Case Report: Severe ANCA-positive eosinophilic granulomatosis with polyangiitis presenting with Loeffler endocarditis and cryofibrinogenemia-associated digital gangrene successfully treated with rituximab - Report - MDSpire

Case Report: Severe ANCA-positive eosinophilic granulomatosis with polyangiitis presenting with Loeffler endocarditis and cryofibrinogenemia-associated digital gangrene successfully treated with rituximab

  • By

  • Irena Ostric Pavlovic

  • Danijela Trifunovic-Zamaklar

  • Branka Bonaci-Nikolic

  • Mileva Bascarevic

  • Vesna Tomic-Spiric

  • Aleksandra Peric-Popadic

  • Natasa Dragasevic Miskovic

  • Boris Ukropina

  • Aleksandar Reljic

  • Natasa Kusic

  • Andrija Pavlovic

  • Antonije Velickovic

  • Sara Radovic

  • Snezana Arandjelovic

  • July 7, 2026

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Clinical Report: Successful Management of Severe ANCA-positive Eosinophilic Granulomatosis

Overview

This case study presents a 37-year-old woman with severe eosinophilic granulomatosis with polyangiitis (EGPA) complicated by Loeffler endocarditis and digital gangrene. Treatment with rituximab was initiated after disease progression despite initial therapies.

Background

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare ANCA-associated vasculitis characterized by asthma, eosinophilia, and small-to-medium vessel inflammation. Cardiovascular involvement can significantly impact prognosis.

Data Highlights

No numerical or trial data provided in the source material.

Key Findings

  • A 37-year-old woman with EGPA presented with severe complications including Loeffler endocarditis and digital gangrene.
  • Despite initial treatment with corticosteroids and cyclophosphamide, the disease progressed, leading to ischemic lesions.
  • Rituximab treatment was initiated, and the patient underwent amputation of affected toes after clear demarcation of necrosis.
  • The patient was followed for three years.

Clinical Implications

This case underscores the importance of early recognition and intervention in severe EGPA cases. B-cell-targeted therapies like rituximab may be critical in managing complex presentations of the disease.

Conclusion

The successful management of this rare EGPA phenotype highlights the need for tailored therapeutic approaches in severe cases. Further research may elucidate the underlying mechanisms linking eosinophilic inflammation and vasculitis.

Related Resources & Content

  1. Frontiers in Immunology, 2026 -- Case Report: sustained five-year remission in eosinophilic granulomatosis with polyangiitis with intestinal perforation after surgery and rituximab-based therapy without glucocorticoid escalation
  2. Frontiers in Immunology, 2026 -- Commentary: Rituximab combined with lenalidomide for the treatment of marginal zone lymphoma with IgM kappa positivity and cold agglutinin syndrome: a case report
  3. Frontiers in Immunology, 2026 -- Rituximab is Associated with Accelerated Dialysis Independence in Anti-glomerular Basement Membrane Disease: A Retrospective Cohort Analysis of Renal Survival
  4. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of ANCA-Associated Vasculitis - PMC
  5. Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis - PMC
  6. Frontiers in Immunology — Case Report: Successful treatment of recurrent COVID-19 with intravenous immunoglobulin in a patient with rituximab-induced B-cell depletion and restoration of Fc-mediated effector functions
  7. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of ANCA-Associated Vasculitis - PMC
  8. Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis - PMC
  9. Rituximab Versus Conventional Therapy for Remission Induction in Eosinophilic Granulomatosis With Polyangiitis : A Randomized Controlled Trial - PubMed

Original Source(s)

Case Report: Severe ANCA-positive eosinophilic granulomatosis with polyangiitis presenting with Loeffler endocarditis and cryofibrinogenemia-associated digital gangrene successfully treated with rituximab

  • by Irena Ostric Pavlovic, Danijela Trifunovic-Zamaklar, Branka Bonaci-Nikolic, Mileva Bascarevic, Vesna Tomic-Spiric, Aleksandra Peric-Popadic, Natasa Dragasevic Miskovic, Boris Ukropina, Aleksandar Reljic, Natasa Kusic, Andrija Pavlovic, Antonije Velickovic, Sara Radovic, Snezana Arandjelovic

  • July 7, 2026

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