¹Lubna Nazir, ¹Hammad Ali Khan, ¹Syeda Rida e Zehra, ¹Tahira Perveen Umer
¹Liaquat National Hospital and Medical College

Purpose Statement(s):

‘IgG4 – related disease’ is one of the recently discovered systemic diseases which is immune mediated and results in tumor like masses or fibrosis of different organs, frequent ones being the pancreas, bile duct, orbital cavity, salivary glands, ureters, kidneys, aorta, lungs, lymph nodes and thyroid gland. There is growing evidence of its autoimmune basis (roles of both B and T cells). Being an uncommon condition with a huge variety of presenting complains, more case reports/ studies are required in order to provide more clues regarding the disease. This case report describes an unusual presentation of this disease and that too in a developing country, where it has not been reported much.

Methods(s):

An 18 years old female, resident of a remote area in Pakistan, presented in maxillofacial OPD with oral swellings. The onset was gradual, initially small but progressively increased in size, were painless, with no bleeding, discharge or redness. There was no history of trauma or recent dental intervention nor were there any associated dental issues. There was no history of TB or TB contact. There was a past history of similar swellings about three years back (biopsy of which was non-specific).

After base-line work-up, a CT scan head and neck was performed which did not reveal any significant finding. Thereafter, surgical excision of the swellings was performed and the specimen was sent for biopsy which showed fibrosis with a typical storiform pattern and lymphoplasmacytic infiltrates.

Result(s): 

The histopathologic findings together with the clinical presentation were highly suggestive of IgG4 – related disease. IgG4 levels were advised which however came out to be normal. Since criteria was met (clinical presentation + biopsy), patient was diagnosed to have IgG4 – related disease.

Conclusion(s):
This case emphasizes on the fact that IgG4-related disease can have any unique presentation involving people of any age, race or gender. Whenever a patient presents with a swelling involving any anatomical location, this disease must be in the list of differential diagnosis.This case report also sheds light on the fact that IgG4 levels can be normal with the biopsy being typical of IgG4 – rd.

Lastly, it reflects the need of further cases to be reported in order to enhance knowledge of the medical community regarding the wide spectrum of features of this disease which would aid in diagnosis and further management.