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 = 0.0004) and renal involvement (P = 0.02). Being Italian was the only prognostic factor associated with a higher relapse-free survival (P = 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 = 0.04) and cardiac (P = 0.03) morbidity. ANCA positivity was correlated with chronic kidney disease (P = 0.03) and chronic neurologic disability (P = 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.

• Authors: Cécile-Audrey Durel, Julien Berthiller, Silvia Caboni, David Jayne, Jacques Ninet, Arnaud Hot
• Journal: Arthritis care & research (Arthritis Care Res (Hoboken)) Vol. 68 Issue 3 Pg. 374-87 (Mar 2016) ISSN: 2151-4658 [Electronic] United States
• PMID: 26315340 (Publication Type: Journal Article, Multicenter Study, Observational Study, Research Support, Non-U.S. Gov't)
• Copyright: © 2016, American College of Rheumatology.

Chemical References

• Adrenal Cortex Hormones
• Antibodies, Antineutrophil Cytoplasmic
• Biomarkers
• Immunosuppressive Agents

Topics

• Adolescent
• Adrenal Cortex Hormones (adverse effects, therapeutic use)
• Adult
• Aged
• Aged, 80 and over
• Antibodies, Antineutrophil Cytoplasmic (blood)
• Biomarkers (blood)
• Churg-Strauss Syndrome (blood, diagnosis, drug therapy, epidemiology)
• Disease Progression
• Disease-Free Survival
• Europe (epidemiology)
• Female
• Humans
• Immunosuppressive Agents (adverse effects, therapeutic use)
• Male
• Middle Aged
• Recurrence
• Remission Induction
• Retrospective Studies
• Severity of Illness Index
• Time Factors
• Treatment Outcome
• Young Adult