Background IgG4-related diseases are rare fibroinflammatory processes rich in IgG4-positive plasma cells, which may involve any organ, leading to persistent infiltration, dysfunction and stenotic complications. Many aspects of this disorder (such as etiopathogenesis or optimal management scheme) are still to be elucidated. The low incidence of this condition hampers large control studies, so case series might be helpful in this context.
Objectives To describe our experience dealing with IgG4-related disease in a single Spanish centre with special interest in connective tissue diseases
Methods We underwent an audit of IgG4-related disease cases currently followed in our Department. Histopathological-proven disease based on currently accepted criteria  was required. Demographic, clinical, pathological, radiological, management and outcome features were recorded.
Results We identified 3 patients with IgG4-related disease, two females and a male, whose ages were between 44 and 58 years. Clinical presentations were as retroperitoneal fibrosis, orbital pseudotumor and inflammatory abdominal aortic aneurism. The follow up in our department was 30, 3 and 1 month, respectively. No other systemic autoimmune disease was identified, but both females had autoantibody-negative thyroid disorders. Serum IgG4 levels were normal in all cases (median 83.7 mg/dL, normal <140). 18FDG-PET did identify a distant involvement previously undetected in the patient with retroperitoneal fibrosis, becoming negative after treatment. All patients required surgery at the onset: the patient with retroperitoneal fibrosis underwent ureterolysis plus external iliac artery bypass, the patient with orbital pseudotumor a resection due to malign exophtalmos, and the patient with inflammatory aortic aneurism resection plus aortoiliac bypass. After surgery, all patients received prednisone plus mycophenolate mofetil 2gr/day, associating with control of the inflammatory process in terms of clinical, imaging (18FDG-PET) and serum inflammatory markers.
Conclusions IgG4-related disease is a recently characterised disorder, probably underdiagnosed, and with many aspects still to be clarified. In our small case series, prednisone plus mycophenolate seemed a promising management scheme for these patients.
Deshpande V. Mod Pathol. 2012; 25:1181-92.
Acknowledgements To Dr Miguel Trigueros, pathologist, and our colleagues from Vascular Surgery department.
Disclosure of Interest None Declared