Diagnostic and Therapeutic Challenges in IgG4-Related Disease in the Sphenoid Sinus
Immunoglobulin G4 (IgG4) related disease is a newly recognized fibro inflammatory condition characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis and often but not always, elevated serum IgG4 concentrations. We present a 38 years old female complaining of headache which was progressive and unilateral resistant to analgesics. Diagnosed as a case of migraine started anti migraine treatment with no benefit, it was further complicated by 3rd, 4th and 6th Cranial Palsy. Radiological finding of opacification of left sphenoid sinus with extension to the left cavernous sinus along with bone erosion was noted. The biopsy from the sphenoid sinus mass showed a moderately dense lymphoplasmacytic infiltration accompanied with fibrosis. Immunostaining for IgG4 show significant increase in IgG4-positive plasma cells with IgG4-IgG ratio > 40% and >100 IgG4 positive plasma cells per High Field Focus (HPF), giving the diagnosis of IgG4 sinusitis. This is a rare case of IgG4 related disease in the sphenoid sinus. Clinical, radiological and therapeutic challenges are discussed.