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.

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.

Polyps, grommets and eosinophilic granulomatosis with polyangiitis

2018 ◽  
Vol 132 (3) ◽  
pp. 236-239 ◽  
Author(s):  
F G Kavanagh ◽  
W Hasan ◽  
D A Smyth ◽  
J E Fenton
Keyword(s):  
Observational Study ◽  
Diagnostic Criteria ◽  
Otitis Media ◽  
Nasal Polyps ◽  
Nasal Polyposis ◽  
Granulomatosis With Polyangiitis ◽  
Otitis Media With Effusion ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
The Mean ◽  
Eosinophilic Granulomatosis

AbstractObjective:To explore the link between nasal polyposis, refractory otitis media with effusion and eosinophilic granulomatosis with polyangiitis.Methods:A retrospective observational study was carried out of patients diagnosed with refractory otitis media with effusion necessitating grommet insertion and who had nasal polyps. Patients were evaluated to determine if they fulfilled the diagnostic criteria of eosinophilic granulomatosis with polyangiitis.Results:Sixteen patients (10 males and 6 females) were identified. The mean age of grommet insertion was 45.4 years. The mean number of grommets inserted per patient was 1.6. The mean number of nasal polypectomies was 1.7. All 16 patients had paranasal sinus abnormalities and otitis media with effusion, 14 had asthma, 9 had serological eosinophilia and 7 had extravascular eosinophilia. Nine patients met the diagnostic criteria for eosinophilic granulomatosis with polyangiitis.Conclusion:The co-presence of nasal polyps and resistant otitis media with effusion should raise the possibility of eosinophilic granulomatosis with polyangiitis.

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 (Churg-Strauss syndrome)

2020 ◽  
Vol 66 (7) ◽  
pp. 904-907
Author(s):  
Gabriela Venade ◽  
Cátia Figueiredo ◽  
Catarina Almeida ◽  
Nídia Oliveira ◽  
Luis Costa Matos
Keyword(s):  
Complex Disease ◽  
Granulomatosis With Polyangiitis ◽  
Previous History ◽  
Specific Treatment ◽  
Disease Diagnosis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Churg Strauss Syndrome ◽  
Strauss Syndrome ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

SUMMARY Churg–Strauss syndrome, Eosinophilic granulomatosis with polyangiitis (EGPA), is a systemic vasculitis that affects small– to medium-sized vessels. It is rare and part of the Anti-neutrophil cytoplasm antibody-associated vasculitis (ANCA) group. We present a 37-year-old man, with a previous history of asthma, that was sent to the ED due to 2 weeks of productive cough, occasional dyspnea on exertion, fever (one week), asthenia, and anorexia. Upon physical examination, he was subfebrile and tachycardic. He had leukocytosis (17.00 x10^9/L) and eosinophilia of 20.0 % (3.4 X10^9/L), creatinine level of 1.5 mg/dL, subtle elevation on liver function tests and CRP of 10.82mg/dL. On Chest X-Ray, there was infiltrate on the right pulmonary base. Due to a strong suspicion of EGPA, he was started on 80mg of prednisolone from admission. ANCA MPO was positive, with the remaining auto-immune study negative. He underwent Thorax CT (under corticotherapy) without relevant changes, as well as bronchoalveolar lavage, without macroscopic signs of alveolar hemorrhage. Because of active urinary sediment, nephrotic proteinuria (6.5g/24h), and acute renal failure he underwent a renal biopsy, which revealed pauci-immune crescentic glomerulonephritis, with predominantly acute findings (in the context of ANCA-MPO Vasculitis – EGPA). After the biopsy, he received three 1g methylprednisolone pulses and was started on Cyclophosphamide. He remained asymptomatic and renal function was restored. This case highlights the importance of integrating all findings in one clinical scenario to prevent a more complex disease diagnosis, with a specific treatment, from being missed.

Connective tissue diseases

2020 ◽  
pp. 127-184
Keyword(s):  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Granulomatosis With Polyangiitis ◽  
Connective Tissue Diseases ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Assessment Tools ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Key Points ◽  
Strauss Syndrome ◽  
Eosinophilic Granulomatosis

This chapter covers the connective tissue diseases including systemic lupus erythematosus, Sjögren’s syndrome, scleroderma, antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (including granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis), eosinophilic granulomatosis with polyangiitis (formerly known as Churg–Strauss syndrome), and microscopic polyangiitis), polyarteritis nodosa, and Behçet’s disease. For each example of a connective tissue disease it provides an overview of the condition and classification criteria, alongside the prognosis. Techniques and tricks for diagnosis, clinical features, assessment tools, and treatment are all covered. Key points of nursing care are described, including the nurse’s role in treatment with thalidomide and cyclophosphamide, and any particular organs that can be affected is detailed.

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