title: Benign pneumatosis intestinalis in a pediatric patient with multiple risk factors including granulomatosis with polyangiitis: a case report and review of the literature.
abstract: OBJECTIVE
  To describe the first reported case of pneumatosis intestinalis (PI) in a pediatric patient with granulomatosis with polyangiitis (GPA) and multiple other risk factors and review the literature for PI in adult and pediatric rheumatologic conditions.
  
  
  METHODS
  A PubMed search was completed using the search phrase "pneumatosis intestinalis." Searches were limited to humans and the English language, and remaining articles involving patients with rheumatologic diagnoses were identified and included in our discussion.
  
  
  RESULTS
  This is the first reported of case of PI in a patient with underlying GPA or antineutrophil cytoplasmic antibody-associated vasculitides. Out of 90 previously reported cases of PI in patients with rheumatologic conditions, 79 cases were in adults and 11 in children. There were 30 patients with systemic sclerosis, 18 with MCTD/overlap syndrome, 18 with dermatomyositis or polymyositis, 16 with SLE, and 8 with other diagnoses. Overall, 81% of the patients were on corticosteroids or other immunosuppressants prior to development of PI. The most common presenting symptom was abdominal pain, and 51% of patients had associated pneumoperitoneum.
  
  
  CONCLUSIONS
  PI can be associated with a broad spectrum of rheumatic diseases, including GPA, and should be included in the differential diagnosis of patients with rheumatologic conditions and nonspecific gastrointestinal symptoms.
authors.affiliation: Division of Rheumatology, Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA. Electronic address: caroline.y.chang@gmail.com.
authors.forename: Caroline Y
authors.initials: CY
authors.lastname: Chang
authors.affiliation: Division of Rheumatology, Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA.
authors.forename: Katherine A
authors.initials: KA
authors.lastname: Marzan
mesh_terms: D000293:Adolescent; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D000935:Antifungal Agents / Q000627:therapeutic use; D015897:Comorbidity; D004359:Drug Therapy, Combination; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology*; D006801:Humans; D008727:Methotrexate / Q000627:therapeutic use; D011006:Pneumatosis Cystoides Intestinalis / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology*; D011241:Prednisone / Q000627:therapeutic use; D012307:Risk Factors; D016896:Treatment Outcome
chemical_list: D000900:Anti-Bacterial Agents; D000935:Antifungal Agents; D011241:Prednisone; D008727:Methotrexate
keywords: Children; Granulomatosis with polyangiitis; Pediatric; Pneumatosis intestinalis