Mepolizumab in patients with eosinophilic granulomatosis with polyangiitis in remission: What is the right dose?

2021 ◽  
Vol 9 (7) ◽  
pp. 2942-2943 ◽  
Author(s):  
Florie Akdime ◽  
Xavier Puéchal ◽  
Nicolas Roche
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
The Right ◽  
Eosinophilic Granulomatosis

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 Eosinophilic granulomatosis with polyangiitis with an unusual presentation

2021 ◽  
Vol 35 (2) ◽  
Author(s):  
Rui Nogueira ◽  
◽  
Ana Belmira ◽  
Vitor Sousa ◽  
Rui Alves ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Central Retinal Artery Occlusion ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Peripheral Eosinophilia ◽  
Reactive Protein ◽  
Necrotizing Crescentic Glomerulonephritis ◽  
The Right ◽  
Eosinophilic Granulomatosis

Eosinophilic granulomatosis with polyangiitis is an ANCA vasculitis characterized by asthma, rhinosinusitis and peripheral eosinophilia. The kidney is infrequently involved, usually in the form of necrotizing crescentic glomerulonephritis. We present the case of a 60-year-old man who presented with painless sudden loss of visual acuity, purpuric exanthem in his legs, asthenia and myalgia. CT-scan ruled out acute vascular and intracranial space occupant lesions. Optical coherence tomography showed signs of left central retinal artery occlusion and perfusion deficits in the right arterial retinal blood supply. Complementary study showed prominent peripheral eosinophilia (24.500 cel/uL), increased serum IgE (1260U/L) and increased C-reactive protein (10.6mg/ dl). During admission, the patient presented with acute kidney failure (serum creatinine of 4.7mg/dl) and an exceptionally high p-ANCA MPO titer (>600U/L). Eosinophilic granulomatosis with polyangiitis was diagnosed and plasmapheresis, pulse steroid therapy and intravenous cyclophosphamide were provided. Kidney biopsy showed interstitial nephritis with high eosinophil content while the glomerulus was relatively spared, with only mild endocapillary proliferation. The patient didn’t require dialysis. Kidney function was normal at discharge, although the visual deficit did not improve.

scholarly journals Multiple cerebral infarction diagnosed as Eosinophilic Granulomatosis with Polyangiitis by autopsy

BMC Neurology ◽  
2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Kenichiro Hira ◽  
Hideki Shimura ◽  
Riyu Kamata ◽  
Masashi Takanashi ◽  
Akane Hashizume ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Brain Infarction ◽  
Eosinophil Infiltration ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Consciousness Disturbance ◽  
The Right ◽  
The Brain ◽  
Eosinophilic Granulomatosis

Abstract Background Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis of unknown cause involving the brain and accompanied by prominent eosinophilia. Intracardiac thrombosis is a major cardiac complication of EGPA that may cause thromboembolism. Case presentation A 53-year-old man presenting with abulia (consciousness disturbance) and left upper limb paralysis was admitted to our hospital. His case was complicated by penetrating branches, small vessel infarcts, and endocardial thrombosis in the right and left ventricle. Cardiomyopathy was also observed. Sixteen days after admission, the patient died from intracranial hemorrhage. Brain autopsy revealed two major findings: 1) large hemorrhagic infarction caused by cardiac embolism; and 2) granuloma and eosinophil infiltration. Vasculitis was accompanied by eosinophil infiltration in the cortical blood vessels and granuloma. Conclusions In this case study, we report autopsy findings of brain infarction in a patient with EGPA and endocardial thrombosis. The brain infarction was caused by the cardiac embolisms and vasculitis.

scholarly journals Eosinophilic Granulomatosis with Polyangiitis that was Difficult to Distinguish from Peroneal Nerve Palsy and Lumbar Disc Herniation: A Case Report

2021 ◽  
Vol 11 (6) ◽  
Author(s):  
Ryotaro Kumahara ◽  
Hitoshi Kudo ◽  
Ryo Inoue ◽  
Akira Fukuda ◽  
Seiya Ota ◽  
...  
Keyword(s):  
Lumbar Disc Herniation ◽  
Disc Herniation ◽  
Peroneal Nerve ◽  
Nerve Palsy ◽  
Granulomatosis With Polyangiitis ◽  
Lumbar Disc ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Peroneal Nerve Palsy ◽  
The Right ◽  
Eosinophilic Granulomatosis

Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) patients with mononeuropathy multiplex often visit orthopedic clinics; however, orthopedic surgeons have limited experience in diagnosing EGPA because of its rarity. We report a case of EGPA that required 1 month to confirm the diagnosis. Case Report: A 48-year-old woman presented with acute onset numbness in the right lower extremity. She had muscle weakness of the right lower extremity; lumbar spine magnetic resonance imaging showed lumbar disc herniation. Despite conservative treatment, her symptoms worsened. Blood tests showed increased eosinophils and serum IgE. She was diagnosed with EGPA, which should be considered in case of atypical paralytic symptoms. Conclusion: EGPA is so difficult to diagnose. In our case, the symptoms worsened on the 30th day after the initial visit. She was diagnosed with EGPA by a blood test at the time of admission. If patients with bronchial asthma or a history of allergies develop lumbar radiculopathy or peroneal nerve palsy-like symptoms, EGPA should be considered, and steroid treatment should be initiated early. Keywords: Eosinophilic granulomatosis with polyangiitis, peroneal nerve palsy, lumbar disc herniation, asthma.

scholarly journals Eosinophilic Granulomatosis with Polyangiitis Presenting with Myocarditis as an Initial Symptom: A Case Report and Review of the Literature

2021 ◽  
pp. 329-333
Author(s):  
Kanako Kurihara ◽  
Jun Tsugawa ◽  
Shinji Ouma ◽  
Toshiyasu Ogata ◽  
Mikiko Aoki ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Nerve Biopsy ◽  
Pulse Therapy ◽  
Sural Nerve Biopsy ◽  
Lower Limbs ◽  
Rapid Progression ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Initial Symptom ◽  
Steroid Pulse ◽  
Eosinophilic Granulomatosis

A 66-year-old woman with a history of bronchial asthma had shortness of breath and fatigue upon mild exercise. She was diagnosed as congestive heart failure. A blood test showed eosinophilia without the presence of anti-neutrophil cytoplasmic antibody (ANCA), and a myocardial biopsy specimen revealed eosinophilic infiltration in the myocardium. Eosinophilia was improved when she was administered short-term methylprednisolone. After that, she had numbness and pain in her lower limbs with re-elevation of eosinophils. She had dysesthesia and hypalgesia in the distal part of the limbs. Sural nerve biopsy revealed axonal degeneration and thickness of the arterial wall, indicating a diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA). Two courses of steroid pulse therapy were performed, resulting in marked improvement of her sensory symptoms. ANCA-negative EGPA might be associated with myocarditis and peripheral neuropathy. A sufficient immunotherapy should have been considered to prevent rapid progression.

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