The many faces of IG4 related disease: two unusual case presentation

Author(s): Nehal�??El Ghobashy, Esraa seif, Rana Hussein, Geilan Mahmoud

First case: Systemic Lupus Erythematosus (SLE) overlap with IgG4 related orbital disease (IgG4 ROD): A 25 –year-old female known case of SLE for the last 8 years diagnosed according 2012 SLICC classification criteria. She presented to us with recurrent left eye protrusion associated with pain and no any other local or systemic manifestations and showed incomplete response to corticosteroids. A computed tomographic scan of the orbit showed left dacryoadenitis. Lacrimal gland biopsy showed mixed acute and chronic inflammatory process with marked collagenization and sclerosis. Immunohistochemistry showed finding compatible with IgG4 related disease and serum level of IgG4 subclass was 156 mg/dl. Pulse intravenous methylprednisolone was administrated followed by rituximab with a dose of 2 g with 2 weeks apart with dramatic response and no recurrence throughout 8 months of follow up. Second case: IG4- related sclerosing cholangitis: A 65-year –old male patient presented with abdominal pain, picture of obstructive jaundice and marked weight loss. CT abdomen showed intrahepatic biliary dilation, hepatomegaly. ERCP showed stenosis in biliary tract after which stenting was done. 1-month later recurrence of symptoms. MRCP showed: Malfunctioning CBD stent with moderate intrahepatic biliary dilation with cholangitis .abrupt termination of the dilated biliary tree noted near the portahepatis region attributed to chronic inflammation and fibrosis. Picture of chronic pancreatitis. Liver biopsy showed: picture of cholestatic liver disease with portal tract fibrosis and relative increased IG4 plasma cells. Pulse intravenous methylprednisolone was administrated followed by mycophenolate mofetyl of a dose 2ag per day with marked improvement and no recurrence throughout 3-month follow up.