Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: Analysis of four prospective trials including 278 patients

Abstract

Objective To determine the long‐term outcome of patients with polyarteritis nodosa (PAN), microscopic polyangiitis (MPA), and Churg‐Strauss syndrome (CSS), to compare the long‐term outcome with the overall French population, to evaluate the impact on outcome of the type of vasculitis, prognostic factors, and treatments administered at diagnosis, and to analyze treatment side effects and sequelae. Methods Data from PAN, MPA, and CSS patients (n = 278) who were enrolled between 1980 and 1993 were collected in 1996 and 1997 and analyzed. Two prognostic scoring systems, the Five‐Factors Score (FFS) and the Birmingham Vasculitis Activity Score (BVAS), were used to evaluate all patients at the time of diagnosis. Results The mean (±SD) followup of the entire population was 88.3 ± 51.9 months (range 3 days to 192 months). Of the 85 deaths recorded, at least 41 were due to progressive vasculitis or its consequences. Death rates reflected disease severity, as assessed by the FFS (P = 0.004) and the BVAS (P < 0.0002), and the 2 scores were correlated (r = 0.69). Relapses, rarer in hepatitis B virus (HBV)–related PAN (7.9%) than in MPA (34.5%) (P = 0.004), occurred in 56 patients (20.1%) and did not reflect disease severity. Survival curves were similar for the subpopulation of 215 patients with CSS, MPA, and non–HBV‐related PAN who were given first‐line corticosteroids (CS) with or without cyclophosphamide (CYC). However, CS with CYC therapy significantly prolonged survival for patients with FFS scores ≥2 (P = 0.041). Relapse rates were similar regardless of the treatment regimen; only patients treated with CS alone had uncontrolled disease. CYC was associated with a greater frequency of side effects (P < 0.00001). Conclusion Rates of mortality due to PAN (related or unrelated to HBV), MPA, and CSS reflected disease severity and were higher than the mortality rate in the general population (P < 0.0004). Rates of relapse, more common in MPA than HBV‐related PAN patients, did not reflect disease severity. Survival rates were better among the more severely ill patients who had received first‐line CYC. Based on these findings, we recommend that the intensity of the initial treatment be consistent with the severity of the disease. The use of the FFS and BVAS scores improved the ability to evaluate the therapeutic response.

Hepatotropic Viral Infection Associated Systemic Vasculitides—Hepatitis B Virus Associated Polyarteritis Nodosa and Hepatitis C Virus Associated Cryoglobulinemic Vasculitis

“…6 In all forms of systemic necrotizing vasculitis, early multisystem involvement, especially gastrointestinal is often fatal. 30 Five factor score (FFS) given by the French vasculitis group can be used to evaluate prognosis in patients with PAN, Eosinophilic granulomatosis with polyangiitis (EGPA, previously known as Churg-Strauss syndrome) and Microscopic polyangiitis. 31 While calculating FFS, a score of one is given for each of the following factors; proteinuria >1 g/24 h, Creatinine >140 mmol/l, specific gastrointestinal involvement, specific cardiomyopathy and specific central nervous system involvement.…”

Translational Mini-Review Series on Immunology of Vascular Disease: Accelerated atherosclerosis in vasculitis

“…Several studies have now shown that, during long-term follow-up, cardiovascular disease is a major cause of mortality in patients with ANCA-associated vasculitis [40][41][42][43][44][45][46][47][48]. Zaenker et al reported that patients with Wegener's granulomatosis had a higher frequency of cardiovascular diseases compared with healthy controls [odds ratio (OR) 6·7] [49].…”

Treatment of polyarteritis nodosa and microscopic polyangiitis with poor prognosis factors: A prospective trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in sixty‐five patients

“…According to a recently published retrospective analysis, it would appear that-for either disease-the addition of CY to CS benefits particularly patients presenting with factors of poor prognosis (4) and not for those without such signs (5). There is an ongoing controversy in the field of vasculitides whether CY should be administered orally (6,7) or intravenously (8,9).…”