Topic: ESOPRS 2021 ePoster sessions
Time: Sep 17, 2021 16:00 Amsterdam, Berlin, Rome, Stockholm, Vienna, 15:00 London
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IgG4-related disease: an easily missed diagnosis
Author: Chia Ching Lin
ePoster Number: 237
To report a case whose diagnosis of IgG4-related dacryoadenitis is made until the third surgical excision.
A case report
A 65-year-old man was referred to our hospital for bilateral orbital tumors. There was bilateral ptosis with palpable masses at lacrimal fossa.
The patient underwent tumor biopsy for 2 times with both results showing lymphoid hyperplasia. Due to progressive ptosis and diplopia, he underwent bilateral lacrimal tumor excision under general anesthesia. The pathology showed IgG4-positive plasma cells more than 100/HPF. The IgG4/IgG-positive plasma cell ratio was >40%. IgG4-related disease was the final diagnosis.
The serum IgG level was 982 mg/dL (normal range: 3~135 mg/dL). The patient started prednisolone 10 mg and azathioprine 50mg oral therapy per day. The serum IgG level decreased to 98 mg/dL after 8 months. There was no recurrence of tumor during 2 years of follow-up.
IgG4-related disease is a fibro-inflammatory condition that can cause major salivary and lacrimal gland enlargement, orbital disease, autoimmune pancreatitis, retroperitoneal fibrosis and tubulointerstitial nephritis. Serum IgG subclass test are helpful to this diagnosis, but a firm histological diagnosis is essential. The histopathological features are lymphoplasmacytic infiltrate enriched with IgG4-positive plasma cells (with an IgG4/IgG ratio >40%), storiform fibrosis, and obliterative phlebitis.
In our case, the final diagnosis was made until the third excision. We suggest that when there is diagnostic uncertainty after biopsy of lacrimal tumors, IgG4-related disease should be considered and serum test for IgG4 should be done as well as reviewing and immunostaining of previous pathology slides.
IgG4-related disease is a disease of underdiagnosis. We should consider this disease when treating patients with dacryoadenitis or orbital tumors. Serum IgG4 test and careful pathology review with immunostaining are essential for the diagnosis.
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Abstract ID: 21-131