may be of benefit.
Frequent clinical follow-up supported by patient self-monitoring to identify disease relapse
Alter diets for patients with renal involvement or hyperglycemia/dyslipidemia.
Prognosis is good for patients with vasculitis and limited organ involvement. Relapsing courses, renal, intestinal, or extensive lung involvement have a poorer prognosis.
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