Rituximab in the treatment of refractory or relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

  title={Rituximab in the treatment of refractory or relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)},
  author={Jens Thiel and Fabian H{\"a}ssler and Ulrich Salzer and Reinhard Edmund Voll and Nils Venhoff},
  journal={Arthritis Research \& Therapy},
  pages={R133 - R133}
IntroductionEosinophilic granulomatosis with polyangiitis (EGPA) is part of antineutrophil cytoplasmic antibodies (ANCAs)-associated vasculitides. In EGPA small-vessel vasculitis is associated with eosinophilia and asthma. About 40% of EGPA patients are ANCA-positive, suggesting a role for B cells in the pathogenesis of EGPA. B cell-depleting therapy with rituximab (RTX) can be effective in ANCA-positive EGPA, but very few patients have been published to date. The role of RTX in the treatment… 

Rituximab for the treatment of eosinophilic granulomatosis with polyangiitis (Churg–Strauss)

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.

Optimal therapy and prospects for new medicines in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Treatment for EGPA still has several unmet needs, and several biologic agents are now under investigation in randomized controlled trials, but a few others should be considered soon.

Biological drugs in ANCA-associated vasculitis.

Omalizumab in patients with eosinophilic granulomatosis with polyangiitis: a 36-month follow-up study

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.

Eosinophilic Granulomatosis with Polyangiitis (EGPA, Churg–Strauss)

Recent advances in EGPA management, including several novel immunomodulatory drugs and biotherapies, were or are being evaluated to improve EGPA patients’ prognoses.

Pharmacological approach to treating eosinophilic granulomatosis with polyangiitis (Churg–Strauss)

The standard of care for EGPA; the different therapeutic options available; and forthcoming therapeutic perspectives are reviewed, based on pathogenesis and clinical characteristics.

Successful use of Rituximab in a patient with recalcitrant multisystemic eosinophilic granulomatosis with polyangiitis

A case of a 56-year-old woman with a long-standing history of asthma, who presented with an acute sudden painless loss of vision after she had been recently diagnosed with EGPA and induced with pulse steroids and cyclophosphamide, and responded remarkably to Rituximab.



Rituximab for the treatment of Churg-Strauss syndrome with renal involvement.

In this pilot study, RTX was safe and successful in controlling renal disease activity in three patients with CSS and deserves further study in CSS.

Eosinophilic granulomatosis with polyangiitis (Churg–Strauss): state of the art

The pathogenesis of EGPA is multifactorial: the disease can be triggered by exposure to allergens or drugs, but a genetic background has also been recognized, particularly an association with HLA‐DRB4.

A multicenter survey of rituximab therapy for refractory antineutrophil cytoplasmic antibody-associated vasculitis.

Rituximab was effective remission induction therapy for refractory ANCA-associated vasculitis in this study and there was no difference in efficacy between the 2 main treatment regimens.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort.

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.

Severe bronchospasm associated with rituximab for refractory Churg–Strauss syndrome

Attention is drawn to the potential risk of rituximab-associated bronchospasm, by reporting that therapy-refractory CSS5 was successfully treated with ritUXimab in three patients, and the potential role of B lymphocytes plays a role as antigen-presenting cells and can modulate the immune response in another way than targeting antibody production.

A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg–Strauss, EGPA): monocentric experiences in 150 patients

Regular re-evaluation and target-orientated adaption of therapy may lead to normalization of life expectancy and attenuation of disease progression and Continued centre based interdisciplinary treatment should be standard of care.

Long-Term Effects of Rituximab Added to Cyclophosphamide in Refractory Patients with Vasculitis

Rituximab proved to be safe and effective in a long-term follow-up, and showed steroid- and immunosuppressive-sparing effects allowing the persistence of long-lasting remissions without maintenance therapy.

Successful use of rituximab in a patient with Churg–Strauss syndrome and refractory central nervous system involvement

The major clinical manifestations of Churg–Strauss syndrome include asthma, hypereosinophilia and the extrapulmonary manifestation of systemic vasculitis, with significant morbidity and mortality in affected patients.

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. The