{
  "abstract": "Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibro-inflammatory condition with an unclear pathophysiological mechanism affecting different parts of the body. If untreated, the disease can lead to fibrosis and irreversible organ damage.14 year old girl, previously fit and well was transferred from local hospital with abdominal pain, temperatures, diarrhoea, weight loss (5–6 kg) over 2 months. Her general and systemic examination was essentially normal. She had raised inflammatory markers with a C-reactive protein of 182 mg/Land erthyrocyte sedimentation rate of 30 mm/hr. Her liver function tests were normal with albumin being lowest at 27 g/. She was empirically treated with broad spectrum antibiotics.MRI of abdomen revealed significant omental thickening with ascites. Tuberculosis and malignancy were ruled out. Initial ascitic tap was normal and histopathology – was suggestive of omental panniculitis with predominant eosinophilic cells. Initial test for autoimmune disease were negative. Subsequent MDTs identified the need to repeat the biopsy and ascitic tap (both diagnostic and therapeutic) with upper GI endoscopy, capsule study and a Bone marrow. Budd Chiari was ruled out by venography as there was high flow in hepatic vein confluence which was probably due to the pressure effect of ascites.Repeat ascitic fluid SAAG was 0.4 g/dl confirming an exudate , cell count could not be done as sample clotted and histopathology was suggestive of sclerosis/fibrosis with plasma cells .IgG4 staining was 40% and serum IgG4 was normal. Stains for IMT (inflammatory myofibroblastic tumour) were negative and genetics awaited for IMT and familial mediterranean fever gene.She improved clinically with resolution of inflammatory markers at the time of second biopsy. She is asymptomatic at present. After extensive review of literature and discussion with a quaternary centre she was diagnosed as IgG4 related disease. In this case her condition resolved spontaneously without the need for immunosuppression.IgG4-related disease (IgG4-RD) is a chronic, systemic, and autoinflammatory disease mediated by the immune system.Gastrointestinal presentation generally involves pancreas.In this case it was an isolated involvement of omentum with a pleural effusion.Abstract OC38 Figure 1This presentation is rare. There are no reported cases of omental involvement as presentation of this disease in children.Abstract OC38 Figure 2",
  "authors": [
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Sunita Amar Rajani"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Dominique Schluckebier"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Ashok Raghavan"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Marta Cohen"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Sona Matthai"
    }
  ],
  "title": "OC38 IgG4-related disease with gastrointestinal involvement: a rare presentation in children",
  "uid": "bcddca1e-5662-5173-9b16-8a43a18140de"
}