Treatment of rheumatoid vasculitis
- Patrick Whelan, MD, PhD
Patrick Whelan, MD, PhD
- Clinical Assistant Professor of Microbiology and Immunology, University of Southern California
Rheumatoid vasculitis (RV) is a protean, inflammatory process in patients with rheumatoid arthritis (RA) that is centered on the blood vessel wall itself. RV has a range of disease severity; in some patients, it is associated with substantial morbidity and an increased risk of mortality and can require intensive immunosuppressive therapy.
RV typically occurs in patients with longstanding, erosive RA and may affect a wide range of blood vessel types, from medium-sized muscular arteries to somewhat smaller arterioles or post-capillary venules. Clinical features of both medium- and small-vessel disease may be found within a given patient, and RV is generally classified among the forms of vasculitis associated with systemic diseases rather than based upon the size of the vessels involved. (See "Overview of and approach to the vasculitides in adults", section on 'Vasculitis associated with systemic disease'.)
RV may lead to blood vessel occlusion, necrosis, 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 (PAN). Treatment practices for RV have been adapted from those for other better studied forms of systemic vasculitis, particularly granulomatosis with polyangiitis (GPA; Wegener's), with which it also shares a number of features. (See "Clinical manifestations and diagnosis of polyarteritis nodosa in adults" and "Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis".)
The treatment of RV is presented here. The pathogenesis, clinical manifestations, and diagnosis of RV are presented separately. (See "Etiology and pathogenesis of rheumatoid vasculitis" and "Clinical manifestations and diagnosis of rheumatoid vasculitis".)
In determining 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. Consequently, at the time of RV onset, intensive and potentially toxic treatments will often be required in patients who have already incurred considerable morbidity from RA and its therapies. (See "Clinical manifestations and diagnosis of rheumatoid vasculitis", section on 'Clinical presentation'.)
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- GENERAL PRINCIPLES
- CUTANEOUS VASCULITIS
- Isolated nailfold infarctions
- Leg ulceration
- - Initial ulcer management with topical measures
- - Ulcers resistant to initial topical measures
- Cutaneous vasculitis during use of TNF inhibitors
- SYSTEMIC VASCULITIS
- Initial drug choice
- - Approach to current DMARD therapy at time of diagnosis of vasculitis
- - Choice of initial therapy for vasculitis
- - Drug administration, dosing, and efficacy
- - Intravenous pulse cyclophosphamide
- - Daily oral cyclophosphamide
- - Alternatives to rituximab and cyclophosphamide
- Other agents
- Resistant to initial therapy
- Maintenance therapy
- - Indications and timing for maintenance therapy
- - Choice of maintenance therapy
- - Duration of maintenance therapy
- - Relapse during maintenance therapy
- Monitoring of response
- Adjunctive care
- - Preventing side effects of therapy
- - Smoking cessation
- - Skin care
- RHEUMATOID AORTITIS
- SUMMARY AND RECOMMENDATIONS