Treatment of Pulmonary Vasculitis

Benjamin Terrier, MD, PhD; Loïc Guillevin, MD


Semin Respir Crit Care Med. 2018;39(4):504-510. 

In This Article

Antineutrophil Cytoplasmic Antibody–associated Vasculitis

Treatment of antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) may be adapted according to prognostic factors. The Five-Factor Score (FFS) was initially designed in 1996, then revisited in 2009 to include granulomatosis with polyangiitis (GPA), to evaluate disease prognosis and adapt therapeutic strategy to disease severity in patients with AAV and polyarteritis nodosa (PAN).[1,2]

Poor-prognostic factors in the 1996 FFS included proteinuria > 1 g/d, renal insufficiency with serum creatinine >140 μmol/L (>1.58 mg/dL), gastrointestinal tract involvement, cardiomyopathy, and central nervous system involvement.[1] The first version of the FFS considered PAN, microscopic polyangiitis (MPA), and eosinophilic GPA (EGPA). In the revisited version considering also GPA, factors that were significantly associated with higher 5-year mortality were age >65 years, cardiac symptoms, gastrointestinal involvement, renal insufficiency with stabilized peak creatinine >150 μmol/L (>1.70 mg/dL), and the absence of ear, nose, and throat symptoms.[2] Importantly, alveolar hemorrhage increased the risk of death by an odds ratio of 8.6, but it was not retained in the multivariable analysis as an independent prognostic factor, probably because it was already taken into account by another well-recognized poor-prognosis factor such as renal insufficiency (pulmonary–renal syndrome). However, no conclusion can be made regarding the prognostic impact of life-threatening alveolar hemorrhage requiring high oxygen or mechanical ventilation.

According to the FFS, we recommended and proposed trials to treat the most severe patients (FFS of at least 1) with a combination of glucocorticoids and immunosuppressive agents, whereas patients with a FFS of 0 were treated with glucocorticoids alone. However, the FFS is not applicable in GPA which requires in all cases a combination of glucocorticoids plus immunosuppressive agents, mostly cyclophosphamide (CYC) or rituximab (RTX).