scholarly journals Successful Use of Plasma Exchange in the Treatment of Corticosteroid-Refractory Eosinophilic Granulomatosis with Polyangiitis Associated with Gastrointestinal Manifestations

2016 ◽  
Vol 2016 ◽  
pp. 1-4
Author(s):  
Kohei Tsujimoto ◽  
Masato Yagita ◽  
Masashi Taniguchi ◽  
Masaaki Fujita
Keyword(s):  
Abdominal Pain ◽  
Plasma Exchange ◽  
Paranasal Sinus ◽  
Granulomatosis With Polyangiitis ◽  
Pulse Therapy ◽  
Methylprednisolone Pulse Therapy ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Gastrointestinal Involvement ◽  
Gastrointestinal Manifestations ◽  
Eosinophilic Granulomatosis

We describe the case of a 33-year-old woman having corticosteroid-refractory eosinophilic granulomatosis with polyangiitis (EGPA) who presented with abdominal pain and responded dramatically to plasma exchange. Eosinophilia, asthma history, neuropathy, pulmonary infiltrates, and paranasal sinus abnormalities confirmed the diagnosis of EGPA. Treatment was initiated with 1 g/day of methylprednisolone pulse therapy for 3 days followed by 60 mg/day of intravenous prednisolone without relieving abdominal pain. Then, plasma exchange was performed thrice. Abdominal pain disappeared after the first plasma exchange. Indication of plasma exchange for EGPA remains controversial; however, it may represent a valid option in cases with gastrointestinal involvement.

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.

scholarly journals Eosinophilic Granulomatosis with Polyangiitis and Diffuse Gastrointestinal Involvement

2014 ◽  
Vol 8 (3) ◽  
pp. 329-336 ◽  
Author(s):  
Diana L. Franco ◽  
Kevin Ruff ◽  
Lester Mertz ◽  
Dora M. Lam-Himlin ◽  
Russell Heigh
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Gastrointestinal Involvement ◽  
Eosinophilic Granulomatosis

scholarly journals Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis: Exuberant Classic Clinical Picture of a Rare Disease

2018 ◽  
Vol 10 (2) ◽  
pp. 175-181
Author(s):  
Luana Vieira Mukamal ◽  
Celso Tavares Sodré ◽  
Lara Beatriz Prata ◽  
Fernanda Nakasato ◽  
Tullia Cuzzi ◽  
...  
Keyword(s):  
Rare Disease ◽  
Clinical Picture ◽  
Granulomatosis With Polyangiitis ◽  
Pulse Therapy ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Churg Strauss Syndrome ◽  
Strauss Syndrome ◽  
Dexamethasone Pulse Therapy ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

The authors present a classic case of Churg-Strauss syndrome with an exuberant clinical picture in a 34-year-old woman. She showed the following diagnostic criteria: asthma, polyneuropathy, rhinopathy, marked eosinophilia, positive p-ANCA with a perinuclear pattern, and skin histopathology results suggestive of vasculitis with eosinophils. There was a good response to prednisone, dexamethasone pulse therapy, and cyclophosphamide.

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 Gastrointestinal involvement in patients with vasculitis: IgA vasculitis and eosinophilic granulomatosis with polyangiitis

2019 ◽  
Vol 07 (11) ◽  
pp. E1333-E1343 ◽  
Author(s):  
Keisuke Kawasaki ◽  
Shotaro Nakamura ◽  
Motohiro Esaki ◽  
Koichi Kurahara ◽  
Makoto Eizuka ◽  
...  
Keyword(s):  
Gastrointestinal Tract ◽  
Small Bowel ◽  
Granulomatosis With Polyangiitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Iga Vasculitis ◽  
Gastrointestinal Involvement ◽  
Duodenal Lesions ◽  
Endoscopic Characteristics ◽  
Eosinophilic Granulomatosis ◽  
Entire Gastrointestinal Tract

Abstract Background and study aims Among vasculitides, IgA vasculitis (IgAV) and eosinophilic granulomatosis with polyangiitis (EGPA) frequently damage the gastrointestinal tract. However, only a few studies have investigated the entire gastrointestinal tract in patients with IgAV or EGPA by endoscopy. The aim of this study was to clarify endoscopic characteristics of patients with IgAV and those with EGPA. Patients and methods Clinicopathological and endoscopic findings were retrospectively compared between 33 patients with IgAV and 19 patients with EGPA. Results Gastrointestinal involvement was observed in 33 patients with IgAV (100 %) and in 8 patients with EPGA (42 %; P = 0.0001). Duodenal involvement was more frequent in patients with IgAV (75.8 %) than in those with EGPA (21.1 %, P = 0.0002). Jejunoileal involvement was frequent in both groups (IgAV 94.4 %; EGPA 77.8 %). Gastric mucosal erythema was more frequent in patients with IgAV (18.2 %) than in those with EGPA (0 %, P = 0.0481). Duodenal mucosal erythema (IgAV 54.6 %; EGPA 21.1 %, P = 0.0227), ulcer (IgAV 33.3 %; EGPA 0 %, P = 0.0041), and hematoma-like protrusion (IgAV 21.1 %; EGPA 0 %, P = 0.039) were more frequently observed in patients with IgAV than in those with EGPA. Conclusions Frequent duodenal involvement, gastric mucosal erythema, and duodenal lesions including erythema, ulcer, and hematoma-like protrusion are characteristic of patients with IgAV. Because jejunoileal involvement was frequent in both groups of patients, small-bowel endoscopies should be performed for diagnosis of small-bowel lesions in patients with IgAV and EGPA.

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