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 Addison’s disease presenting with perimyocarditis

2018 ◽  
Vol 31 (1) ◽  
pp. 101-105
Author(s):  
Elisa Baranski Lamback ◽  
Grazia Morandi ◽  
Eleni Rapti ◽  
Georgi Christov ◽  
Paul A. Brogan ◽  
...  
Keyword(s):  
Systemic Inflammation ◽  
Granulomatosis With Polyangiitis ◽  
Clinical Manifestations ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Polyglandular Autoimmune Syndrome ◽  
Case Presentation ◽  
Prompt Treatment ◽  
Autoimmune Syndrome ◽  
Life Saving ◽  
Eosinophilic Granulomatosis

AbstractBackground:Polyglandular autoimmune syndrome (PGA) and eosinophilic granulomatosis with polyangiitis (EGPA) do not seem to represent a coincidental association.Case presentation:A case of a 15-year-old boy is reported who presented with severe systemic inflammation, perimyocarditis and cardiogenic shock, in whom EGPA was initially suspected and later diagnosed with autoimmune adrenalitis with PGA.Conclusions:The severity of the systemic inflammation and perimyocarditis suggests a more widespread autoimmune-mediated process. Autoimmune adrenal insufficiency should be considered in all cases of pericarditis and perimyocarditis, especially when the severity of clinical manifestations exceeds the expected for the severity of the cardiac findings, as timely identification and prompt treatment may be life-saving.

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 Effectiveness and Safety of Mepolizumab in Combination with Corticosteroids in Patients with Eosinophilic Granulomatosis with Polyangiitis

2020 ◽  
Author(s):  
Masanobu Ueno ◽  
Ippei Miyagawa ◽  
Kazuhisa Nakano ◽  
Shigeru Iwata ◽  
Kentaro Hanami ◽  
...  
Keyword(s):  
Real World ◽  
Eosinophil Count ◽  
Granulomatosis With Polyangiitis ◽  
Remission Rate ◽  
Retention Rate ◽  
Damage Index ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Interleukin 5 ◽  
Number Of Patients ◽  
Eosinophilic Granulomatosis

Abstract Objectives: Mepolizumab (MPZ), an anti-interleukin-5 antibody, is effective for treating eosinophilic granulomatosis with polyangiitis (EGPA). However, its effectiveness has not been adequately evaluated in real-world clinical practice. In this study, we assessed the effectiveness and safety of 300 mg MPZ for relapsing/refractory EGPA resistant to corticosteroids (CS) for 1 year in real-world settings. Methods: We administered MPZ (300 mg) to 16 patients with relapsing/refractory EGPA resistant to CS (with-MPZ). We also retrospectively collected data from the same patients for 12 months before administering MPZ (without-MPZ). The primary endpoint was the 12-month remission rate after MPZ administration, and the secondary endpoints were the Birmingham vasculitis activity score (BVAS), vasculitis damage index (VDI), eosinophil count, changes in concomitant CS doses/concomitant immunosuppressant use, MPZ retention rate, and incidence of adverse events. The clinical course was compared between Without-MPZ and With MPZ.Results: The 12-month remission rate after initiating MPZ was 75%. No change was observed in BVAS, eosinophil count, or concomitant CS dose in the without-MPZ, whereas all these parameters were significantly decreased in the with-MPZ. The number of patients on concomitant immunosuppressants also decreased in the with-MPZ. VDI did not increase in both groups. The MPZ retention rate was 100%, and only three patients (18.8%) had infections.Conclusion: This study demonstrated that MPZ is effective and safe for EGPA, furthermore, compared to Without-MPZ, MPZ improves disease activity and possesses a higher remission rate and CS sparing effect.

scholarly journals Safety and Efficacy of Mepolizumab in Patients with Eosinophilic Granulomatosis with Polyangiitis

