Use of Intravenous Immunoglobulin Therapy at Unconventional Doses in Refractory Fulminant Systemic Lupus Erythematosus

Authors

• Nicolò Binello Rare Diseases and Periodic Fevers Research Centre, Department of Internal Medicine, Agostino Gemelli University Hospital Foundation, Catholic University of the Sacred Heart, Rome, Italy
• Cristina Cancelli Rare Diseases and Periodic Fevers Research Centre, Department of Internal Medicine, Agostino Gemelli University Hospital Foundation, Catholic University of the Sacred Heart, Rome, Italy
• Stefano Passalacqua Department of Nephrology and Dialysis, Agostino Gemelli University Hospital Foundation, Catholic University of the Sacred Heart, Rome, Italy
• Francesco De Vito Rare Diseases and Periodic Fevers Research Centre, Department of Internal Medicine, Agostino Gemelli University Hospital Foundation, Catholic University of the Sacred Heart, Rome, Italy
• Gianmarco Lombardi Department of Nephrology and Dialysis, Agostino Gemelli University Hospital Foundation, Catholic University of the Sacred Heart, Rome, Italy
• Giovanni Gambaro Department of Nephrology and Dialysis, Agostino Gemelli University Hospital Foundation, Catholic University of the Sacred Heart, Rome, Italy
• Raffaele Manna Rare Diseases and Periodic Fevers Research Centre, Department of Internal Medicine, Agostino Gemelli University Hospital Foundation, Catholic University of the Sacred Heart, Rome, Italy

nicolo.binello@gmail.com

Keywords

Abstract

The use of human intravenous immunoglobulins (IVIg) in systemic lupus erythematosus (SLE) currently relies on evidence from small case series and is mainly regarded as an off-label strategy in cases that are refractory to conventional therapies or poorly controlled with high doses of corticosteroids. Standard dosage regimens typically entail the administration of a total amount of 2 g/kg of IVIg divided into five consecutive days in order to minimize the risk of severe adverse events. We herein describe the case of a 28-year-old woman with a known history of antiphospholipid syndrome (APS) who was admitted to our hospital following fulminant onset of SLE in spite of ongoing immunosuppressive therapy. Acute renal insufficiency with nephrotic-range proteinuria, central nervous system involvement, severe thrombocytopenia, malar rash, pancreatic injury and moderate-severe aortic valve steno-insufficiency were the most prominent clinical manifestations, along with high titres of anti-dsDNA antibodies. Pulses of methyl-prednisolone followed by high-dose corticosteroids proved ineffective. Strikingly, IVIg therapy delivered at unconventional doses (1.2 g/kg) due to the presence of multiple risk factors for adverse events resulted in a significant, comprehensive clinical improvement. Although large-scale randomized double-blind studies are needed, the use of IVIg might constitute a valuable therapeutic modality as a last-resort strategy in cases of fulminant SLE. The total dose of immunoglobulins should be dictated by the clinical response as well as the presence of pre-existing risk factors for adverse events.