Clinical manifestations and diagnosis of rheumatoid vasculitis
- Patrick Whelan, MD, PhD
Patrick Whelan, MD, PhD
- Associate in Rheumatology (Pediatrics)
- Massachusetts General Hospital for Children
Blood vessel inflammation is a central feature of rheumatoid arthritis (RA). Synovial membrane inflammation is characterized histologically by mononuclear cell cuffing of postcapillary venules. In addition, vascular inflammation is considered the primary event in the formation of rheumatoid nodules . In nodule formation, small-vessel vasculitis leads to fibrinoid necrosis that forms the core of the lesion, surrounded by fibroblastic proliferation.
In contrast to this type of blood vessel inflammation, however, the term “rheumatoid vasculitis” (RV) refers specifically to a protean, destructive inflammatory process that is centered on the blood vessel wall itself and that is associated with substantial morbidity. RV may affect a wide range of blood vessel types, from medium-sized muscular arteries to somewhat smaller arterioles to post-capillary venules. Within a given patient, clinical features of both medium- and small-vessel disease may be found. RV leads to necrosis, to blood vessel occlusion, and to tissue ischemia in a manner that resembles other forms of systemic vasculitis, particularly polyarteritis nodosa. (See "Clinical manifestations and diagnosis of polyarteritis nodosa in adults".)
This topic will review the clinical features of RV and the ways in which the diagnosis is established. The epidemiology, pathophysiology, and treatment of RV are presented separately. (See "Epidemiology and pathogenesis of rheumatoid vasculitis" and "Treatment of rheumatoid vasculitis".)
Rheumatoid vasculitis (RV) typically occurs in patients with longstanding, joint-destructive rheumatoid arthritis (RA). In one study, the mean duration between the diagnosis of RA and the onset of vasculitic symptoms was 13.6 years . Presentations of RV within five years of the RA diagnosis are very unusual. In a study of patients with RV diagnosed between 1980 and 1993, many (68 percent) had rheumatoid nodules (picture 1) and patients were typically strongly positive for rheumatoid factor . In a study of patients between 2000 and 2010, rheumatoid nodules were seen in only 44 percent of patients . RV usually develops at a time when the inflammatory arthritis is “burned out” (ie, when the erosive process that led to joint destruction has become less active). At such times, unfortunately, patients who have already incurred considerable morbidity from RA and its therapies require intensive, potentially toxic treatments more than ever.
In a cohort of 86 patients with RV diagnosed between 2000 and 2010, risk factors associated with the development of RV, after adjusting for age and disease duration, included current smoking at time of RA diagnosis, coexistent vascular disease (both peripheral vascular disease and cerebrovascular disease), and severe RA (defined by the presence of radiographic erosions, rheumatoid nodules, or a requirement for joint surgery) . Patients who had used biologic agents were at increased risk, while those who had received hydroxychloroquine or low-dose aspirin were at reduced risk.
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- CLINICAL PRESENTATION
- Constitutional symptoms
- Cutaneous vasculitis
- Neurologic disease
- Ocular disease
- Cardiac disease
- Pulmonary disease
- Renal disease
- Gastrointestinal, pancreatic, and hepatic disease
- LABORATORY FINDINGS
- - Rheumatoid factor
- - Anti-CCP antibodies
- - Antinuclear antibodies
- - Antineutrophil cytoplasmic antibodies
- - Anti-endothelial cell antibodies
- - Antibodies to glucose-6-phosphate isomerase
- Confirming the diagnosis
- - Angiography
- DIFFERENTIAL DIAGNOSIS
- Mimics of vasculitis
- Other vasculitides
- SUMMARY AND RECOMMENDATIONS