Infantile bullous pemphigoid successfully treated with combined pulse corticosteroids and high-dose IVIG

2018 ◽  
Vol 31 (5) ◽  
pp. e12635 ◽  
Author(s):  
Bartlomiej Wawrzycki ◽  
Dorota Krasowska ◽  
Aldona Pietrzak ◽  
Justyna Szumilo ◽  
Lukasz Blazowski ◽  
...  
Keyword(s):  
Bullous Pemphigoid ◽  
High Dose ◽  
High Dose Ivig

scholarly journals AB0601 RAPID AND SUSTAINED EFFICACY OF AN INDUCTION TREATMENT WITH A TRIPLE THERAPY INCLUDING HIGH-DOSE INTRAVENOUS IMMUNOGLOBULINS, METHOTREXATE AND GLUCOCORTICOIDS IN ANTI-3-HYDROXY-3-METHYLGLUTARYL-COENZYME A REDUCTASE MYOPATHY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1597.1-1597
Author(s):  
E. Treppo ◽  
M. Infantino ◽  
M. Benucci ◽  
V. Ravagnani ◽  
B. Palterer ◽  
...  
Keyword(s):  
Triple Therapy ◽  
Antibody Titer ◽  
Intravenous Immunoglobulins ◽  
High Dose ◽  
Sustained Remission ◽  
Red Rice ◽  
Gradual Improvement ◽  
High Doses ◽  
High Dose Ivig

Background:Anti-3-hydroxy-3-methylglutaryl-coenzime A reductase (HMGCR) myopathy is a new entity, which has been clearly associated to statin use, even if it can be diagnosed in patients without a history of exposure to statin or even in the childhood (1).Objectives:The aim of the study is to describe the efficacy of a triple therapy regimen consisting in high-doses of intravenous immunoglobulins (IVIG), methotrexate (MTX), and glucocorticoids (GC) in 16 patients with Anti-HMGCR myopathy enrolled in 6 specialized centres.Methods:A total of 16 patients with anti-HMGCR myopathy (7 females; 9 males) were collected. Mean (±standard deviation) age at the onset of disease was 72.4±10.3 years old. All patients were diagnosed having anti-HMGCR myopathy [anti-HMGCR antibodies were measured by chemiluminescence assay (BioFlash, Inova, CA)] (2). Median follow-up was 29.5 months (interquartile range: 15.75-60 months). Anti-HMGCR antibodies were available in the follow-up in 8/16 patients.Results:Thirteen out of 16 patients (81.3%) had been exposed to statin (1/13 to red rice), 3/16 (18.7%) were not exposed. As induction therapy, 11/16 patients have been treated with triple therapy (high-dose IVIG, MTX and GC), 2/16 with double therapy (high-dose IVIG and GC), 2/16 have been treated with GC alone, the patient exposed to red rice resolved only with red rice suspension. Clinical remission and normalization of CPK values within month +24 were obtained in all the patients. All the patients were in remission at the last follow-up. Gradual improvement started soon from the first month, and among the 13 patients treated with an aggressive immunosuppresssive therapy including IVIG (13/13), GC (13/13) and methotrexate (11/13), 9/13 normalized the CPK value within 6 months. Clinical and laboratory response was accompanied by significant decrease or normalization of the anti-HMGCR antibody titer. All the patients were either not taking GC (56.3%), or were taking low doses of GC (43.7%) at the last follow-up. Four patients had stopped GC within 6 months. No serious side effects were recorded. After persistent remission, a maintenance immunosuppressive therapy was then administered. Only 3 relapses in 3 different cases were recorded, all of them during drug-free remission in long-term follow-up. Reinduction was again effective in all.Conclusion:Anti-HMGCR myopathy is a rare and serious myopathy which usually affects older people during statin treatment. After statin suspension, a rapid and sustained remission can be achieved by induction with a triple aggressive therapy consisting in medium-to high doses of GC, high-dose IVIG, and MTX (3). GC should be tapered as soon as possible. Relapse appears infrequent during maintenance treatment. Monitoring anti-HMGCR antibody titer may be clinically relevant.References:[1]AL Mammen et al. N Engl J Med. 2016;374:664-9[2]Musset L et al. Autoimmun Rev. 2016;15:983-93.[3]Aggarwal A et al. Scand J Rheumatol. 2019; 1-7.Acknowledgments:We thank MD Francesca Grosso and MD Valentina Mecheri from the University of Florence, MD Angela Zuppa and MD Chiara De Michelis, from San Martino Hospital, Genova, for their valued collaboration in data collectionDisclosure of Interests:Elena Treppo: None declared, Maria Infantino: None declared, Maurizio Benucci: None declared, Viviana Ravagnani: None declared, Boaz Palterer: None declared, Marina Grandis: None declared, Martina Fabris: None declared, Paola Tomietto: None declared, Mariangela Manfredi: None declared, Arianna Sonaglia: None declared, Maria Grazia Giudizi: None declared, Francesca Ligobbi: None declared, Daniele Cammelli: None declared, Paola Parronchi: None declared, Salvatore De Vita Consultant of: Roche, GSK, Speakers bureau: Roche, GSK, Novartis, Luca Quartuccio Consultant of: Abbvie, Bristol, Speakers bureau: Abbvie, Pfizer

