Intestinal pseudo-obstruction due to IgG4-related disease

Esplugues Clos, Joan; Feijoo-Massó, Carlos; Solé, Cristina

doi:10.1016/j.medcli.2023.08.014

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This disease has been identified in multiple organs such as salivary glands and pancreas; however, peritoneal and mesenteric involvement is extremely rare.<a class="elsevierStyleCrossRef" href="#bib0030">1</a> After an exhaustive bibliography review (PUBMED/MEDLINE database, science direct and using the terms “intestinal obstruction” and “IgG4-RD” from the definition of the disease in 2012 to our days) only five cases of intestinal pseudo-obstruction due to IgG4-RD have been reported. Herein, we report an unusual case of ascites and intestinal pseudo-obstruction due to IgG4-RD with peritoneal involvement.A 70-year-old man presented with abdominal distension, nausea, vomiting, and anorexia for 2 months. His medical history included an alcohol use disorder and alcohol-associated liver disease. Physical examination revealed ascites and abnormal bowel movements. Laboratory tests showed leukocytosis of 14.82×109/L (normal range (NR): 4–11×109/L), with 83% neutrophils, alkaline phosphatase of 187U/L (NR: 40–129U/L), gamma-glutamyl transpeptidase of 193U/L (NR: 8–61U/L) and total bilirubin of 1.4mg/dL (NR: 0.1–1.3mg/dL). The remaining parameters of the analysis were within normal limits. Paracentesis was performed, and ascitic fluid analysis revealed 105 nucleated cells with 77% of mononuclear cells and proteins of 41g/L. Ascitic fluid cytology was negative for malignant cells. Autoimmunity tests (ANA, IgG-4, ANCA) were negative.Abdominal ultrasound showed signs of liver cirrhosis and ascites. Upper gastrointestinal endoscopy revealed a mass effect at the duodenum, suggesting extrinsic compression or a submucosal lesion. Gastroesophageal transit unveiled duodenal dilatation with minimal and delayed passage of the contrast to the jejunum. Upper enteroscopy with mucosal biopsies resulted normal. Liver biopsy was suggestive of alcohol-associated liver cirrhosis.Abdominal computed tomography (CT) showed increased mesenteric density with multiple nodules, diffuse thickening of the peritoneum, and ascites. A hybrid 18 fluoro-2-deoxy-D-glucose positron emission tomography/CT (FDG-PET/CT) was performed confirming mesenterial and peritoneal thickening with diffuse radiotracer uptake (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).<elsevierMultimedia ident="fig0005"></elsevierMultimedia>Parenteral nutrition was initiated, and exploratory laparoscopy was performed. Signs of inflammation of the peritoneal lining were observed. The small intestine had slow peristalsis, and could not be individualized, forming a compact block. Histopathology of the peritoneal biopsy showed lymphoplasmacytic infiltration with fibrosis and obliterative phlebitis. An immunohistochemical study for IgG and IgG4 was performed, detecting more than 30 positive IgG4 cells per field at high magnification with an IgG4/IgG ratio >60%. Therefore, the patient met the criteria of IgG4-RD with peritoneal involvement.<a class="elsevierStyleCrossRef" href="#bib0035">2</a> Treatment was started with boluses of 250mg of methylprednisolone for 5 days, and, in the absence of clinical improvement, two doses of rituximab 1000mg were administered 15 days apart.<a class="elsevierStyleCrossRef" href="#bib0040">3</a> The patient significantly improved; ascites and abdominal pain disappeared and oral intake was recovered. On successive FGD-PET/CT abdominal scans peritoneal thickening clearly improved.Intestinal pseudo-obstruction is a gastrointestinal motility disorder characterized by symptoms of intestinal obstruction in the absence of any anatomic obstruction. The diagnostic approach includes screening for causes of secondary pseudo-obstruction, such as paraneoplastic syndromes, neurological diseases, metabolic disorders, celiac disease, and IgG4-RD, among others.<a class="elsevierStyleCrossRefs" href="#bib0030">1,4</a>On the other hand, IgG4-RD is an immune-mediated condition characterized by widespread fibroinflammatory lesions. IgG4-RD can affect salivary glands, orbits, pancreas, retroperitoneum, bile duct, lacrimal glands, and aorta among others. This disease is presented most of the time as a multiorgan disease that can mimic other conditions such as infection disorders, other immune-mediated conditions or malignancy.Accurate diagnosis requires taking into account clinical, serological and radiological findings with immunostaining and histopathological findings once having ruled out more frequent diagnosis. Moreover, IgG4 levels are not necessary criteria for the diagnosis of the disease, being normal in up to one third of patients, making the diagnosis of the disease even harder.<a class="elsevierStyleCrossRef" href="#bib0035">2</a>Although there are limited studies regarding the treatment, it is recommended to start corticosteroids at high doses with a subsequent gradual reduction in order to limit inflammation and prevent fibrosis.<a class="elsevierStyleCrossRef" href="#bib0050">5</a> Immunosuppressive treatments such as rituximab can also be an effective treatment.<a class="elsevierStyleCrossRefs" href="#bib0035">2,3</a>This case illustrates an unusual form of IgG4-RD in a patient with cirrhosis that presented as an intestinal pseudo-obstruction and ascites. Despite the uncommon peritoneal involvement in IgG4-RD, early detection and treatment of this disease is essential to prevent significant organ damage from end-stage fibrosis. In these infrequent cases, histopathological findings remain the gold standard for the diagnosis of this disease. For this reason, we point out that all physicians should be aware of this systemic disease.Ethical approvalFor the realization of this article, the ethics committee of our hospital approved the data collection, and consent was signed by the patient.FundingNo funding was needed for this article.Conflict of interestsThe authors declare that they have no conflict of interest or personal relationships that could have influenced the work presented in this article." 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