Heart failure not responsive to standard immunosuppressive therapy is successfully treated with high dose intravenous immunoglobulin therapy in a patient with Eosinophilic Granulomatosis with Polyangiitis (EGPA)

2017 ◽  
Vol 45 ◽  
pp. 13-15 ◽  
Author(s):  
Antonio Pecoraro ◽  
Ludovica Crescenzi ◽  
Laura Carucci ◽  
Arturo Genovese ◽  
Giuseppe Spadaro
Keyword(s):  
Heart Failure ◽  
Immunosuppressive Therapy ◽  
Intravenous Immunoglobulin ◽  
Granulomatosis With Polyangiitis ◽  
Immunoglobulin Therapy ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Intravenous Immunoglobulin Therapy ◽  
Eosinophilic Granulomatosis

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 Severe biventricular thrombosis in eosinophilic granulomatosis with polyangiitis: a case report

2020 ◽  
Author(s):  
Jihad Hamudi ◽  
Basheer Karkabi ◽  
Devy Zisman ◽  
Avinoam Shiran
Keyword(s):  
Heart Failure ◽  
Ejection Fraction ◽  
Complete Resolution ◽  
Cardiac Involvement ◽  
Granulomatosis With Polyangiitis ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Preserved Ejection Fraction ◽  
Eosinophilic Granulomatosis

Abstract Background Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg–Strauss syndrome, is a rare multisystem disease characterized by asthma, rhinosinusitis, and eosinophilia. Cardiac involvement, present in half the patients, may be life threatening. Case summary A young woman with long-standing asthma and nasal polyposis was admitted with new-onset dyspnoea, sinus tachycardia, and eosinophilia. She had severe biventricular thrombosis and severe tricuspid regurgitation (TR) on echocardiography, with preserved ejection fraction of both ventricles. Cardiac magnetic resonance (CMR) imaging showed diffuse subendocardial late gadolinium enhancement (LGE). She had a positive test for perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) confirming the diagnosis of ANCA positive EGPA. She was treated with anticoagulation, high-dose corticosteroids, cyclophosphamide, and rituximab with gradual resolution of her symptoms. Follow-up echocardiography showed significant improvement in ventricular thrombi and TR but could not reliably exclude residual ventricular thrombus. Repeat CMR at 11 months confirmed complete resolution of both ventricular thrombi and near complete resolution of LGE. Discussion Cardiac involvement in EGPA, a rare cause of heart failure, can manifest as severe biventricular thrombosis and severe TR, resulting in heart failure with preserved ejection fraction. Combined immunosuppression and anticoagulation can lead to complete remission within a year. CMR is instrumental for both diagnosis and follow-up of EGPA, allowing for safe discontinuation of oral anticoagulation.

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.

High-dose intravenous immunoglobulin therapy for myasthenia gravis

1997 ◽  
Vol 245 (1) ◽  
pp. 26-31 ◽  
Author(s):  
J. L. M. Jongen ◽  
P. A. van Doorn ◽  
Frans G. A. van der Meché
Keyword(s):  
Myasthenia Gravis ◽  
Intravenous Immunoglobulin ◽  
Immunoglobulin Therapy ◽  
High Dose ◽  
Intravenous Immunoglobulin Therapy
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