The successful treatment of IgG4-positive colitis with adalimumab in a patient with IgG4-related sclerosing disease--a new subtype of aggressive colitis?
We present the case of a 16 year 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 13 year. 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.
Department of Gastroenterology, Hampshire Hospitals NHS Foundation Trust, Winchester, United Kingdom.
Mani
M
Naghibi
Adil
A
Ahmed
Adnan M
AM
al Badri
Adrian C
AC
Bateman
Hugh A
HA
Shepherd
John N
JN
Gordon
D000068879:Adalimumab; D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D015415:Biomarkers / Q000378:metabolism; D003092:Colitis / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism*
D000893:Anti-Inflammatory Agents; D061067:Antibodies, Monoclonal, Humanized; D015415:Biomarkers; D007074:Immunoglobulin G; D000068879:Adalimumab