2019 ◽  
Vol 2019 ◽  
pp. 1-6 ◽  
Author(s):  
Ravi Ranjan Pradhan ◽  
Gaurav Nepal ◽  
Shobha Mandal
Keyword(s):  
Side Effects ◽  
Granulomatosis With Polyangiitis ◽  
Multiple Organ ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Sustained Remission ◽  
Organ Systems ◽  
Frequent Relapses ◽  
Systemic Glucocorticoids ◽  
Eosinophilic Granulomatosis

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of vasculitis disorder which involves multiple organ systems and is characterized by asthma, pulmonary infiltrates, sinusitis, neuropathy, and peripheral eosinophilia. It also has an effect on the heart, skin, kidneys, and gastrointestinal tract. Interlukin-5 (IL-5) is involved in maturation and activation of eosinophil, the production of which is increased in the EGPA. Treatments of EGPA are limited to systemic corticosteroids and immunomodulators. These drugs are associated with significant side effects. Besides this, the response of patients to these drugs may be disappointing. Frequent relapses, the need for long-term medium-to-high-dose glucocorticoid therapy, and failure to achieve remission are not uncommon findings. There is a need for noble agents that could reduce frequent relapses and the dose of systemic glucocorticoids and maintain a sustained remission without significant side effects. Mepolizumab is IL-5 antagonist and may have value in treating patients with EGPA. Therefore, we did a systematic review to evaluate the efficacy and safety of mepolizumab in patients with EGPA.

scholarly journals Cardiac manifestations of eosinophilic granulomatosis with polyangiitis from a single-center cohort in China: clinical features and associated factors

2021 ◽  
Vol 12 ◽  
pp. 204062232098705
Author(s):  
Suying Liu ◽  
Ling Guo ◽  
Zhaocui Zhang ◽  
Mengtao Li ◽  
Xiaofeng Zeng ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pericardial Effusion ◽  
Eosinophil Count ◽  
Associated Factors ◽  
Granulomatosis With Polyangiitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Single Center ◽  
Myocardial Involvement ◽  
Cardiac Manifestations ◽  
Eosinophilic Granulomatosis

Background: Cardiac manifestations are common and life-threatening in eosinophilic granulomatosis with polyangiitis (EGPA), which remains poorly studied in China. We aim to investigate its clinical features, associated factors, treatment, and outcomes. Methods: We reviewed the clinical records of 110 EGPA patients and examined the independent factors associated with cardiac manifestations using multivariate logistic regression. Receiver operating characteristic curves determined the cut-off values, and survival was calculated via Kaplan–Meier curves. Results: Cardiac involvement was present in 36.4% (40/110) of EGPA patients, which mainly manifested as pericardial effusion (16.4%, 18/110), myocardial involvement (13.6%, 15/110), and heart failure (8.2%, 9/110). The mean age was 42.1 ± 14.23 years with no female/male predominance. Compared with the cardiac-unaffected group, the cardiac-affected group showed a lower rate of biopsy-proved vasculitis (0% versus 20%, p = 0.002). The eosinophil count [odds ratio (OR) = 1.142, 95% confidence interval (CI) 1.029–1.267] was independently associated with cardiac manifestations in EGPA, with a cut-off value of 3.66 × 109/L [area under the curve (AUC) = 0.692, p = 0.001]. Regarding treatment, the cardiac-affected group displayed a higher ratio of glucocorticoid pulse combined with intravenous cyclophosphamide (CYC-IV) (40% versus 21.4%, p = 0.037), and intravenous immunoglobulin combined with glucocorticoid plus CYC-IV (17.5% versus 4.3%, p = 0.035) than the control group. Outcomes ( p = 0.131) and survival ( p = 0.1972) were not significantly different between the groups. Conclusion: In this single-center Chinese EGPA cohort, cardiac manifestations are observed in 36.4% of patients, which primarily presents as myocardial involvement, pericardial effusion, and heart failure, independently associated with eosinophil count. Glucocorticoid combined with cyclophosphamide is the treatment cornerstone for EGPA patients with cardiac manifestations.

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.

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