scholarly journals Novel Targets for Drug Use in Eosinophilic Granulomatosis With Polyangiitis

2021 ◽  
Vol 8 ◽  
Author(s):  
Martina Uzzo ◽  
Francesca Regola ◽  
Barbara Trezzi ◽  
Paola Toniati ◽  
Franco Franceschini ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Immunosuppressive Drugs ◽  
Term Therapy ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Small Vessels ◽  
New Therapeutic Approaches ◽  
Personalized Approach ◽  
Eosinophilic Granulomatosis

Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare autoimmune disease characterized by medium and small vessels inflammation. Cardiac vasculitic involvement is one of the most severe manifestations with a significant impact on patients' long-term prognosis: anyway, a specific therapeutic approach for heart involvement in EGPA has not been explored yet. Current regimen consists of a long-term therapy with high dose of glucocorticoids, causing the well-known related-adverse events; immunosuppressive drugs are used in patients with severe manifestations, with some limitations. New therapeutic approaches are needed for patients with refractory disease or contraindications to conventional therapies. The quest for the ideal therapy is going toward a more and more personalized approach: on the one hand, efforts are made to use already existing therapies in the most appropriate way; on the other hand, new insights into EGPA pathogenesis allow the discovery of new targets, as demonstrated by mepolizumab and rituximab, targeting eosinophils, and B-cell compartments. This review summarizes the emerging therapies used in EGPA, focusing on the most recent studies on biologics and analyzing their efficacy and safety.

scholarly journals Multiple hepatic aneurysms and dry gangrene of fingertips in eosinophilic granulomatosis with polyangiitis: a case report

2020 ◽  
Vol 16 (1) ◽  
Author(s):  
Eunsil Koh ◽  
Noeul Kang ◽  
Jin-Young Lee ◽  
Duk-Kyung Kim ◽  
Young Soo Do ◽  
...  
Keyword(s):  
Eosinophil Count ◽  
Granulomatosis With Polyangiitis ◽  
Stable State ◽  
Clinical Situation ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Case Presentation ◽  
The Right ◽  
Small Aneurysms ◽  
Eosinophilic Granulomatosis

Abstract Background Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis mainly affecting small-sized arteries. Involvement of medium-sized vessels is very rare in EGPA. Here we present the case of a patient with EGPA who showed multiple hepatic aneurysms and distal gangrene. Case presentation A known EGPA patient visited to the emergency room (ER) with abrupt squeezing abdominal pain. She had suffered from gangrene in the fingertips of both hands for 1 year because of arterial thrombosis associated with hypereosinophilia. However, her absolute eosinophil count in the ER was 1120 cells/µL. An abdomen-pelvis CT demonstrated subcapsular hematoma in the right hepatic lobe. A celiac angiogram demonstrated multiple sized aneurysms in both hepatic lobes and some aneurysms in S7 and S8 were huge, more than 1 cm in size. The shape of the small aneurysms resembled a string of beads, as in polyarteritis nodosa. Given the clinical situation, emergency embolization was performed. Before this patient visited to the ER, she had been treated with a high dose of systemic corticosteroid, azathioprine, and cyclophosphamide. After addition of mepolizumab, the eosinophil count remained stable state with a near zero percentage of total white blood cell count. Conclusions Aneurysm and gangrene resulting from the involvement of medium-sized vessels can occur in EGPA. Destruction of vessels might occur even if eosinophil count is below 1500 cells/µL. If involvement of medium-sized arteries is suspected, thorough investigation to identify the involved organs and prompt management are needed to prevent fatal complications.

scholarly journals A Progressive Case of Eosinophilic Myocarditis Due to Eosinophilic Granulomatosis With Polyangiitis in a Caucasian Male

2020 ◽  
Vol 8 ◽  
pp. 232470962096685
Author(s):  
Valerie F. Civelli ◽  
Vishal K. Narang ◽  
Rupam Sharma ◽  
Ritika Sharma ◽  
Jessica Kim ◽  
...  
Keyword(s):  
Allergic Rhinitis ◽  
Inflammatory Process ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Small Vessels ◽  
History Of ◽  
Eosinophilic Myocarditis ◽  
Eosinophilic Granulomatosis ◽  
Large Vessels

Vasculitis is an inflammatory process involving blood vessels of various sizes, including the small vessels in the kidneys to the large vessels, such as the aorta. This inflammatory condition is usually autoimmune in nature and is associated with involvement of many locations, such as the sinuses, lungs, kidneys, and even the heart. Specifically, eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis that may initially hide as asthma, allergic rhinitis, and/or sinusitis. However, it is known to become a lethal disease once progressed to include cardiovascular manifestations. It is important to remember EGPA as a differential for any patient with a history of asthma, allergic rhinitis, and/or sinusitis who also presents with cardiovascular complaints and eosinophilia. Treatment recommendations focus on immunosuppression in such cases. In this article, we discuss the case of a 62-year-old male, with a known history of asthma, who presented to the emergency department with concern for his chest pain and right-sided weakness. He was later diagnosed with EGPA with eosinophilic myocarditis. Diagnosis and treatment are described.

scholarly journals Eosinophilic granulomatosis with polyangiitis in comorbidpatient: is the immunological diagnostics always unambiguous

2020 ◽  
Vol 22 (2) ◽  
pp. 383-392
Author(s):  
V. N. Marchenko ◽  
E. A. Bruchkus ◽  
A. A. Lebedeva ◽  
D. A. Davydov ◽  
N. L. Shaporova ◽  
...  
Keyword(s):  
Bronchial Asthma ◽  
Granulomatosis With Polyangiitis ◽  
Smoking History ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Occupational Hazards ◽  
Clinical Pattern ◽  
Pulmonary Infiltrates ◽  
Eosinophilic Granulomatosis ◽  
Necrotising Vasculitis

Eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg—Strauss syndrome, represents a rare form of ANCA-associated necrotising vasculitis which affects small vessels. This disease is characterized by typical combination of immunological disturbances, hypereosinophilia, severe bronchial asthma, transient pulmonary infiltrates, and kidney injury which is less frequent than in other forms of necrotising vasculitis.Verification of the diagnosis is often hampered by comorbidities, such as chronic obstructive pulmonary disease (COPD) in the patients with long-term smoking history and occupational hazards.In this article, we report a clinical case of EGPA in elderly patient with preceding COPD which caused diagnostic difficulties for this eosinophilic syndrome. Clinical pattern at the beginning of disease was presented by moderate inspiratory dyspnea and cough with small amounts of mucus sputum, which appeared after longterm exposure to chlorine-containing substances. COPD diagnosis in this patient was based on clinical pattern, long smoking experience, and occupational hazards. However, persistence of the symptoms during the ongoing therapy, as well as multidirectional dynamics of transient pulmonary infiltrates found on repeated CT-scans, prompted us to intensify diagnostic search for a systemic disease. Clinical, laboratory and instrumental signs of bronchial asthma were revealed, as well as hypereosinophilia and sensory polyneuropathy, which, if combined with CT-scan data, allowed us to prove the EGPA diagnosis.This case shows that, despite great value of immune diagnostics, with negative blood tests for ANCA, it is necessary to detect mutually complicating comorbid pathology. EGPA was considered the basic diagnosis, and COPD as accompanying disorder, taking into account such reasons as an unfavorable prognosis for EGPA and the need for long-term chemotherapy with systemic corticosteroids and monoclonal antibodies. ANCA-negative testing in the patient, absence of severe kidney and skin lesions allows to suggest better clinical prognosis in this patient.

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