A Challenge For All
In this episode, Dr. Daniel Ennis presents a challenging case that has developed over an extended amount of time. The group discusses treatment methods, relevant research as well as the age old question of “what would you do, if this was you?” How aggressive should treatments be, when the diagnoses is not guaranteed.. join us to find out!
St. Paul's Morning Report 
Did you ever solve the case? Was IgG4 disease ever considered? I know the igg subclasses were unremarkable but that doesnt rule ut out. There was parotid glans involvement. Was tissue examined for IgG4 and were plasmablast checked out?
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This is a really good thought and was indeed considered in this case. You are right that IgG4 disease often has normal IgG4 serum levels. Although it was not examined specifically for IgG4+ cells or for the IgG4+/IgG ratio, the lymph node biopsy performed at the time of valve replacement showed non-necrotizing granulomatous lymphadenitis and lacked the other hallmarks of IgG4 disease (storiform fibrosis, lymphoplasmacytic predominant infiltration, obliterative phlebitis, eosinophilia). The parotid gland involvement and skin rash were quite evanescent and could no longer be biopsied by the time the diagnostic dilemma arose. Additionally, stroke and intracerebral vascular involvement appear to be quite rare in IgG4 disease compared to its other CNS symptoms.
Let’s keep IgG4 on the list if her parotid gland swelling returns. A biopsy specifically looking for IgG4 disease is a good idea.
Thank you for your help!
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Any updates? Did the radiologist(s) comment whether it had the appearance of Moyamoya disease? There’s actually a case report of neurosarcoid presenting with that imaging appearance.
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Hi Kevin, thanks so much for your comment and for being part of our audience, we truly appreciate it. The group will be doing an ‘updates’ episode in the near future to address your question and a few others that we have received. Stay tuned!
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