title: A case of macrophage activation syndrome developing in a patient with chronic granulomatous disease-associated colitis.
abstract: Although macrophage activation syndrome (MAS) develops in some patients with chronic granulomatous disease (CGD), all of the reported cases have been associated with pathogenic microbial infections. We report a 2-year-old boy with CGD-associated colitis who suffered from MAS without any clinical signs of a microbial infection. He was treated with 1 course of methylprednisolone pulse therapy and the clinical symptoms improved; however, the colitis was difficult to control even with immunosuppressive drugs, and he eventually required hematopoietic stem cell transplantation 1 year after the onset of MAS. It is likely that MAS develops in patients with CGD colitis independent of microbial infections.
authors: Departments of *Interdisciplinary Medicine †Human Genetics ‡Division of Gastroenterology, National Center for Child Health and Development §Department of Pediatrics, The Jikei University School of Medicine, Tokyo, Japan.
authors: Kazuko
authors: K
authors: Akagi
authors: Toshinao
authors: T
authors: Kawai
authors: Nobuyuki
authors: N
authors: Watanabe
authors: Midori
authors: M
authors: Yokoyama
authors: Katsuhiro
authors: K
authors: Arai
authors: Shizuko
authors: S
authors: Harayama
authors: Shinji
authors: S
authors: Oana
authors: Masafumi
authors: M
authors: Onodera
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002675:Child, Preschool; D003092:Colitis / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D003131:Combined Modality Therapy; D006105:Granulomatous Disease, Chronic / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D055501:Macrophage Activation Syndrome / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use*; D011379:Prognosis
chemical_list: D000893:Anti-Inflammatory Agents; D008775:Methylprednisolone