The successful treatment of IgG4-positive colitis with adalimumab in a patient with IgG4-related sclerosing disease – a new subtype of aggressive colitis?

• Published in: Journal of Crohn's and colitis 2013-04, Vol.7 (3), p.e81-e84
• Main Authors: Naghibi, Mani, Ahmed, Adil, al Badri, Adnan M., Bateman, Adrian C., Shepherd, Hugh A., Gordon, John N.
• Format: Article
• Language: English
• Subjects: Adalimumab; Adolescent; Anti-Inflammatory Agents - therapeutic use; Antibodies, Monoclonal, Humanized - therapeutic use; Autoimmune Diseases - diagnosis; Autoimmune Diseases - drug therapy; Autoimmune Diseases - immunology; Biomarkers - metabolism; Colitis; Colitis - diagnosis; Colitis - drug therapy; Colitis - immunology; Female; Humans; IgG4 sclerosing disease; Immunoglobulin G - metabolism; Pancreatitis
• ISSN: 1873-9946; 1876-4479
• DOI: 10.1016/j.crohns.2012.05.003

We present the case of a 16year old girl who developed an aggressive colitis in the context of a prior biopsy proven autoimmune pancreatitis, which presented with obstructive jaundice at the age of 13year. This history prompted prospective investigation and the discovery of compelling evidence to make a diagnosis of IgG4-related sclerosing disease with extra-pancreatic colonic involvement on the basis of raised serum IgG4 levels and a florid colonic IgG4 plasma cell infiltrate with over 20 IgG4 positive plasma cells/hpf. The colitis was resistant to conventional therapy but responded dramatically to treatment with the anti-TNFα monoclonal antibody, adalimumab. This is the first case to report both the effectiveness of adalimumab in treating IgG4 positive colitis in a patient with IgG4-related sclerosing disease, and to prospectively record resolution of an IgG4 positive colonic infiltrate with immunosuppression.