Outcome of polyarteritis nodosa and churg‐strauss syndrome

Abstract

PAN and CSS differ in their morbidity and mortality; therefore, they should be considered as distinct clinical entities.

Dynamic pattern of endothelial cell adhesion molecule expression in muscle and perineural vessels from patients with classic polyarteritis nodosa

“…Interestingly, in PAN patients followed up longitudinally, circulating ICAM-1 and E-selectin levels remained elevated in spite of an appropriate clinical response to corticosteroid and immunosuppressive drug therapy (34). The persistence of inflammatory lesions despite treatment has also been observed in other vasculitides and suggests that, while some components of the disease are highly responsive to treatment, others are more refractory and probably contribute to subsequent relapses (46)(47)(48)(49)(50). Monoclonal antibodies that block functional epitopes of adhesion molecules have efficiently prevented the development of inflammatory lesions and clinically apparent disease in animal models of several autoimmune disorders (51)(52)(53)(54)(55), and some of these antibodies have been investigated in phase 1/11 clinical trials (13,56,57).…”

Churg–Strauss Syndrome

“…The most prominent organs involved are the peripheral nervous system, the skin, and the cardiovascular system. The GIT, the brain, the eyes, and the kidneys are less affected (Chumbley et al 1977;Lanham et al 1984;Guillevin et al 1988;Abu-Shakra et al 1994;Haas et al 2001;Guillevin and Pagnoux 2006). Occasionally, isolated involvement of the respiratory system, the GIT, or the heart can be observed, making the diagnosis difficult (Churg et al 1995;Noth et al 2003).…”

“…Renal involvement is reported in 8-49% of cases (Lanham et al 1984;Abu-Shakra et al 1994). It includes microscopic hematuria, hypertension, and sometimes proteinuria.…”

“…GIT involvement is reported in 8-62% of cases (Abu-Shakra et al 1994;Lanham et al 1984) and usually has a poor prognosis (Lanham et al 1984). Sometimes the involvement of the GIT occurs in isolation as a limited intestinal form of CSS.…”

“…Peripheral neuropathy is the second most common manifestation, occurring in 58-92% of cases (Abu-Shakra et al 1994;Della Rossa et al 2002). It is secondary to the involvement of the peripheral nerve nervosa vasorum.…”