The Five-Factor Score Revisited

Abstract

The 1996 Five-Factor Score (FFS) for systemic necrotizing vasculitides (polyarteritis nodosa [PAN], microscopic polyangiitis [MPA], and Churg-Strauss syndrome [CSS]) is used to evaluate prognosis at diagnosis. In the current study we revisited the FFS, this time including Wegener granulomatosis (WG).We analyzed clinical, laboratory, and immunologic manifestations present at diagnosis of systemic necrotizing vasculitides for 1108 consecutive patients registered in the French Vasculitis Study Group database. All patients met the American College of Rheumatology and Chapel Hill nomenclature criteria. Univariable and multivariable analyses yielded the 2009 FFS for the 4 systemic necrotizing vasculitides.Overall mortality was 19.8% (219/1108); mortality for each of the SNV is listed in descending order: MPA (60/218, 27.5%), PAN (86/349, 24.6%), CSS (32/230, 13.9%), and WG (41/311, 13.2%) (p < 0.001). The following factors were significantly associated with higher 5-year mortality: age >65 years, cardiac symptoms, gastrointestinal involvement, and renal insufficiency (stabilized peak creatinine ≥150 μmol/L). All were disease-specific (p < 0.001); the presence of each was accorded +1 point. Ear, nose, and throat (ENT) symptoms, affecting patients with WG and CSS, were associated with a lower relative risk of death, and their absence was scored +1 point (p < 0.001). Only renal insufficiency was retained (not proteinuria or microscopic hematuria) as impinging on outcome. According to the 2009 FFS, 5-year mortality rates for scores of 0, 1, and ≥2 were 9%, 21% (p < 0.005), and 40% (p < 0.0001), respectively.The revised FFS for the 4 systemic necrotizing vasculitides now comprises 4 factors associated with poorer prognosis and 1 with better outcome. The retained items demonstrate that visceral involvement weighs heavily on outcome. The better WG prognosis for patients with ENT manifestations, even for patients with other visceral involvement, compared with the prognosis for those without ENT manifestations, probably reflects WG phenotype heterogeneity.

Severity-based treatment for Japanese patients with MPO-ANCA-associated vasculitis: the JMAAV study

“…Thus, tailor-made treatment based on patients’ disease severity and disease type seemed to be useful in determining treatment regimens. The validity of the stratification we used, however, is also a matter of investigation in that it is related to other severity-based classifications, such as the five-factor score used by the French study group [14, 15]. …”

The 2016 European (Eular/Era-Edta) Recommendations for the Diagnosis and Management of Anca-Associated Systemic Vasculitis

“…Необходима регулярная комплексная клиническая оценка состояния пациента, для которой предложено ис-пользовать такие высокоинформативные клинические ин-струменты, как BVAS, индекс повреждения (Vasculitis Damage Index, VDI) [29,30], степени заболевания (Disease Extent Index) и 5 факторов прогноза (Five Factor Score) [31,32].…”

Clinical Expert Case of Eosinophilic Granulomatous Vasculitis

“…В плевральной жидкости также обнаружены в большом количестве эозинофилы (33 %). Обсле-дование на аутоантитела показало отрицательный результат, что встречается меньше чем в половине случав ЭГПА [5,7] (антитела к двуспиральной ДНК, антинейтрофильные цитоплазматические анти-тела (ANCA), антитела класса IgG к фосфолипидам, антитела класса IgМ к фосфолипидам, антиядерные антитела не превышали референтных значений). То есть пациентка относится кANCA-негативному типу ЭГПА с преобладанием клиники эозинофильной ин-фильтрации в органы и ткани [1].…”

“…Но, по-видимому, у нашей пациентки активность процесса и поражение лёгких, периферической нервной системы, ЦНС не позволили добиться ремиссии, несмотря на проведённую пульс-терапию и продолжающуюся пероральную глюкокортикостероидную терапию на протяжении года. По представленным обзорам по-следних лет [7,9] рецидивы и высокая активность больше характерны для ANCA-позитивных пациен-тов. Наша больная -ANCA-негативная.…”