Long‐Term Followup of a Multicenter Cohort of 101 Patients With Eosinophilic Granulomatosis With Polyangiitis (Churg‐Strauss)

Abstract

Objective. To assess the long-term outcome in eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA). Methods. A total of 101 patients fulfilling the American College of Rheumatology criteria for EGPA were included between 1990 and 2011. Clinical features, antineutrophil cytoplasm autoantibodies (ANCAs), and Five-Factors Score (FFS) were assessed at diagnosis. Overall and cumulative survival rates, relapse-free survival, and sequelae were studied based on ANCA status and FFS.Results. The rate of cardiomyopathy did not differ according to ANCA status. A total of 79.6% of patients achieved first remission, but 81.1% relapsed. ANCA-positive patients did not relapse more frequently but exhibited more severe disease with mononeuritis (P 5 0.0004) and renal involvement (P 5 0.02). Being Italian was the only prognostic factor associated with a higher relapse-free survival (P 5 0.01), thanks to a longer maintenance of immunosuppressive drugs, suggesting the need for prolonged low-dose corticosteroids. Overall, survival reached 93.1% after a median followup of 6 years. No factor was associated with mortality, but patients over age 65 years with cardiomyopathy or ANCA positivity had more serious outcomes. Sequelae affected 83.2% of patients. Ear, nose, and throat (ENT) involvement was a protective factor for renal (P 5 0.04) and cardiac (P 5 0.03) morbidity. ANCA positivity was correlated with chronic kidney disease (P 5 0.03) and chronic neurologic disability (P 5 0.02). Conclusion. The actual challenges of EGPA management concern morbidity prevention and quality of life improvement. Long-term corticosteroid treatment appears to reduce relapse risk. ENT involvement is associated with less renal and cardiac morbidity. ANCA positivity predicts renal and neurologic damage.

Innate immune response reflects disease activity in eosinophilic granulomatosis with polyangiitis

“…53 It is reported that 22.7% to 42.6% of patients with EGPA are positive for MPO-ANCAs or ANCAs. 2,[54][55][56][57][58][59][60] The prevalence of ANCAs in EGPA patients was less than that in microscopic polyangiitis or granulomatosis polyangiitis, such as ANCA-associated vasculitis. In the present study, there was no difference in serum IL-33 concentra- 27 In the present study, we had detected more CD4 + T cells producing IL-25 in peripheral blood in patients with EGPA at relapse than in those at remission or in patients with BA, although the difference was very small.…”

Updates in antineutrophil cytoplasmic antibody (ANCA)‐associated vasculitis for the ENT surgeon

“…9,36 Head and neck manifestations are reported in 48%-96% cases at diagnosis. 37,38 Eosinophilia (>1.0 × 10 9 /L and/or >10% of leucocytes) develops subsequently, 39 which may be accompanied by fever. Whilst nasal crusting may occur in some cases, destructive features are typically absent.…”

“…ANCA positive 31%-58% 26,37 MPO ANCA positive 25%-50% 26,47 ENT involvement 48%-96% 37,38 Rhinologic CRSwNP 42%-51% 37,38 Change in sense of smell 40% 47 Nasal crusting 8%-43% 47,48 Otologic OME/CHL 3%-12% 26 59 although the importance of abstinence from cocaine for a good outcome cannot be overstated.…”

Five factor score of more than 1 is associated with relapse during the first 2 year‐follow up in patients with eosinophilic granulomatosis with polyangiitis

“…Previous studies reported the different clinical patterns between ANCA‐positive and ANCA‐negative EGPA; kidney involvement and peripheral neuropathy are more frequently observed in patients with EGPA having ANCA than those not having ANCA . EGPA has relatively good prognosis in comparison with other systemic vasculitides: the overall remission rate reaches up to 93%; however, 20–30% of patients achieving remission may experience relapse . The overall mortality rate during long‐term follow‐up is approximately 10% of patients having experienced relapse …”

“…EGPA has relatively good prognosis in comparison with other systemic vasculitides: the overall remission rate reaches up to 93%; however, 20–30% of patients achieving remission may experience relapse . The overall mortality rate during long‐term follow‐up is approximately 10% of patients having experienced relapse …”