Page 330 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

304 Elana J. Bernstein and Robert F. Spiera

Prognosis

Using actuarial methods and then current ARA criteria for RA [38], Vollerstein and
colleagues [1] noted decreased survival of patients with RV when compared to an age- and
sex-, and region matched general population. Moreover, their survival was also decreased in
comparison to that of an incidence cohort of community patients with RA. In comparison to
the latter cohort, the decreased survival was less than that of those with classic but not
definite RA. The clinical factors that predicted decreased survival in RV using an univariate
proportional-hazards model indicated that older patients, the failure to receive previous non-
steroidal anti-inflammatory drug (NSAID) therapy, the previous administration of cytotoxic
immunosuppressive agents, a higher dosage of corticosteroids at diagnosis, a decision to
continue or initiate corticosteroids, and an abnormal urinary sediment all showed statistical
significance with decreased survival (p<0.05). Increasing referral distance, other diseases,
hypogammaglobulinemia, and hypoalbuminemia demonstrated a trend toward an association
with decreased survival. The authors did not demonstrate a statistically significant association
between decreased survival and sex, type of skin lesion, MNM or number of extremities
involved, a positive biopsy, initial manifestation of vasculitis, duration of RA, activity of RA,
ExRA manifestations of subcutaneous nodules, previous treatment, presence of concentration
of RF, C3 and C4 levels, anemia, thrombocytosis, ESR, or abnormal chest radiograph.

Voskuyl and colleagues [39] studied the mortality of 61 RV patients in comparison to
244 RA controls. The unadjusted risk of death (HR) in RV patients compared with RA
controls was 1.65 (95% CI 1.05-2.58). After adjustment for prognostic factors, the HR was
reduced to 1.26 (95% CI 0.79-2.01), mainly due to removal of the effects of age and sex.
There was no excess mortality seen in RV patients with severe organ involvement when
compared with RV patients without severe organ involvement, although the former patients
were treated more often with cytostatic and immunosuppressive drugs. Infection was the main
cause of death in RV patients and cardiovascular disease in the RA controls. Vasculitis was
reported as the cause of death in only 1 RV patient. After allowance for general risk factors
such as age and sex, there remained only a slight excess mortality in RV patients compared
with RA controls.

Treatment

Isolated nail fold lesions tend to be benign and typically do not require an escalation of
immunosuppressive therapy [28].

Cyclophosphamide

Cyclophosphamide in combination with high dose corticosteroids is the mainstay of
therapy for RV [40]. In an open label study of 24 patients treated with intravenous
cyclophosphamide plus intravenous methylprednisolone in 21 patients versus a variety of
other medications including azathioprine, prednisolone, D-penicillamine, chlorambucil, oral
cyclophosphamide, prostaglandin infusion and intramuscular methotrexate, those receiving

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