{
  "abstract": "Introduction The early and persistent endothelial damage is one of the hallmarks of systemic sclerosis (SSc), an autoimmune connective tissue disease characterized by progressive skin and organ fibrosis. Aminaphtone (3-Methyl-1,4-dioxo-1,4-dihydro-naphthalen-2-y4-amino-benzoate) is a synthetic molecule marketed for clinical use in microvascular disorders. Nailfold videocapillaroscopy (NVC) is a reliable and non-invasive tool to assess microvascular damage in SSc patients. The aim of this retrospective study was to evaluate long term (4 years) safety and possible beneficial effects on microvascular damage induced by aminaphtone when added to standard therapy (ST) in SSc patients.Material and Methods Seventy-six SSc patients (68 females and 8 males, mean age 69±15 years) according to 2013 ACR/EULAR criteria with symptomatic secondary Raynaud’s phenomenon were treated with aminaphtone (75 mg BID) in addition to their stable ST for SSc. As control group was considered a cohort of 42 age-, sex- and therapy-matched SSc patients treated only with ST. All SSc patients provided written standard informed consent. Long term side effects related to aminaphtone treatment were carefully checked at least every six months. NVC was assessed at baseline, after 1 and 4 years of active treatment, recording the SSc-pattern classification (‘Early’, ‘Active’, ‘Late’). The timing of transition of the SSc-patterns was compared between groups.Results No serious side-effects were reported during the full observational time frame of four years. Nevertheless, 10% of SSc treated patients presented mild intolerance due to already reported reversable adverse events (headache 2.7%, itching 1.3%, others 6%) leading to aminaphtone discontinuation. NVC patterns in SSc patients treated with aminaphtone remained stable during the observational time frame in 91% of cases; in 9% of patients was observed a transition from ‘Early’ to ‘Active’ or from ‘Active’ to ‘Late’ SSc-pattern (6.6% and 3.4%, respectively). Of note, SSc patients treated with aminaphtone showed a significantly longer time of transition between NVC patterns compared to the control group (from ‘Active’ to ‘Late’ 120±64 months vs 50±26 months, p=0.05), while a similar transition time from ‘Early’ to ‘Active pattern (36±30 months vs 36±42 months; p>0.05) was observed.Conclusions Aminaphtone seems to be a safe and well tolerated compound in SSc patients with secondary RP during long term treatment. The NVC scleroderma-pattern stability over 4 years follow-up, suggests a potential supplementary therapeutic effect by aminaphtone when added to ST, at least on reducing the microvascular tissue damage progression. Further investigations to confirm the outcomes are ongoing.",
  "authors": [
    {
      "affiliations": [
        "Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, Genova, Italy",
        "Department of Experimental Medicine (DIMES), University of Genova, Genova, Italy"
      ],
      "name": "Rosanna Campitiello"
    },
    {
      "affiliations": [
        "Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, Genova, Italy",
        "IRCCS Ospedale Policlinico San Martino, Genova, Italy"
      ],
      "name": "Sabrina Paolino"
    },
    {
      "affiliations": [
        "Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, Genova, Italy",
        "IRCCS Ospedale Policlinico San Martino, Genova, Italy"
      ],
      "name": "Emanuele Gotelli"
    },
    {
      "affiliations": [
        "Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, Genova, Italy",
        "Department of Experimental Medicine (DIMES), University of Genova, Genova, Italy"
      ],
      "name": "Elvis Hysa"
    },
    {
      "affiliations": [
        "Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, Genova, Italy",
        "IRCCS Ospedale Policlinico San Martino, Genova, Italy"
      ],
      "name": "Carmen Pizzorni"
    },
    {
      "affiliations": [
        "Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, Genova, Italy",
        "IRCCS Ospedale Policlinico San Martino, Genova, Italy"
      ],
      "name": "Elisa Alessandri"
    },
    {
      "affiliations": [
        "Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, Genova, Italy",
        "IRCCS Ospedale Policlinico San Martino, Genova, Italy"
      ],
      "name": "Arlin Lysha"
    },
    {
      "affiliations": [
        "Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, Genova, Italy",
        "IRCCS Ospedale Policlinico San Martino, Genova, Italy"
      ],
      "name": "Antonio Maggio"
    },
    {
      "affiliations": [
        "Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, Genova, Italy",
        "IRCCS Ospedale Policlinico San Martino, Genova, Italy"
      ],
      "name": "Giulia Bernardi"
    },
    {
      "affiliations": [
        "Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, Genova, Italy",
        "IRCCS Ospedale Policlinico San Martino, Genova, Italy"
      ],
      "name": "Gianluca Mairone"
    },
    {
      "affiliations": [
        "Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, Genova, Italy",
        "IRCCS Ospedale Policlinico San Martino, Genova, Italy"
      ],
      "name": "Tamara Vojinovic"
    },
    {
      "affiliations": [
        "Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, Genova, Italy",
        "IRCCS Ospedale Policlinico San Martino, Genova, Italy"
      ],
      "name": "Maurizio Cutolo"
    },
    {
      "affiliations": [
        "Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, Genova, Italy",
        "IRCCS Ospedale Policlinico San Martino, Genova, Italy"
      ],
      "name": "Alberto Sulli"
    }
  ],
  "title": "P.263 Safety and efficacy of four years aminaphtone administration in systemic sclerosis patients",
  "uid": "d465652d-26d1-5f8c-9266-5ce960b63a53"
}