Treatment of rheumatoid vasculitis
- Patrick Whelan, MD, PhD
Patrick Whelan, MD, PhD
- Associate in Rheumatology (Pediatrics)
- Massachusetts General Hospital for Children
The term “rheumatoid vasculitis” (RV) refers specifically to a protean, inflammatory process in patients with rheumatoid arthritis (RA) that is centered on the blood vessel wall itself. Although RV has a range of disease severity, the condition may be associated with substantial potential morbidity, may require intensive immunosuppressive therapy, and may lead to a significantly higher mortality than does RA itself. The disease typically occurs in patients with longstanding, erosive RA. RV may affect a wide range of blood vessel types, from medium-sized muscular arteries to somewhat smaller arterioles or post-capillary venules. Within a given patient, clinical features of both medium- and small-vessel disease may be found. RV may lead to necrosis, blood vessel occlusion, and tissue ischemia in a manner that resembles other forms of systemic vasculitis.
In its tendency to affect medium-sized arteries and involve the skin, peripheral nerves, eyes, heart, muscles, and other organs, RV often has a clinical appearance similar to polyarteritis nodosa. (See "Clinical manifestations and diagnosis of polyarteritis nodosa in adults".)
The treatment of RV is the focus of this topic review. Many of the standard treatment practices for RV have been adapted from those for other forms of systemic vasculitis, particularly granulomatosis with polyangiitis (Wegener’s). The pathogenesis, clinical manifestations, and diagnosis of RV are presented separately. (See "Epidemiology and pathogenesis of rheumatoid vasculitis" and "Clinical manifestations and diagnosis of rheumatoid vasculitis".)
THE CLINICAL CONTEXT OF RV
In considering treatment approaches for the patient with rheumatoid vasculitis (RV), an understanding of the clinical context in which this extraarticular manifestation of rheumatoid arthritis (RA) occurs is essential. The typical patient with RV has suffered from RA for at least a decade before systemic vasculitis becomes apparent. At the time of RV onset, unfortunately, patients who have already incurred considerable morbidity from RA and its therapies require intensive, potentially toxic treatments more than ever.
The incidence of RV appears to be decreasing since the 1980s, suggesting that more aggressive treatment of RA may help to prevent the development of RV [1,2]. (See "Epidemiology and pathogenesis of rheumatoid vasculitis", section on 'Epidemiology'.)
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- THE CLINICAL CONTEXT OF RV
- CUTANEOUS VASCULITIS
- Isolated nailfold infarctions
- Leg ulceration
- RHEUMATOID AORTITIS
- SYSTEMIC VASCULITIS
- Initial therapy
- - Glucocorticoids
- - Rituximab
- - Cyclophosphamide
- - Alternatives to rituximab and cyclophosphamide
- Other agents
- Maintenance therapy
- Adjunctive care
- - Preventing side effects of therapy
- - Smoking cessation
- - Skin care
- SUMMARY AND RECOMMENDATIONS