scholarly journals FRI0219 MEPOLIZUMAB FOR EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS IN REAL WORLD DATA

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 693.2-693
Author(s):  
T. Shoda ◽  
T. Takeuchi ◽  
K. Nagai ◽  
J. Konma ◽  
S. Arawaka
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Age Of Onset ◽  
Remission Induction ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Clinical Settings ◽  
Osaka Medical College ◽  
Median Dose ◽  
Real World Data ◽  
Medical College ◽  
Eosinophilic Granulomatosis

Objectives:To investigate the treatments of eosinophilic granulomatosis with polyangiitis (EGPA) and evaluate the usage of mepolizumab in clinical settings.Methods:The subjects were consecutive EGPA patients who were hospitalized and treated at our department and the Rheumatology, Department of Internal Medicine IV, Osaka Medical College between 2002 and 2018. Their clinical data, treatments, and courses were examined, and the usage of mepolizumab was evaluated.Results:Of 49 EGPA patients, 41 could be analyzed (14 males and 27 females, mean age of onset: 56.4 years). The percentage of positive ANCA was 31.7%, and affected sites were peripheral nerve (92%), central nervous system (17%), skin (51%), ENT (39%), lungs (29%), heart (22%), digestive organs (12%), and kidneys (15%). Remission induction therapy was performed with PSL (41 cases, 100%), PSL pulse (16 cases, 39%), IVCY (17 cases, 41%), RTX (4 cases, 10%), IVIG (22 cases, 54%), AZA (22 cases, 54%), MTX (4 cases, 10%), MMF (2 cases, 5%), MIZ (1 case, 2%), and MEPO (1 case, 2%). Maintenance therapy was performed with PSL (41 cases, 100%), AZA (21 cases, 51%), MTX (6 cases, 15%), MMF (2 cases, 5%), MIZ (3 cases, 7%), and MEPO (10 cases, 24%). In 10 patients who received mepolizumab, the percentage of positive ANCA was 40%, and the median dose of PSL was reduced from 9.5 mg to 5.5 mg after administration. Neither relapses nor adverse events occurred in patients who had received mepolizumab.Conclusion:Mepolizumab reduced the dose of steroids and improved tolerability in EGPA patients with or without ANCA.Disclosure of Interests:None declared

scholarly journals A real-world assessment of mycophenolate mofetil for remission induction in eosinophilic granulomatosis with polyangiitis

2021 ◽  
Author(s):  
Mariana Philobos ◽  
Amy Perkins ◽  
Maira Karabayas ◽  
Paula Dospinescu ◽  
Nick Fluck ◽  
...  
Keyword(s):  
Mycophenolate Mofetil ◽  
Real World ◽  
Granulomatosis With Polyangiitis ◽  
Controlled Trial ◽  
Remission Induction ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Newly Diagnosed ◽  
Real World Data ◽  
Disease Remission ◽  
Eosinophilic Granulomatosis

AbstractEosinophilic granulomatosis with polyangiitis (EGPA) is a form of ANCA-associated vasculitis (AAV). Clinical trials demonstrating the efficacy of mycophenolate mofetil (MMF) for remission induction in AAV excluded patients with EGPA. Despite this, MMF is commonly used in these patients. The objective of this study was to evaluate, for the first time, the effectiveness and tolerance of MMF in EGPA remission induction. A retrospective, two-center, real-world study was conducted in patients with EGPA who received MMF in addition to prednisolone for newly diagnosed or relapsing disease between 2009 and 2019. Baseline, 3-, 6- and 12-month outcome data were extracted from electronic health records. The primary outcome was disease remission, defined as a Birmingham Vasculitis Activity Score of 0 at 6 months. Secondary outcomes included disease relapse, median prednisolone dose at 12 months and drug tolerance. In total, 15 patients (73% male, median age 57) with EGPA (11 newly diagnosed/4 relapsing) were identified. At 6 months, 67% had achieved disease remission. At 12 months, this was maintained (66.7%) and 4 patients had relapsed. All but one patient remained on MMF at study completion and all patients tolerated MMF. Our real-world data suggest that MMF is an effective and well-tolerated agent for achieving disease remission in EGPA. A future randomized controlled trial of MMF in this neglected orphan disease is now warranted.

Non-severe eosinophilic granulomatosis with polyangiitis: long-term outcomes after remission-induction trial

Rheumatology ◽  
2019 ◽  
Vol 58 (12) ◽  
pp. 2107-2116 ◽  
Author(s):  
Xavier Puéchal ◽  
Christian Pagnoux ◽  
Gabriel Baron ◽  
François Lifermann ◽  
Loïk Geffray ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Task Force ◽  
Controlled Trial ◽  
Remission Induction ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Long Term Outcomes ◽  
First Relapse ◽  
Eosinophilic Granulomatosis

