Ocular Manifestation in a patient with IgG4 Related Disease

Background: IgG4-related disease is a newly recognized fibroinflammatory disease presenting with multiple features including mass forming lesion; a dense lymphoplasmacytic infiltrate; a characteristic histopathological appearance and often elevated serum of IgG4. This disease can potentially affect...

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Main Authors: Samaneh Azizianesh, Fatema Rizvi, Hamed Zainaldain, Samira Alesaeidi
Format: Article
Language:English
Published: Babol University of Medical Sciences 2022-01-01
Series:Caspian Journal of Internal Medicine
Subjects:
Online Access:http://caspjim.com/article-1-1896-en.html
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author Samaneh Azizianesh
Fatema Rizvi
Hamed Zainaldain
Samira Alesaeidi
author_facet Samaneh Azizianesh
Fatema Rizvi
Hamed Zainaldain
Samira Alesaeidi
author_sort Samaneh Azizianesh
collection DOAJ
description Background: IgG4-related disease is a newly recognized fibroinflammatory disease presenting with multiple features including mass forming lesion; a dense lymphoplasmacytic infiltrate; a characteristic histopathological appearance and often elevated serum of IgG4. This disease can potentially affect any organ and interestingly, the affected organs share common histopathological features including a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and mild to moderate eosinophilia Case Presentation: A 45-year-old man presented complaining of proptosis and gradual decrease in visual acuity of right eye. He had undergone many work-ups but without any definitive diagnosis. Through a combination of clinical and para-clinical investigations, the diagnosis of IgG4-RD was established. 693 mg/dL). Aggressive treatment (pulse of cyclophosphamide and pulse of corticosteroid) was started hoping to save the patient’s vision. Two weeks following the treatment, there was improvement with his visual acuity and proptosis. Conclusion: In any patient with chronic tumor like lesions and pseudotumors without the evidence of malignancy, we should think of IgG4-related disease. In this circumstance, biopsy may lead us to the definitive diagnosis. Early diagnosis and treatment of IgG4-RD may inhibit further irreversible organ damages.
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spelling doaj.art-6636e010e7484f5086643d464a25f18e2022-12-21T19:50:18ZengBabol University of Medical SciencesCaspian Journal of Internal Medicine2008-61642008-61722022-01-01131127131Ocular Manifestation in a patient with IgG4 Related DiseaseSamaneh Azizianesh0Fatema Rizvi1Hamed Zainaldain2Samira Alesaeidi3 Research Center for Rheumatologic Diseases, Amir-Alam Hospital, Tehran University of Medical Sciences, Tehran, Iran School of medicine, Tehran University of Medical Science, Tehran, Iran School of medicine, Tehran University of Medical Science, Tehran, Iran Department of Rheumatology, Amir-Alam Hospital, Tehran, Iran Background: IgG4-related disease is a newly recognized fibroinflammatory disease presenting with multiple features including mass forming lesion; a dense lymphoplasmacytic infiltrate; a characteristic histopathological appearance and often elevated serum of IgG4. This disease can potentially affect any organ and interestingly, the affected organs share common histopathological features including a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and mild to moderate eosinophilia Case Presentation: A 45-year-old man presented complaining of proptosis and gradual decrease in visual acuity of right eye. He had undergone many work-ups but without any definitive diagnosis. Through a combination of clinical and para-clinical investigations, the diagnosis of IgG4-RD was established. 693 mg/dL). Aggressive treatment (pulse of cyclophosphamide and pulse of corticosteroid) was started hoping to save the patient’s vision. Two weeks following the treatment, there was improvement with his visual acuity and proptosis. Conclusion: In any patient with chronic tumor like lesions and pseudotumors without the evidence of malignancy, we should think of IgG4-related disease. In this circumstance, biopsy may lead us to the definitive diagnosis. Early diagnosis and treatment of IgG4-RD may inhibit further irreversible organ damages.http://caspjim.com/article-1-1896-en.htmligg4-related diseaseigg4-related orbitopathyvisual acuityproptosis.
spellingShingle Samaneh Azizianesh
Fatema Rizvi
Hamed Zainaldain
Samira Alesaeidi
Ocular Manifestation in a patient with IgG4 Related Disease
Caspian Journal of Internal Medicine
igg4-related disease
igg4-related orbitopathy
visual acuity
proptosis.
title Ocular Manifestation in a patient with IgG4 Related Disease
title_full Ocular Manifestation in a patient with IgG4 Related Disease
title_fullStr Ocular Manifestation in a patient with IgG4 Related Disease
title_full_unstemmed Ocular Manifestation in a patient with IgG4 Related Disease
title_short Ocular Manifestation in a patient with IgG4 Related Disease
title_sort ocular manifestation in a patient with igg4 related disease
topic igg4-related disease
igg4-related orbitopathy
visual acuity
proptosis.
url http://caspjim.com/article-1-1896-en.html
work_keys_str_mv AT samanehazizianesh ocularmanifestationinapatientwithigg4relateddisease
AT fatemarizvi ocularmanifestationinapatientwithigg4relateddisease
AT hamedzainaldain ocularmanifestationinapatientwithigg4relateddisease
AT samiraalesaeidi ocularmanifestationinapatientwithigg4relateddisease