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Intravenous immunoglobulins (IVIG) in systemic sclerosis: a challenging yet promising future

  • Interpretive synthesis review article
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Abstract

The etiology and pathogenesis of systemic sclerosis are still largely unknown, but a variety of humoral and cellular autoimmune phenomena have been documented. In addition, the rarity of the disease, the broad spectrum of clinical manifestations, and the relevant risk of severe complications as well as the highly variable disease course render its management a major challenge. Some immunomodulatory agents have been used, but no single agent has given a convincing proof of effectiveness, and treatment has remained largely symptomatic through recent years. Novel therapies are currently being tested and may have the potential of modifying the disease process and overall clinical outcome. Efficacy of intravenous immunoglobulins (IVIG) in different regimens (1–2 g/kg of body weight, administered over 2–5 consecutive days) has been described in a limited number of trials and small case series, showing benefits in skin, articular, and lung interstitial disease symptoms. However, studies on IVIG in systemic sclerosis still remain few, and further randomized controlled trials should be undertaken to assess their clinical effectiveness or define the optimal dosage and times of administration.

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Authors and Affiliations

  1. Research Center of Systemic Autoinflammatory Diseases and Behçet’s Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy

    Luca Cantarini & Antonio Vitale

  2. Institute of Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy

    Donato Rigante & Salvatore Napodano

  3. Department of Rheumatology, School of Health Sciences, University of Thessaly, Larissa, Greece

    Lazaros I. Sakkas & Dimitrios P. Bogdanos

  4. Division of Transplantation Immunology and Mucosal Biology, King’s College London, London, UK

    Dimitrios P. Bogdanos

  5. The Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel-Hashomer, Israel

    Yehuda Shoenfeld

  6. Sackler Faculty of Medicine, Tel Aviv University, Tel-Hashomer, Israel

    Yehuda Shoenfeld

  7. Rheumatology Unit, Policlinico “Le Scotte”, University of Siena, Viale Bracci 1, 53100, Siena, Italy

    Luca Cantarini

Authors
  1. Luca Cantarini
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  2. Donato Rigante
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  3. Antonio Vitale
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  4. Salvatore Napodano
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  5. Lazaros I. Sakkas
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  6. Dimitrios P. Bogdanos
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  7. Yehuda Shoenfeld
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Corresponding author

Correspondence to Luca Cantarini.

Additional information

Luca Cantarini and Donato Rigante have equally contributed to this manuscript.

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Cantarini, L., Rigante, D., Vitale, A. et al. Intravenous immunoglobulins (IVIG) in systemic sclerosis: a challenging yet promising future. Immunol Res 61, 326–337 (2015). https://doi.org/10.1007/s12026-014-8615-z

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  • DOI: https://doi.org/10.1007/s12026-014-8615-z

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