scholarly journals Gastrointestinal perforation due to vasculitis at primary diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) despite a high dose glucocorticosteroids treatment

SpringerPlus ◽  
2014 ◽  
Vol 3 (1) ◽  
Author(s):  
Gunter Assmann ◽  
Marc Molinger ◽  
Michael Pfreundschuh ◽  
Rainer Bohle ◽  
Vincent Zimmer
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Primary Diagnosis ◽  
Gastrointestinal Perforation ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
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 Clinical features of central nervous system involvement in patients with eosinophilic granulomatosis with polyangiitis: a retrospective cohort study in China

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Suying Liu ◽  
Ling Guo ◽  
Xiaoyuan Fan ◽  
Zhaocui Zhang ◽  
Jiaxin Zhou ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Clinical Features ◽  
Associated Factors ◽  
Granulomatosis With Polyangiitis ◽  
Disease Duration ◽  
Gastrointestinal Perforation ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Cns Involvement ◽  
Eosinophilic Granulomatosis

Abstract Background Central nervous system (CNS) involvement is extremely rare in eosinophilic granulomatosis with polyangiitis (EGPA), but is associated with a poor prognosis in the five-factor score. This study aims to elucidate the clinical features and independently associated factors of EGPA with CNS involvement. Results CNS involvement was observed in 17.3% (19/110) of EGPA patients from Peking Union Medical College Hospital between 2007 and 2019. We retrospectively reviewed their clinical data and analyzed the independently associated factors. Their mean age was 51.7 ± 11.56 years with no male/female predominance. Ischemic lesions were the most common manifestations, accounting for 63.2% of the 19 cases, followed by posterior reversible encephalopathy syndrome (36.8%), spinal cord involvement (15.8%), medulla oblongata involvement (15.8%), and intracranial hemorrhages (15.8%). Compared to the control group, patients with CNS involvement were of older age (51.7 ± 11.56 vs. 43.7 ± 13.78 years, p = 0.019) and had a higher ratio in the digestive tract involvement (52.6% vs. 28.6%, p = 0.042). Further multivariate analysis revealed that age, disease duration, and fever were the potential independent risk factors for CNS involvement of EGPA. Glucocorticoids combined with cyclophosphamide were the strategic therapy (94.7%). Intrathecal injections of dexamethasone and methotrexate were administered to 21.1% of the patients. Although seven patients relapsed during glucocorticoid reduction, seventeen patients finally achieved clinical remission. One patient died of acute intracerebral hemorrhage within one month, and another died of gastrointestinal perforation. Outcomes and cumulative survival show no significant differences between the two groups. Conclusions CNS involvement is uncommon in EGPA with various manifestations, and ischemic lesions are the most frequent. Age, disease duration, and fever are independent factors associated with CNS involvement in EGPA. The therapy of glucocorticoids combined with cyclophosphamide and intrathecal injections yields favorable responses. Acute intracranial hemorrhage and gastrointestinal perforation may be the principal causes of death.

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 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 A case of eosinophilic granulomatosis with polyangiitis after prolonged intervals of an anti-interleukin-6 receptor antibody for rheumatoid arthritis

2021 ◽  
Author(s):  
Yuki Imai ◽  
Yasushi Kondo ◽  
Sho Ishigaki ◽  
Naoshi Nishina ◽  
Yuichiro Ota ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Granulomatosis With Polyangiitis ◽  
Allergic Diseases ◽  
Pericardial Fluid ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Upper Gastrointestinal Endoscopy ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Receptor Antibody ◽  
Eosinophilic Granulomatosis

ABSTRACT An 83-year-old woman with a history of asthma complained of left abdominal pain and was admitted to our hospital. She was treated with tocilizumab, an anti-interleukin (IL)-6 receptor antibody, with a prolonged interval for rheumatoid arthritis (RA). Laboratory tests revealed a remarkable increase in eosinophil count and inflammatory markers with negative antineutrophil cytoplasmic antibodies. Echocardiography revealed pericardial fluid retention, and contrast-enhanced computed tomography revealed the thickening of the gastric antrum wall. Upper gastrointestinal endoscopy and biopsy revealed eosinophilic infiltration into the gastric mucosal epithelium. She was diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA) with pericarditis and eosinophilic gastroenteritis. High-dose glucocorticoids with intermittent intravenous cyclophosphamide (IVCY) were initiated, resulting in remission. As IL-6 is involved in the pathogenesis of allergic diseases such as asthma, our case can provide insights into the pathogenic role of IL-6 in EGPA as the development of EGPA in our case may have been triggered by IL-6 signals enhanced with tocilizumab interval prolongation.

scholarly journals Rituximab and mepolizumab combination therapy for glucocorticoid-resistant myocarditis related to eosinophilic granulomatosis with polyangiitis

