Abstract
Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss
Syndrome, is a multisystem antineutrophil cytoplasmic antibody (ANCA) positive vasculitis,
characterized by the presence of chronic rhinosinusitis, asthma and prominent peripheral
blood eosinophilia. Although the most commonly involved organ is the lung, followed
by the skin, EGPA can affect any organ system. Herein, we present the complicated
case of an 18-year-old male patient with severe life-threatening EGPA, with central
nervous system, cardiac and gasterointestinal involvement, which was resistant to
initial treatment with glucocorticoids and cyclophosphamide. The patient responded
well, achieving complete remission after the addition of rituximab and mepolizumab
to glucocorticoids and cyclophosphamide.
Key Indexing Terms
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References
- Antineutrophil cytoplasmic antibodies (ANCA).Autoimmunity. 2005; 38 (doi: 0.1080/08916930400022673): 93-103
- European Vasculitis Genetics Consortium. Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratifed by ANCA status.Nat Commun. 2019; 10: 5120https://doi.org/10.1038/s41467-019-12515-9
- Cardiac involvement in Churg-Strauss syndrome: impact of endomyocarditis.Medicine (Baltimore). 2009; 88: 236-243https://doi.org/10.1097/MD.0b013e3181af35a5
- A neutrophil activation biomarker panel in prognosis and monitoring of patients with rheumatoid arthritis.Arthritis Rheumatol. 2020; 72: 47-56https://doi.org/10.1002/art.41062
- Role of Neutrophil extracellular traps regarding patients at risk of increased disease activity and cardiovascular comorbidity in systemic lupus erythematosus.J Rheumatol. 2020; 47: 1652-1660https://doi.org/10.3899/jrheum.190875
- Eosinophil cationic protein stimulates TGF-β1 release by human lung fibroblasts in vitro.Inflammation. 2007; 30: 153-160https://doi.org/10.1007/s10753-007-9032-4
- Cardiac involvement in eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome): prospective evaluation at a tertiary referral centre.Eur J Intern Med. 2021; 85: 68-79https://doi.org/10.1016/j.ejim.2020. 12.008
- Eosinophilic granulomatosis with polyangiitis: dissecting the pathophysiology.Front Med. 2021; 8627776https://doi.org/10.3389/fmed.2021.627776
- Central nervous system involvement in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): report of 26 patients and review of the literature.Autoimmun Rev. 2017; 16: 963-969https://doi.org/10.1016/j. autrev.2017.07.007
- Multitargeted therapy for refractory eosinophilic granulomatosis with polyangiitis characterized by intracerebral hemorrhage and cardiomyopathy: a casebased review.Rheumatol Int. 2021; https://doi.org/10.1007/s00296-021-04950-z
- Rapid onset of small intestinal perforation after successful steroid treatment in eosinophilic granulomatosis with polyangiitis.Mod Rheumatol. 2016; 26: 968-970https://doi.org/10.3109/14397595.2014.940126
- Multiple ulcerations and perforation in the small intestine after steroid treatment in eosinophilic granulomatosis with polyangiitis: a case report and literature review.Cardiovasc Pathol. 2020; 47107193https://doi.org/10.1016/j.carpath.2019.107193
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome) complicated by perforation of the small intestine and cholecystitis.Intern Med. 2018; 57: 737-740https://doi.org/10.2169/internalmedicine.8975-17
- Multiple perforations and fistula formation following corticosteroid administration: a case report.World J Clin Cases. 2017; 5: 67-72https://doi.org/10.12998/wjcc.v5.i2.67
- French Vasculitis Study Group (FVSG). The five-factor score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort.Medicine (Baltimore). 2011; 90: 19-27https://doi.org/10.1097/MD.0b013e318205a4c6
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort.Arthritis Rheum. 2013; 65: 270-281
- Treatment of Churg-Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients.Arthritis Rheum. 2008; 58: 586-594
- Clinical manifestations and treatment of Churg-Strauss syndrome.Rheum Dis Clin North Am. 2010; 36: 527-543
- Long-term outcomes of 118 patientswith eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) enrolled in two prospective trials.J Autoimmun. 2013; 43: 60-69
- Defining conditions where long-term glucocorticoid treatment has an acceptably low level of harm to facilitate implementation of existing recommendations: viewpoints from an EULAR task force.Ann Rheum Dis. 2016; 75: 952-957
- Incidence and US costs of corticosteroid-associated adverse events: a systematic literature review.Clin Ther. 2011; 33: 1413-1432
- Canadian Vasculitis Network (CanVasc). Efficacy of conventional immunosuppressants in relapsing or refractory eosinophilic granulomatosis with polyangiitis: evidence from a Canadian single-center cohort.Clin Exp Rheumatol. 2020; 38 (Suppl 124): 171-175
- Rituximab in the treatment of refractory or relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).Arthritis Res Ther. 2013; 15 (R133. 10.1186/ar4313)
- Rituximab for the treatment of eosinophilic granulomatosis with polyangiitis (Churg-Strauss).Ann Rheum Dis. 2016; 75: 396-401https://doi.org/10.1136/annrheumdis-2014-206095
- Mepolizumab as the first targeted treatment for eosinophilic granulomatosis with polyangiitis: a review of current evidence and potential place in therapy.Ther Clin Risk Manag. 2018; 14: 2385-2396https://doi.org/10.2147/TCRM.S159949
- EGPA mepolizumab study team. Mepolizumab or placebo for eosinophilic granulomatosis with polyangiitis.N Engl J Med. 2017; 18 (376): 1921-1932https://doi.org/10.1056/NEJMoa1702079
- Refractory diffuse alveolar hemorrhage caused by eosinophilic granulomatosis with polyangiitis in the absence of elevated biomarkers treated successfully by rituximab and mepolizumab: a case report.Respir Med Case Rep. 2019; 26: 112-114https://doi.org/10.1016/j.rmcr.2018.12.010
- Concurrent use of mepolizumab and rituximab for eosinophilic granulomatosis with polyangiitis and multisystem involvement.Cureus. 2020; 12: e9242https://doi.org/10.7759/cureus.9242
- Eosinophilic granulomatosis with polyangiitis: newer therapies.Curr Rheumatol Rep. 2018; 20: 23https://doi.org/10.1007/s11926-018-0736-2
- Use of rituximab and mepolizumab for EGPA.Rheumatology. 2019; 58 (Suppl 2 (abstr 340))https://doi.org/10.1093/rheumatology/kez063.002
- Rituximab and mepolizumab combination therapy for glucocorticoid-resistant myocarditis related to eosinophilic granulomatosis with polyangiitis.Mod Rheumatol Case Rep. 2021; : 1-6https://doi.org/10.1093/mrcr/rxab022
Article info
Publication history
Published online: August 11, 2022
Accepted:
July 21,
2022
Received:
January 13,
2022
Identification
Copyright
© 2022 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.
