Page 242 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

216 Loic Guillevin

A small number of patients suffer die within the first weeks or months following the
diagnosis of PAN despite adequate treatment. Bourgarit and colleagues [60] showed that
38/309 (12%) patients died during the first year predominantly due to vasculitis (58%), while
others were related to treatment side effects or factors independent of vasculitis. The primary
cause of early mortality in PAN is severe gastrointestinal involvement with associated
perforation and hemorrhage, so noted by the FFS [52]; however HBV infection was not a
factor of severity. Among patients hospitalized in intensive care units (ICU), the main
prognostic factor for early mortality was predicted by the Acute Physiology and Chronic
Health Evaluation (APACHE) [82] which was not specifically devised for vasculitis. Neither
the vasculitis-specific Birmingham Vasculitis Activity Score (BVAS) [83] nor the FFS
scoring system [52], predicted ICU mortality [82].

Treatment

The FFS [52] is a useful treatment guide to treatment [84], the associated features of
which predicted increased mortality: proteinuria >1 g/day, renal insufficiency (creatininemia
>140 µmol/L or 1.6 mg/dL), specific cardiomyopathy, gastrointestinal and CNS involvement.
Although treatment should not overly influenced by FFS criteria [52], they may be considered
in deciding the therapeutic strategy. Accordingly, patients lacking the poorest prognostic
symptoms (FFS=0) may be treated with corticosteroids alone to reduce the number and
severity of treatment related side effects. This strategy is effective with a few minor relapses
necessitating transient dose intensification and addition of an immunosuppressant agent.
When the 1996 FFS for systemic necrotizing vasculitides was revisited [52], the following
factors were significantly associated with increased 5-year mortality: age >65 years, cardiac
symptoms, GI involvement, and renal insufficiency (stabilized peak creatinine ?150 µmol/L
or 1.7 mg/dL), each accorded +1 point; ear, nose and throat manifestations were scored –1
point, as they were associated with better outcomes. Respective 5-year mortality rates for FFS
of 0, 1 or ?2 were respectively 9%, 21% or 40%. The same strategy previously described for
the 1996 FFS [52] has been applied to patients with PAN.

Supportive care represents an important part of the therapeutic regimen for patients with
potentially fatal disease. Since maximal immunosuppression is given at the beginning of
treatment, antibiotic prophylaxis of opportunistic infections is often necessary but decided on
an individual basis; and pain control, prevention of pressure sores and physical therapy may
be needed for symptomatic mononeuritis multiplex. Angiotensin-converting-enzyme
inhibitors are effective antihypertensive agents in those with renal vasculitis due to the
beneficial effect on renal function. Fulminating vasculitis, which may manifest
gastrointestinal involvement, renal failure, pulmonary hemorrhage and rarely, cerebral
involvement, may be unresponsive to treatment resulting in excessive mortality and morbidity
in the first few month following diagnosis. Persistent abdominal pain should lead to
consideration of exploratory laparotomy to identify treatable bowel perforation; such patients
may require medication via intravenous route to circumvent impairment of orally
administered drugs. Rapid and severe weight loss due to severe gastrointestinal involvement
can be counterbalanced with parenteral nutrition. Although weight loss is not a proven poor
prognostic factor [52] common sense dictates the importance of optimizing all factors,
notwithstanding optimal weight, that might mitigate increased susceptibility to infection rate

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