2021 ◽  
Author(s):  
Kana Higashitani ◽  
Ryusuke Yoshimi ◽  
Yuichiro Sato ◽  
Toshiyuki Watanabe ◽  
Atsushi Ihata
Keyword(s):  
Combination Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Granulomatous Inflammation ◽  
Myocardial Damage ◽  
Pulse Therapy ◽  
Left Ventricular ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Cardiac Wall ◽  
Eosinophilic Granulomatosis

ABSTRACT Eosinophilic granulomatosis with polyangiitis (EGPA), which belongs to the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides, is characterised by eosinophil-rich granulomatous inflammation and small- to medium-sized vessel vasculitis associated with bronchial asthma and eosinophilia. It sometimes causes severe organ damage, of which myocardial damage is one of the most important for determining the prognosis. A case of EGPA-associated myocarditis that was refractory to glucocorticoid therapy and responded successfully to rituximab (RTX) and mepolizumab (MPZ) combination therapy is presented. A 46-year-old woman was diagnosed with EGPA-associated myocarditis due to pre-existing asthma, eosinophilia, mononeuritis multiplex, and eosinophilic myocarditis by myocardial biopsy. Transthoracic echocardiography showed thickening of the cardiac wall, pericardial effusion, and left ventricular hypokinesis. Although the myocarditis was refractory to methylprednisolone pulse therapy followed by oral high-dose prednisolone, the disease activity reached remission with the successful tapering of glucocorticoid after initiation of the RTX and MPZ combination therapy. Combination therapy with RTX and MPZ can be a good treatment option for EGPA-associated myocarditis for which it is difficult to give intravenous cyclophosphamide due to cardiac dysfunction.

scholarly journals High-dose Intravenous Immunoglobulin Treatment Increases Regulatory T Cells in Patients with Eosinophilic Granulomatosis with Polyangiitis

2012 ◽  
Vol 39 (5) ◽  
pp. 1019-1025 ◽  
Author(s):  
NAOMI TSURIKISAWA ◽  
HIROSHI SAITO ◽  
CHIYAKO OSHIKATA ◽  
TAKAHIRO TSUBURAI ◽  
KAZUO AKIYAMA
Keyword(s):  
T Cells ◽  
Intravenous Immunoglobulin ◽  
Cd4 T Cells ◽  
Conventional Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Treg Cells ◽  
Ivig Treatment ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Eosinophilic Granulomatosis

Objective.We studied the effects of intravenous immunoglobulin (IVIG) treatment on clinical symptoms and regulatory T (Treg) cell frequency in patients with eosinophilic granulomatosis with polyangiitis (EGPA).Methods.Twenty-two EGPA patients with severe mononeuritis multiplex or cardiac dysfunction received IVIG therapy combined with conventional therapy (corticosteroid, immunosuppressants, or both). As a control, 24 EGPA patients without severe vasculitic symptoms were treated with conventional therapy. Before, during, and after treatment, we determined percentages of Treg cells and other relevant cells in patients’ peripheral blood.Results.The frequency of CD25+ among CD4+ T cells was lower at onset in the study group than in controls but increased significantly after IVIG treatment, relative to controls. The frequency of CD25+ among CD4+ T cells correlated with the frequency of FOXP3+ among CD4+ T cells and interleukin 10 produced by CD25+CD4+ T cells.Conclusion.The increase in Treg cells seen with the combination of IVIG and conventional therapy may promote remission in EGPA.

scholarly journals Effects of surgery and topical medication on eosinophilic granulomatosis with polyangiitis with otitis media and sinusitis: a case report

2020 ◽  
Vol 48 (4) ◽  
pp. 030006052092004
Author(s):  
Zijing Wang ◽  
Lin Han ◽  
Lisheng Yu
Keyword(s):  
Otitis Media ◽  
Granulomatosis With Polyangiitis ◽  
Otitis Media With Effusion ◽  
Granulomatous Inflammation ◽  
Sinus Surgery ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Strauss Syndrome ◽  
Ear Symptoms ◽  
Eosinophilic Granulomatosis

Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg–Strauss syndrome, is eosinophil-rich, necrotizing granulomatous inflammation often involving the respiratory tract. Furthermore, EGPA is necrotizing vasculitis that predominantly affects small to medium vessels and is associated with asthma and eosinophilia. Most patients with EGPA have sinusitis and some complain of hearing loss and refractory otitis media with effusion. Systemic use of immunosuppressants and glucocorticoids is currently recommended, despite the inevitable associated side effects. However, systemic treatment is not always effective for nasal and ear symptoms. We report a case of EGPA with refractory otitis media and chronic sinusitis, which were resistant to systemic high-dose steroids and immunosuppressants. However, these symptoms responded well to functional endoscopic sinus surgery and myringotomy and grommet insertion. We also administered budesonide nasal irrigation and glucocorticoid injection by intratympanic and postaural methods in this patient. The therapeutic effect was satisfactory.

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