{
  "abstract": "Background Systemic sclerosis (SSc) is a rare, heterogeneous autoimmune disease characterised by vasculopathy, immune activation and progressive fibrosis, resulting in multiorgan involvement and significant morbidity and mortality.Aim of the work This nationwide multicentre cross-sectional study aimed to evaluate the demographic, clinical, serological and therapeutic profiles of Egyptian SSc patients, enhancing understanding of disease patterns, comorbidities and management strategies.Methods A total of 1080 patients fulfilling the 2013 American College of Rheumatology/European League Against Rheumatism classification criteria for SSc were recruited from rheumatology centres across 18 Egyptian governorates. Data on demographics, clinical features, autoantibody profiles and treatments were collected and analysed.Results The mean patient age was 40.5±11.5 years, with a pronounced female predominance (88.4%, F: M ratio 7.6:1). Limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc were equally represented, while overlap syndromes occurred in 6.7%, most frequently with rheumatoid arthritis, myositis and systemic lupus erythematosus. Juvenile-onset SSc was identified in 5%, predominantly affecting females. Pulmonary and gastrointestinal involvement were common, including interstitial lung disease and gastro-oesophageal reflux disease. Pulmonary hypertension was more prevalent in lcSSc. Autoantibody analysis showed antinuclear antibodies positivity in 57.9%, RF in 6.1%, anti-topoisomerase I (anti-Scl-70) antibody in 28.1%, anticentromere antibody (ACA) in 24.8% and double positivity for anti-Scl-70 and ACA in 6.5%, with notable gender-based differences. Overlap syndrome patients showed higher comorbidity rates and more frequent therapeutic use. Regional variations in disease patterns were also observed.Conclusions This nationwide study provides a comprehensive profile of SSc in Egypt, highlighting a predominance of female patients with disease onset in the fourth decade of life. We observed significant gender-based differences in clinical manifestations and autoantibody profiles, with prevalent organ involvement including pulmonary and gastrointestinal complications. These findings underscore the importance of early recognition, tailored management strategies, and ongoing research to improve patient outcomes in SSc.",
  "authors": [
    {
      "affiliations": [
        "Rheumatology Department, Faculty of Medicine, Assiut University, Assiut, Egypt"
      ],
      "name": "Nevin Hammam"
    },
    {
      "affiliations": [
        "Rheumatology Department, Faculty of Medicine, Assiut University, Assiut, Egypt"
      ],
      "name": "Manal Hassanien"
    },
    {
      "affiliations": [
        "Rheumatology Unit, Internal Medicine Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt"
      ],
      "name": "Nermeen N Aziz"
    },
    {
      "affiliations": [
        "Rheumatology Department, Faculty of Medicine, Tanta University, Tanta, Egypt"
      ],
      "name": "Hanan El Saadany"
    },
    {
      "affiliations": [
        "Rheumatology Unit, Internal Medicine Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt"
      ],
      "name": "Nermeen Samy"
    },
    {
      "affiliations": [
        "Rheumatology Department, Faculty of Medicine, Cairo University, Cairo, Egypt"
      ],
      "name": "Noha A Azab"
    },
    {
      "affiliations": [
        "Rheumatology and Immunology Unit, Internal Medicine Department, Faculty of Medicine (Girls), Al-Azhar University, Cairo, Egypt"
      ],
      "name": "Eman F Mohamed"
    },
    {
      "affiliations": [
        "Rheumatology Department, Faculty of Medicine, Assiut University, Assiut, Egypt"
      ],
      "name": "Rania M Gamal"
    },
    {
      "affiliations": [