Abstract Objective In a previous controlled trial, 1-year adjunction of AZA to glucocorticoids (GC) for patients with non-severe, newly diagnosed eosinophilic granulomatosis with polyangiitis (EGPA) failed to lower remission failure, vasculitis relapse and isolated asthma/rhinosinus exacerbation rates, or cumulative GC use at month (M) 24. The aim of this study was to analyse longer-term outcomes to determine whether subsequent vasculitis relapse or isolated asthma/rhinosinus exacerbation (IARE) rates differed. Methods After M24, patients were followed prospectively, being treated based on physicians’ best judgment. Flares and reasons for increased GC dose or immunosuppressant use were recorded, and reviewed according to randomization group to distinguish vasculitis relapses from IAREs according to EGPA Task Force recommendations. Results Fifty EGPA trial participants were followed for a median (interquartile range) of 6.3 (5.4–7.6) years; two (4%) died 11 months post-inclusion. By M24, vasculitis had relapsed in 21/49 (43%) patients and 14/50 (28%) had IAREs. Another patient died 4.8 years post-inclusion (infection). Among nine patients with subsequent vasculitis relapses, three had a major relapse and three had their first relapse after M24; among 25 patients with later IAREs, 17 occurred after M24. At 5 years, respective vasculitis relapse and IARE rates were 48% (95% CI 34.0, 62.6) and 56% (95% CI 41.7, 70.8), with no between-arm differences (P = 0.32 and 0.13). No entry clinical or biological parameter was associated with these outcomes during follow-up. Conclusion These results confirmed that 1-year AZA and GC induction obtained good overall survival but no long-term benefit for non-severe EGPA patients. Vasculitis relapses, occurring mostly during the first 2 years, and IAREs, occurring throughout follow-up, require other preventive treatments. Trial registration ClinicalTrials.gov, https://clinicaltrials.gov, NCT00647166.

Interferon-α for Induction and Maintenance of Remission in Eosinophilic Granulomatosis with Polyangiitis: A Single-center Retrospective Observational Cohort Study

2017 ◽  
Vol 44 (6) ◽  
pp. 806-814 ◽  
Author(s):  
Benjamin Seeliger ◽  
Martin Förster ◽  
Janett Happe ◽  
Thomas Forberg ◽  
Anne Moeser ◽  
...  
Keyword(s):  
Cohort Study ◽  
Adverse Events ◽  
Pulmonary Function ◽  
Granulomatosis With Polyangiitis ◽  
Pulmonary Function Tests ◽  
Remission Induction ◽  
Interferon Α ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Function Tests ◽  
Eosinophilic Granulomatosis

Objective.Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by frequent relapses following induction therapy. Interferon-α (IFN-α) can reverse the underlying Th2-driven immune response and has successfully induced remission in previous reports. We undertook this study to investigate its efficacy and safety in patients with EGPA.Methods.We conducted a retrospective monocentric cohort study including 30 patients (16 women) with active EGPA under IFN-α treatment. Primary endpoints were remission induction, occurrence of relapses, prednisolone (PSL) dosage at time of remission, and adverse events. Remission was defined by a Birmingham Vasculitis Activity Score (BVAS) of 0. Pulmonary function tests were recorded at baseline and at time of remission. Health-related quality of life was analyzed by questionnaire at baseline and following 12 months of treatment.Results.At baseline, the median BVAS was 6 (interquartile range 4–13.5) and remission or partial response was achieved in 25/30 patients. After initiation of IFN-α treatment, the median PSL dosages could be reduced from 17.5 mg/day at baseline to 5.5 mg/day at time of remission. Following remission, 17 relapses (5 major) in 16 patients were observed. Pulmonary function tests improved and the time of hospitalization decreased. Adverse events at initiation of treatment were common, but mostly transient. Severe adverse events occurred during treatment in 4 patients (autoimmune hepatitis, n = 1; drug-induced neuropathy, n = 3).Conclusion.IFN-α treatment results in high rate of remission and maintenance in EGPA with significant reduction in oral corticosteroids, although reversible adverse events may occur. IFN-α represents an alternative therapeutic option in cases of refractory to standard treatment.

scholarly journals Safety and Effectiveness of Mepolizumab Therapy in Remission Induction Therapy for Eosinophilic Granulomatosis with Polyangiitis: A Retrospective Study

2021 ◽  
Author(s):  
Masanobu Ueno ◽  
Ippei Miyagawa ◽  
Takafumi Aritomi ◽  
Koichi Kimura ◽  
Shigeru Iwata ◽  
...  
Keyword(s):  
Induction Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Retention Rate ◽  
Remission Induction ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Primary Endpoints ◽  
Secondary Endpoints ◽  
Eosinophilic Granulomatosis ◽  
Eosinophil Counts

Abstract Objectives: To investigate the safety and effectiveness of mepolizumab (MPZ), an anti-interleukin-5 antibody, as remission induction therapy for severe eosinophilic granulomatosis with polyangiitis (EGPA).Methods: The clinical courses of patients with severe EGPA over 6 months were retrospectively investigated and compared between patients treated with high-dose corticosteroid (CS) plus MPZ therapy (MPZ group, n=7) and those treated with high-dose CS plus intravenous cyclophosphamide (IVCY) pulse therapy (IVCY group, n=13). The primary endpoints were the MPZ retention rate and the IVCY completion rate. The secondary endpoints were adverse events and changes in the Birmingham Vasculitis Activity Score (BVAS), Vascular Damage Index (VDI), eosinophil counts, and concomitant CS doses, and the extent and rates of these changes were compared between the MPZ and IVCY groups.Results: Regarding the primary endpoints, the MPZ retention rate was 100%, and the IVCY completion rate was 61.5%. Regarding the secondary endpoints, adverse events were detected in 2/7 patients (28.6%) in the MPZ group and 7/13 patients (53.8%) in the IVCY group. BVAS and eosinophil counts significantly decreased in both groups at and after month 1, but there was no significant difference in the magnitude of changes between the two groups. VDI scores did not significantly increase in either group, and the degree of changes did not significantly differ between the two groups. Although concomitant CS doses significantly decreased at and after month 1 in both groups, the rates of decrease in CS doses at and after month 3 were significantly higher in the MPZ group.Conclusions: This study suggested that the use of MPZ as remission induction therapy for severe EGPA might be safe and effective for controlling disease activity and reducing CS doses.

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.

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