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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Format: | Article |
Language: | English |
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Babol University of Medical Sciences
2022-01-01
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Series: | Caspian Journal of Internal Medicine |
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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. |
first_indexed | 2024-12-20T06:25:54Z |
format | Article |
id | doaj.art-6636e010e7484f5086643d464a25f18e |
institution | Directory Open Access Journal |
issn | 2008-6164 2008-6172 |
language | English |
last_indexed | 2024-12-20T06:25:54Z |
publishDate | 2022-01-01 |
publisher | Babol University of Medical Sciences |
record_format | Article |
series | Caspian Journal of Internal Medicine |
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 |