PMID- 34718166 OWN - NLM STAT- MEDLINE DCOM- 20211214 LR - 20221119 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 20 IP - 12 DP - 2021 Dec TI - Intravenous immunoglobulins reduce skin thickness in systemic sclerosis: evidence from Systematic Literature Review and from real life experience. PG - 102981 LID - S1568-9972(21)00261-5 [pii] LID - 10.1016/j.autrev.2021.102981 [doi] AB - INTRODUCTION: Intravenous immunoglobulins (IVIG) are a new therapeutic approach in systemic sclerosis SSc. An immunomodulatory and antifibrotic activity has been postulated. IVIG are generally well tolerated and have only rare side effects. Our retrospective study focused its attention on SSc, an autoimmune connective tissue disease, characterized by several complications which has a significant impact on patient's quality of life. The pathophysiology comprises fibrotic, vascular and immunological aspects. AIM: The aim of this study was to verify the effectiveness of IVIG on SSc skin involvement. Moreover, a systematic review of the literature (SLR) of the results obtained to date on the use of Intravenous immunoglobulin (IVIG) in SSc has been also performed. PATIENTS AND METHODS: The data of 24 patients (21 women, 3 male) with refractory diffuse SSc skin involvement were evaluated (mean age was 52.13 years). IVIG infusion at a dosage of 2 g/Kg body weight for 4 consecutive days/month, was started between 2002 and 2019. Skin involvement was evaluated with the modified Rodnan Skin Score (mRSS) before therapy and then again after 6 and 12 months. To perform the SLR, the PubMed, Medline, Embase, and Web of Science database were searched from 1990 to 2020 (keywords: IVIG, systemic sclerosis). Three assessors (E.A., C.B. & M.M.C) identified the criteria to scan all papers. RESULTS: From the total SLR (106 results), 17 papers were identified after the separation of the clinical cases from the studies (total number of treated patients 183). The studies were classified according to the organ involvement considered in each study, as well as the prescribed dose (high or low doses), and the therapeutic regimens. In the selected papers, the organs mainly involved were the skin, the gastrointestinal, the joint and the cardiovascular systems. Only in one case, plasmapheresis was associated to IVIG. All papers reported significant reduction of the skin involvement, although generally the strength of the works was limited the lack of control cases or by the low number of patients involved. From the real life experience, a statistically significant reduction of mRSS was obtained at 6 months follow-up (average value of -6.61 +/- 5.2, p < 0.001), and it was further maintained with a significant stabilization after 12-months (-11.45 +/- 9.63, p < 0.002). DISCUSSION: This SLR and the data of the retrospective study suggest that IVIG may improve skin involvement reducing mRSS in particular in those patients that were refractory to other standard of care therapies and represents a therapeutic option in patients with concomitant myositis. The literature review revealed encouraging perspectives on the use of this therapy, given the effectiveness found in the selected works. CI - Copyright (c) 2021. Published by Elsevier B.V. FAU - Agostini, Elana AU - Agostini E AD - Dept. Experimental and Clinical Medicine, University of Florence, Italy; Division of Rheumatology, AOUC, Florence, Italy. FAU - De Luca, Giacomo AU - De Luca G AD - Unit of Immunology, Rheumatology, Allergy and Rare diseases (UnIRAR), IRCCS San Raffaele Hospital, 20132 Milan, Italy; Vita-Salute San Raffaele University, 20132 Milan, Italy. FAU - Bruni, Cosimo AU - Bruni C AD - Dept. Experimental and Clinical Medicine, University of Florence, Italy; Division of Rheumatology, AOUC, Florence, Italy. FAU - Bartoli, Francesca AU - Bartoli F AD - Dept. Experimental and Clinical Medicine, University of Florence, Italy; Division of Rheumatology, AOUC, Florence, Italy. FAU - Tofani, Lorenzo AU - Tofani L AD - Dept. Experimental and Clinical Medicine, University of Florence, Italy; Division of Rheumatology, AOUC, Florence, Italy. FAU - Campochiaro, Corrado AU - Campochiaro C AD - Unit of Immunology, Rheumatology, Allergy and Rare diseases (UnIRAR), IRCCS San Raffaele Hospital, 20132 Milan, Italy; Vita-Salute San Raffaele University, 20132 Milan, Italy. FAU - Pacini, Giovanni AU - Pacini G AD - Dept. Experimental and Clinical Medicine, University of Florence, Italy; Division of Rheumatology, AOUC, Florence, Italy. FAU - Moggi-Pignone, Alberto AU - Moggi-Pignone A AD - Dept. Experimental and Clinical Medicine, University of Florence, Italy; Division of Internal Medicine, AOUC, Florence, Italy. FAU - Guiducci, Serena AU - Guiducci S AD - Dept. Experimental and Clinical Medicine, University of Florence, Italy; Division of Rheumatology, AOUC, Florence, Italy. FAU - Bellando-Randone, Silvia AU - Bellando-Randone S AD - Dept. Experimental and Clinical Medicine, University of Florence, Italy; Division of Rheumatology, AOUC, Florence, Italy. FAU - Shoenfeld, Yehuda AU - Shoenfeld Y AD - Ariel University, Israel; Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel Hashomer, Israel; Saint Petersburg State University, Saint-Petersburg, Russia. FAU - Dagna, Lorenzo AU - Dagna L AD - Unit of Immunology, Rheumatology, Allergy and Rare diseases (UnIRAR), IRCCS San Raffaele Hospital, 20132 Milan, Italy; Vita-Salute San Raffaele University, 20132 Milan, Italy. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Dept. Experimental and Clinical Medicine, University of Florence, Italy; Unit of Immunology, Rheumatology, Allergy and Rare diseases (UnIRAR), IRCCS San Raffaele Hospital, 20132 Milan, Italy; Division of Rheumatology, AOUC, Florence, Italy. Electronic address: marco.matuccicerinic@unifi.it. LA - eng PT - Journal Article PT - Review PT - Systematic Review DEP - 20211028 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0 (Immunoglobulins, Intravenous) SB - IM EIN - Autoimmun Rev. 2023 Jan;22(1):103198. PMID: 36402668 MH - Female MH - Humans MH - *Immunoglobulins, Intravenous/therapeutic use MH - Male MH - Middle Aged MH - Quality of Life MH - Retrospective Studies MH - *Scleroderma, Systemic/drug therapy MH - Skin MH - Treatment Outcome OTO - NOTNLM OT - Gastrointestinal involvement OT - Intravenous immunoglobulins OT - Skin involvement OT - Systemic sclerosis EDAT- 2021/11/01 06:00 MHDA- 2021/12/15 06:00 CRDT- 2021/10/31 20:50 PHST- 2021/07/09 00:00 [received] PHST- 2021/07/17 00:00 [accepted] PHST- 2021/11/01 06:00 [pubmed] PHST- 2021/12/15 06:00 [medline] PHST- 2021/10/31 20:50 [entrez] AID - S1568-9972(21)00261-5 [pii] AID - 10.1016/j.autrev.2021.102981 [doi] PST - ppublish SO - Autoimmun Rev. 2021 Dec;20(12):102981. doi: 10.1016/j.autrev.2021.102981. Epub 2021 Oct 28.