Page 325 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Rheumatoid Arthritis Vasculitis 299

Terminology and Nosology

In 2010 the American College of Rheumatology (ACR), formerly the American
Rheumatism Association, and the European League Against Rheumatism Collaborative
Initiative, published RA classification criteria [6] that replaced the 1987 Revised ACR criteria
for RA [7] defining definite RA based on the confirmed presence of synovitis in at least one
joint, absence of an alternative diagnosis that better explains it, and achievement of a
threshold score from the individual scores in four domains. The latter included the number
and site of the involved joints, serological abnormalities, elevation of acute-phase responses,
and symptom duration.

Table 1. Scott and Bacon Criteria for RV1

Mononeuritis multiplex or peripheral neuropathy
Peripheral gangrene
Biopsy evidence of acute necrotizing arteritis plus systemic illness such as fever or weight loss
Deep cutaneous ulcers or active extra-articular disease (e.g., pleurisy, pericarditis, scleritis) if
associated with typical digital infarcts or biopsy evidence of vasculitis
1Modified from [9].

This represented a focus upon features associated with earlier disease that was associated
with persistent and erosive disease rather than late-stage features thereof. The Revised 2012
Chapel Hill Consensus Conference (CHCC) [8] provided a useful nosology for the
vasculitides, categorizing RV as a vasculitis associated with systemic disease. Scott and
Bacon [9] described the response to early treatment with intravenous cyclophosphamide and
methylprednisolone compared to more conventional drug regimens and defined eligible
patients as those meeting one or more criteria of the following criteria including, MNM,
peripheral gangrene, biopsy evidence of acute necrotizing arteritis plus systemic disease
manifested by fever and weight loss; and deep cutaneous ulcers or active ExRA disease if
associated with typical digital infarcts or biopsy tissue evidence of vasculitis (Table 1).
Overall, the combination of intravenous cyclophosphamide and methylprednisolone has
resulted in more frequent healing of vasculitic lesions including, leg ulcers and neuropathy,
and a lower mortality than other conventional treatments.

Epidemiology

In a population-based study of patients living within the Norwich Health Authority in the
United Kingdom from 1988 to 1994, the overall annual incidence rate of RV was 12.5 per
million (95% confidence interval [CI] 8.5-17.7) [10]. In a retrospective cohort study of
hospitalized RA patients in Malmo, Sweden, from 1990 to 1994, the annual incidence rate of
RV was calculated to be 7.1 per million (95% CI 2.7-16.1) [11]. This annual incidence rate
may have underestimated the true incidence rate, as not all patients with RV were
hospitalized. There are conflicting data as to whether the incidence of RV has changed or
remained stable over recent decades. However, the preponderance of data does suggest that
the incidence of RV may in fact be decreasing, perhaps due to improvements in therapy for

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