Which of the following is incorrect about IgG4-related sclerosing disease?
A. It is associated with sclerosing pancreatitis and sclerosing cholangitis, and it can also involve the salivary gland, retroperitoneum (and hence periaortitis and obstructive uropathy), kidneys, lung and prostate
B. Serum total IgG and IgG4 levels are raised.
C. It is steroid reponsive, and may recure if steroid is tailed down or stopped.
D. It is also associated with raised serum IgM.
Answer: D
It has been reported that patients with IgG4-related inflammatory abdominal aortic aneurysms frequently showed an allergic condition, such as bronchial asthma, drug allergy and high serum IgE concentrations, the clinical significance of the abnormally high levels of serum IgE remains unknown.
Reference
1. Hideaki Hamano, M.D., Shigeyuki Kawa, M.D., Akira Horiuchi, M.D., Hiroshi Unno, M.D., Naoyuki Furuya, M.D., Taiji Akamatsu, M.D., Mana Fukushima, M.D., Toshio Nikaido, Ph.D., Kohzo Nakayama, Ph.D., Nobuteru Usuda, M.D., and Kendo Kiyosawa, M.D. High Serum IgG4 Concentrations in Patients with Sclerosing Pancreatitis. NEJM Volume 344:732-738 March 8, 2001 Number 10
2. Hideaki Miura, Yasutaka Miyachi. IgG4-Related Retroperitoneal Fibrosis and Sclerosing Cholangitis Independent of Autoimmune Pancreatitis. A Recurrent Case after a 5-Year History of Spontaneous RemissionJOP. J Pancreas (Online) 2009; 10(4):432-437. (available free online here)