        "Rheumatology Department, Faculty of Medicine, Al-Azhar University, Assiut, Egypt"
      ],
      "name": "Abdelhafeez Moshrif"
    },
    {
      "affiliations": [
        "Rheumatology Department, Faculty of Medicine, Minia University, Minia, Egypt"
      ],
      "name": "Rawhya ElShereef"
    },
    {
      "affiliations": [
        "Rheumatology Department, Faculty of Medicine, Minia University, Minia, Egypt"
      ],
      "name": "Fatma Ali"
    },
    {
      "affiliations": [
        "Rheumatology Department, Faculty of Medicine, Zagazig University, Sharkia, Egypt"
      ],
      "name": "Amany El-Najjar"
    },
    {
      "affiliations": [
        "Rheumatology Department, Faculty of Medicine, Aswan University, Aswan, Egypt"
      ],
      "name": "Khaled A Abdelgalil"
    },
    {
      "affiliations": [
        "Rheumatology Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt"
      ],
      "name": "Reem El-Mallah"
    },
    {
      "affiliations": [
        "Rheumatology and Immunology Unit, Internal Medicine Department, Faculty of Medicine, Mansoura University, Dakahlia, Egypt"
      ],
      "name": "Samar Tharwat"
    },
    {
      "affiliations": [
        "Rheumatology and Clinical Immunology Unit, Internal Medicine Department, Faculty of Medicine, Cairo University, Cairo, Egypt"
      ],
      "name": "Gehad Maghraby"
    },
    {
      "affiliations": [
        "Rheumatology, Mansoura University Faculty of Medicine, Mansoura, Egypt"
      ],
      "name": "Amany S El-Bagnasawy"
    },
    {
      "affiliations": [
        "Rheumatology Unit, Department of Internal Medicine, Tanta University, Tanta, Egypt"
      ],
      "name": "Thanaa F Mansour"
    },
    {
      "affiliations": [
        "Rheumatology Department, Faculty of Medicine, Sohag University, Sohag, Egypt"
      ],
      "name": "Ahmed Elsaman"
    },
    {
      "affiliations": [
        "Department of Rheumatology and Rehabilitation, Faculty of Medicine, New Valley University, New Valley, Egypt"
      ],
      "name": "Osman Hammam"
    },
    {
      "affiliations": [
        "Rheumatology Department, Faculty of Medicine, Suez Canal University, Ismailia, Egypt"
      ],
      "name": "Gehad Elsehrawy"
    },
    {
      "affiliations": [
        "Rheumatology Department, Faculty of Medicine, Benha University, Kalyoubia, Egypt"
      ],
      "name": "Rasha Fawzy"
    },
    {
      "affiliations": [
        "Rheumatology Department, Faculty of Medicine, Alexandria University, Alexandria, Egypt"
      ],
      "name": "Yousra Abdel Fattah"
    },
    {
      "affiliations": [
        "Rheumatology and Rehabilitation Department, Faculty of Medicine, Capital University, Cairo, Egypt"
      ],
      "name": "Nesrin A Aboeladl"
    },
    {
      "affiliations": [
        "Health Radiation Research Department, Rheumatology Clinic, NCRRT, Egyptian Atomic Energy Authority (EAEA), Cairo, Egypt"
      ],
      "name": "Dina F El-Essawi"
    },
    {
      "affiliations": [
        "Rheumatology Department, Faculty of Medicine, Beni-Suef University, Beni-Suef, Egypt"
      ],
      "name": "Enas A Abdelaleem"
    },
    {
      "affiliations": [
        "Internal Medicine Department, Rheumatology Unit, Faculty of Medicine, Alexandria University, Alexandria, Egypt"
      ],
      "name": "Asmaa Beltagy"
    },
    {
      "affiliations": [
        "Rheumatology Department, Faculty of Medicine, Cairo University, Cairo, Egypt"
      ],
      "name": "Yasser Emad"
    },
    {
      "affiliations": [
        "Rheumatology Department, Faculty of Medicine, Cairo University, Cairo, Egypt"
      ],
      "name": "Tamer A Gheita"
    }
  ],
  "title": "Systemic sclerosis spectrum from the Egyptian College of Rheumatology: a nationwide observational study",
  "uid": "13d2e1fe-4ee7-5a71-8292-340b8228e9be"
}