Rheumatoid Vasculitis
Rheumatoid vasculitis (RV) is the type of vasculitis most commonly associated with rheumatoid arthritis, characterized by necrotizing or leukocytoclastic inflammation of small to medium-sized vessels. 1, 2
Clinical Characteristics
Rheumatoid vasculitis occurs as an extra-articular complication of long-standing, erosive, seropositive RA with high titers of rheumatoid factor. 1, 3 This represents a severe manifestation that significantly impacts both morbidity and mortality. 4
Key Clinical Features:
- Cutaneous manifestations are most common, including ischemic ulcers, digital gangrene, palpable purpura, and livedo reticularis 3, 5
- Peripheral nerve involvement presents as mononeuritis multiplex, representing another classic presentation 3
- Systemic manifestations can include weight loss, pleuritis, pericarditis, ocular inflammation, and hepatomegaly 3
- Renal involvement may be more common than historically recognized 3
Epidemiology and Prognosis
The incidence of rheumatoid vasculitis has declined significantly since the 1990s following widespread early use of methotrexate and modern immunosuppressive therapies. 1, 4 Despite this decline, RV is now considered a rare form of vasculitis. 1
However, mortality rates remain high despite therapeutic advances, making this a devastating complication when it occurs. 1, 2
Pathophysiology
The pathogenesis involves multiple mechanisms 4:
- High titers of rheumatoid factor and circulating immune complexes 3
- Cryoglobulins and diminished circulating complement 3
- Expansion of cytotoxic CD28null T cells 4
- Increased prevalence of HLA-DR4 3
- Recent evidence shows increased frequency among patients with anti-cyclic citrullinated peptide (anti-CCP) antibodies 4
Diagnostic Approach
Diagnosis ideally requires tissue biopsy or angiography demonstrating vasculitis, though clinical diagnosis is often necessary when biopsy is not feasible. 3
Diagnostic Considerations:
- Blind biopsies are generally not helpful, with the possible exception of rectal biopsy 3
- Laboratory findings include elevated ESR, increased C-reactive protein, anemia, thrombocytosis, hypoalbuminemia, and positive rheumatoid factor 3
- These laboratory abnormalities overlap significantly with uncomplicated RA, limiting their diagnostic utility 3
Clinical Variants
Isolated digital vasculitis must be distinguished from systemic rheumatoid vasculitis, as it carries a relatively favorable prognosis. 3 Small digital infarctions may occur alone or accompany other manifestations of clinical vasculitis. 3
Rarely, rheumatoid vasculitis can present in early RA, potentially representing an early aggressive form of the disease. 5 This challenges the traditional association with long-standing disease. 5
Treatment Approach
For systemic rheumatoid vasculitis, conventional treatment consists of glucocorticoids and cyclophosphamide. 1
Rituximab has an increasing role in treatment, similar to its use in other forms of vasculitis. 1
Treatment Algorithm by Severity:
- Isolated digital vasculitis: Requires only standard RA treatment without additional immunosuppression 3
- Ischemic ulcers: Appropriate dermatologic management plus standard RA therapy 3
- Systemic manifestations: High-dose corticosteroids with cyclophosphamide for induction, followed by maintenance therapy with agents such as methotrexate 5
Important Caveats:
The role of TNF inhibitors in rheumatoid vasculitis remains controversial and unclear. 4 Anti-TNF agents may paradoxically be both associated with triggering RV and potentially useful in treating it once established. 2, 4
All patients require attention to cardiovascular risk factors, including smoking cessation, blood pressure control, and nutritional optimization. 3 This is particularly important given the high mortality associated with cardiovascular comorbidity in patients with extra-articular manifestations. 4