PMID- 30543921 OWN - NLM STAT- MEDLINE DCOM- 20191202 LR - 20191202 IS - 1521-7035 (Electronic) IS - 1521-6616 (Linking) VI - 199 DP - 2019 Feb TI - Intravenous immunoglobulin (IVIG) in the vanguard therapy of Systemic Sclerosis. PG - 25-28 LID - S1521-6616(18)30714-9 [pii] LID - 10.1016/j.clim.2018.12.006 [doi] AB - Systemic Sclerosis (SSc) is a rare autoimmune disease that is characterized by a progressive skin fibrosis, an obliteration of the microvasculature and an exaggerated extracellular matrix deposition, which lead to a multisystemic dysfunction. Various pathogenetic mechanisms were described. The lack of a successful therapy make SSc a disease with a poor prognosis. The intravenous immunoglobulin (IVIG) has been used for a long time in different autoimmune diseases, and firstly used in SSc patients in 2000. IVIG has multiple non-specific mechanisms of action and, beyond an impressive improvement in muscle symptoms, a French nationwide cohort demonstrated that IVIG ameliorates the skin disease and systemic inflammation, and helps the daily dose corticosteroid's tapering at the end of the treatment. The benefits on gastrointestinal symptoms of IVIG was reported by a recent English article, in which the patients consistently reported a decrease in the gastro-esophageal reflux disease symptoms and their frequencies. The impact on the lung involvement still remains unclear. One of the advantages of IVIG is its safe profile. Few adverse effects were reported and most of them are mild, and can be managed and usually they do not relapse. Harmful effects were described, but they can be avoid with cautious and judicious use of this therapy. CI - Copyright (c) 2018 Elsevier Inc. All rights reserved. FAU - Gomes, Joao Pedro AU - Gomes JP AD - Zabludowicz Center for Autoimmune Disease, Sheba Medical Center, Tel Hashomer, Israel; Department A of Internal Medicine, Hospital and University Centre of Coimbra, Coimbra, Portugal; Faculty of Medicine, University of Coimbra, Portugal. Electronic address: jpcxl@hotmail.com. FAU - Santos, Lelita AU - Santos L AD - Department A of Internal Medicine, Hospital and University Centre of Coimbra, Coimbra, Portugal; Faculty of Medicine, University of Coimbra, Portugal. Electronic address: lelitasantos@netcabo.pt. FAU - Shoenfeld, Yehuda AU - Shoenfeld Y AD - Sackler Faculty of Medicine, Tel Aviv University, Israel; Zabludowicz Center for Autoimmune Disease, Sheba Medical Center, Tel Hashomer, Israel. Electronic address: shoenfel@post.tau.ac.il. LA - eng PT - Journal Article DEP - 20181210 PL - United States TA - Clin Immunol JT - Clinical immunology (Orlando, Fla.) JID - 100883537 RN - 0 (Immunoglobulins, Intravenous) SB - IM MH - Humans MH - Immunoglobulins, Intravenous/adverse effects/pharmacology/*therapeutic use MH - Scleroderma, Systemic/*drug therapy OTO - NOTNLM OT - Autoimmune diseases OT - Autoimmunity OT - Fibrosis OT - Intravenous immunoglobulins (IVIG) OT - Systemic sclerosis EDAT- 2018/12/14 06:00 MHDA- 2019/12/04 06:00 CRDT- 2018/12/14 06:00 PHST- 2018/12/14 06:00 [pubmed] PHST- 2019/12/04 06:00 [medline] PHST- 2018/12/14 06:00 [entrez] AID - S1521-6616(18)30714-9 [pii] AID - 10.1016/j.clim.2018.12.006 [doi] PST - ppublish SO - Clin Immunol. 2019 Feb;199:25-28. doi: 10.1016/j.clim.2018.12.006. Epub 2018 Dec 10.