{
  "abstract": "Introduction The gastrointestinal tract is the second most commonly affected organ system in systemic sclerosis (SSc). Any segment of the gastrointestinal tract—from the oral cavity to the anorectum—may be involved. Approximately 90% of patients exhibit some degree of esophageal dysmotility. Small bowel hypomotility and small intestinal bacterial overgrowth are also common, leading to malabsorption, malnutrition, and increased mortality (1). Gastrointestinal manifestations of SSc frequently demonstrate limited therapeutic response to standard immunomodulatory regimens. A recent literature review (2024) suggests that intravenous immunoglobulins (IVIG) may offer significant therapeutic benefit in gastrointestinal involvement of SSc (2).Aim To present the clinical efficacy of IVIG therapy in a patient with SSc and pronounced gastrointestinal involvement.Material and Methods A 33-year-old woman with ANA 1:2560 and anti-fibrillarin positivity, puffy fingers, Raynaud’s phenomenon, fingertip ulcerations, facial telangiectasias, megacapillaries with microhemorrhages on capillaroscopy, and arthralgias was diagnosed with SSc. Initial treatment included antimalarials, mycophenolate mofetil, and vasodilators. At follow-up, she reported a 5 kg weight loss over several months, accompanied by severe bloating and gastroesophageal reflux.Esophageal manometry revealed absent motility, while gastroscopy demonstrated Los Angeles grade B esophagitis with a hiatal hernia. Ultrasound of the esophagus and small intestine showed marked esophageal dysmotility with repetitive back-and-forth movements, fluid stagnation in ileal loops, and reduced peristalsis. IVIG was added to her existing therapy at an immunomodulatory dose (2 g/kg/month) administered monthly for one year.After six months, her gastrointestinal symptoms had resolved and she experienced weight gain. After one year of therapy, manometry was normal, gastroscopy showed improvement to Los Angeles grade A esophagitis, and ultrasound demonstrated resolution of esophageal dysmotility. Small bowel peristalsis remained mildly reduced but without fluid retention. Cutaneous improvement was also noted, with no recurrence of digital ulcers and a marked reduction in telangiectasias.Results In this patient, gastrointestinal involvement was a dominant clinical feature of SSc. Monthly IVIG therapy at a dose of 2 g/kg produced a favorable therapeutic response.Conclusions IVIG represents a valuable add-on therapy for systemic manifestations of SSc that respond poorly to conventional immunosuppressive treatment. Recent literature supports the therapeutic efficacy and safety of IVIG, particularly in gastrointestinal and cutaneous involvement of SSc (2).",
  "authors": [
    {
      "affiliations": [
        "Department of Rheumatology, Internal Medicine Clinic, Clinical Center of Montenegro, Podgorica, Montenegro"
      ],
      "name": "Milica Markovic Vlaisavljevic"
    },
    {
      "affiliations": [
        "Department of Rheumatology, Internal Medicine Clinic, Clinical Center of Montenegro, Podgorica, Montenegro"
      ],
      "name": "Natasa Miketic"
    },
    {
      "affiliations": [
        "Department of Rheumatology, Internal Medicine Clinic, Clinical Center of Montenegro, Podgorica, Montenegro"
      ],
      "name": "Milan Bogojevic"
    },
    {
      "affiliations": [
        "Department of Rheumatology, Internal Medicine Clinic, Clinical Center of Montenegro, Podgorica, Montenegro"
      ],
      "name": "Rifat Medjedovic"
    },
    {
      "affiliations": [
        "Department of Experimental and Clinical Medicine, University of Florence, and Division of Internal Medicine AOUC, Florence, Italy"
      ],
      "name": "Giulia Bandini"
    },
    {
      "affiliations": [
        "Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milano, Italy"
      ],
      "name": "Marco Matucci Cerinic"
    }
  ],
  "title": "P.162 Assessment of the effectiveness of intravenous immunoglobulin therapy in gastrointestinal manifestations of systemic sclerosis",
  "uid": "4476057b-e039-5698-adcb-d804f1f635e6"
}