Eosinophilic Granulomatosis with Polyangiitis (Churg‐Strauss)

@article{Nguyen2018EosinophilicGW,
  title={Eosinophilic Granulomatosis with Polyangiitis (Churg‐Strauss)},
  author={Yann Nguyen and Lo{\"i}c Guillevin},
  journal={Seminars in Respiratory and Critical Care Medicine},
  year={2018},
  volume={39},
  pages={471–481}
}
&NA; Eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg‐Strauss syndrome, is a systemic necrotizing vasculitis of small‐ and medium‐size vessels, characterized by asthma and blood eosinophilia. EGPA typically occurs in patients with preexisting asthma, and involves the skin, lungs, and peripheral nerves. Poor‐prognosis factors (i.e., involvement(s) of the gastrointestinal tract, heart, and/or kidney) have been assessed with the Five‐Factor Score (FFS). One‐third of the… Expand
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Granulomatosis eosinofílica con poliangitis: reporte de un caso y revisión de la literatura
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Lung involvement in ANCA-associated vasculitis.
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The case of a 79-year-old woman diagnosed in 2016 with EGPA, being treated with Prednisone and Azathioprine, who presented to the Neurological Emergency Department with right hemiplegia, dysmetria in the left arm and right hemi-hypoesthesia is presented. Expand
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References

SHOWING 1-10 OF 148 REFERENCES
Rituximab for the treatment of eosinophilic granulomatosis with polyangiitis (Churg–Strauss)
TLDR
The treatment of EGPA with rituximab resulted in high rates of improvement and reduced requirement of prednisolone, and may be considered for the treatment ofEGPA. Expand
Systemic Vasculitis with Asthma and Eosinophilia: A Clinical Approach to the Churg‐Strauss Syndrome
Drawing on our experience of 16 cases and a review of the English literature, we propose that CSS is under-diagnosed because of exclusive emphasis upon pathologic recognition of the disorder. TheExpand
Churg-Strauss syndrome: evolving concepts.
TLDR
Progress is needed in Churg-Strauss syndrome's therapeutic management, and better understanding of the complex disease mechanisms may aid such a quest. Expand
Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Report and analysis of 30 cases.
TLDR
The clinical and morphologic findings of allergic granulomatosis and angiitis of Churg and Strauss in 21 men and 9 women were reviewed and Corticosteroids seem to influence long-term survival favorably. Expand
Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg–Strauss)
TLDR
In EGPA, asthma is severe, antedates systemic manifestations by a mean of 12 years, and progresses to long-term persistent airflow obstruction despite corticosteroids in a large proportion of patients, which affects long- term management and morbidity. Expand
Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Report and analysis of 30 cases.
TLDR
The clinical and morphologic findings of allergic granulomatosis and angiitis of Churg and Strauss in 21 men and 9 women were reviewed and Corticosteroids seem to influence long-term survival favorably. Expand
Childhood-onset Eosinophilic Granulomatosis with Polyangiitis (formerly Churg-Strauss Syndrome): A Contemporary Single-center Cohort
TLDR
Eosinophilia, in combination with upper airway, pulmonary, musculoskeletal, neurologic, and cardiac manifestations, is frequently observed in cEGPA. Expand
Renal involvement in Churg-Strauss syndrome.
  • E. Clutterbuck, D. Evans, C. Pusey
  • Medicine
  • Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
  • 1990
TLDR
Treatment with high-dose prednisolone was generally successful in milder cases, but was not always sufficient to halt the progression of more severe vasculitis, and Renal disease improved in 14 of 16 patients, ten of whom have maintained normal renal function on follow-up. Expand
Omalizumab in patients with eosinophilic granulomatosis with polyangiitis: a 36-month follow-up study
TLDR
In patients with EGPA and moderate to severe allergic asthma, omalizumab can be beneficial and safe and enables corticosteroid tapering while decreasing eosinophilia and improving asthma symptoms over 36 months. Expand
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort.
TLDR
Although EGPA relapses remain frequent, mortality has declined, at least since 1996, and multivariable analysis identified cardiomyopathy, older age, and diagnosis during or prior to 1996 as independent risk factors for death and lower eosinophil count at diagnosis as predictive of relapse. Expand
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