scholarly journals High-dose intravenous immunoglobulin therapy induces and maintains complete remission for polyarteritis nodosa

2014 ◽  
Vol 1 (1) ◽  
pp. 13
Author(s):  
Kazu Ode ◽  
Yoshinori Taniguchi ◽  
Yoshitaka Kumon ◽  
Yoshio Terada
Keyword(s):  
Complete Remission ◽  
Intravenous Immunoglobulin ◽  
Polyarteritis Nodosa ◽  
Alternative Therapy ◽  
Ivig Therapy ◽  
Immunoglobulin Therapy ◽  
High Dose ◽  
Intravenous Immunoglobulin Therapy ◽  
First Line Therapy ◽  
High Dose Ivig

We report a case of successful high-dose intravenous immunoglobulin (IVIG) use in a patient with refractory polyarteritis nodosa (PAN). Treatments with prednisolone (PSL) and various types of immunosuppressants including methotrexate (MTX) and intravenous cyclophosphamide (IVCY) were unsuccessful, and then, high-dose IVIG therapy was added. High-dose IVIG therapy improved all symptoms including high fever, arthralgia, mononeuritis multiplex and indurated erythema due to PAN. Moreover, serum inflammatory markers were also normalized. High-dose IVIG is maintaining complete remission for PAN without flare-up for additional 4 years. Therefore, high-dose IVIG therapy might be considered as a first-line therapy in patients with PAN or alternative therapy in refractory PAN.

scholarly journals Occurrence of hemolytic anemia in patients with GBS treated with high-dose IVIg: Table

2014 ◽  
Vol 1 (4) ◽  
pp. e50 ◽  
Author(s):  
Thy P. Nguyen ◽  
Suur Biliciler ◽  
Amer Wahed ◽  
Kazim Sheikh
Keyword(s):  
Hemolytic Anemia ◽  
High Dose ◽  
High Dose Ivig

scholarly journals Treatment of Antibody-Mediated Renal Allograft Rejection: Improving Step by Step

2017 ◽  
Vol 2017 ◽  
pp. 1-9 ◽  
Author(s):  
Nils Lachmann ◽  
Michael Duerr ◽  
Constanze Schönemann ◽  
Axel Pruß ◽  
Klemens Budde ◽  
...  
Keyword(s):  
Graft Survival ◽  
Treatment Regimen ◽  
Low Dose ◽  
Immunosuppressive Treatment ◽  
Graft Function ◽  
Intravenous Immunoglobulins ◽  
Ivig Treatment ◽  
High Dose ◽  
Antibody Mediated Rejection ◽  
High Dose Ivig

Throughout the past years we stepwise modified our immunosuppressive treatment regimen for patients with antibody-mediated rejection (ABMR). Here, we describe three consecutive groups treated with different regimens. From 2005 until 2008, we treated all patients with biopsy-proven ABMR with rituximab (500 mg), low-dose (30 g) intravenous immunoglobulins (IVIG), and plasmapheresis (PPH, 6x) (group RLP,n=12). Between 2009 and June 2010, patients received bortezomib (1.3 mg/m2, 4x) together with low-dose IVIG and PPH (group BLP,n=11). In July 2010, we increased the IVIG dose and treated all subsequent patients with bortezomib, high-dose IVIG (1.5 g/kg), and PPH (group BHP,n=11). Graft survival at three years after treatment was 73% in group BHP as compared to 45% in group BLP and 25% in group RLP. At six months after treatment median serum creatinine was 2.1 mg/dL, 2.9 mg/dL, and 4.2 mg/dL in groups BHP, BLP, and RLP, respectively (p=0.02). Following treatment, a significant decrease of donor-specific HLA antibody (DSA) mean fluorescence intensity from8467±6876to5221±4711(p=0.01) was observed in group BHP, but not in the other groups. Our results indicate that graft survival, graft function, and DSA levels could be improved along with stepwise modifications to our treatment regimen, that is, the introduction of bortezomib and high-dose IVIG treatment.

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