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

  • 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

Keywords

Intravenous human immunoglobulins, refractory fulminant systemic lupus erythematosus

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.

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  • Published: 2018-08-24

    Issue: LATEST ONLINE (view)

    Section: Articles

    How to cite:
    Binello, N., Cancelli, C., Passalacqua, S., De Vito, F., Lombardi, G., Gambaro, G., & Manna, R. (2018). Use of Intravenous Immunoglobulin Therapy at Unconventional Doses in Refractory Fulminant Systemic Lupus Erythematosus. European Journal of Case Reports in Internal Medicine, 2. https://doi.org/https://doi.org/10.12890/2018_000934