Dr. Sivaraman A, Dr. Ajay Narasimhan, Dr. Aravind Moorthy


IGG4-related disease is a newly recognized idiopathic sclerosing lesions with wide spectrum of manifestations
starting with autoimmune pancreatitis (AIP) ,a speci􀃶c type of chronic pancreatitis and an original member of the
IgG4-RD family ,sclerosing cholangitis,sialoadenitis,retroperitoneal 􀃶brosis,tubulointerstitial nephritis etc. In this report, we describe a case
of a 31-year old man with marked periaortic 􀃶brous thickening localized to the aortic arch initially presented only as vocal cord palsy.
Histologic examination revealed in􀃶ltration of lymphoplasmacytes and marked 􀃶brosis with numerous IgG4-positive plasma cells which are
hallmark of this disease. The serum concentration of IgG4 was 263 mg/dL,Twice the normal value.Treatment with steroid is well proven for
this spectrm of disorder.


Periaortitis,IgG4 disease

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