Bing–Neel Syndrome: An Unknown GCA Mimicker

Giant cell arteritis (GCA) is a chronic granulomatous vasculitis of medium and large arteries leading to cranial and extracranial manifestations. Temporal artery biopsy is considered the gold standard; however, its sensitivity is low at 47%. We report a unique case of Bing–Neel Syndrome (BNS) presen...

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Main Authors: Arifa Javed, Sadia Arooj Javed, Barbara Ostrov, Jiang Qian, Khoa Ngo
Format: Article
Language:English
Published: Wiley 2024-01-01
Series:Case Reports in Rheumatology
Online Access:http://dx.doi.org/10.1155/2024/2043012
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author Arifa Javed
Sadia Arooj Javed
Barbara Ostrov
Jiang Qian
Khoa Ngo
author_facet Arifa Javed
Sadia Arooj Javed
Barbara Ostrov
Jiang Qian
Khoa Ngo
author_sort Arifa Javed
collection DOAJ
description Giant cell arteritis (GCA) is a chronic granulomatous vasculitis of medium and large arteries leading to cranial and extracranial manifestations. Temporal artery biopsy is considered the gold standard; however, its sensitivity is low at 47%. We report a unique case of Bing–Neel Syndrome (BNS) presenting as biopsy-proven GCA. BNS is a rare complication (1%) of Waldenstrom Macroglobulinemia (WM), which results from infiltration of lymph plasmacytoid cells and plasma cells into the central nervous system. A 77-year-old female with a past medical history of glaucoma, hypertension, diabetes, and chronic ocular ischemic syndrome in her right eye presented with progressive left eye vision loss for 5 days. Fundoscopic examination was notable for pseudophakic pseudopallor but no optic disc edema. Intraocular pressure was >40 and normalized after acetazolamide. The patient was started on pulse dose steroids by her neuro-ophthalmologist. She was discharged home on 60 mg of prednisone. At follow up with her neuro-ophthalmologist, new dot blot hemorrhages in the left eye were noted and she was readmitted for pulse dose of intravenous methylprednisolone. Temporal artery biopsy was consistent with GCA spectrum. Work up revealed paraproteinemia and subsequent bone marrow biopsy demonstrated WM. The patient was treated for her WM and her ophthalmic complications stabilized.
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spelling doaj-art-e31a3413d1ce4b528837890f77911dac2025-01-03T01:43:58ZengWileyCase Reports in Rheumatology2090-68972024-01-01202410.1155/2024/2043012Bing–Neel Syndrome: An Unknown GCA MimickerArifa Javed0Sadia Arooj Javed1Barbara Ostrov2Jiang Qian3Khoa Ngo4Albany Medical CenterPunjab Medical CollegeAlbany Medical CenterAlbany Medical CenterAlbany Medical CenterGiant cell arteritis (GCA) is a chronic granulomatous vasculitis of medium and large arteries leading to cranial and extracranial manifestations. Temporal artery biopsy is considered the gold standard; however, its sensitivity is low at 47%. We report a unique case of Bing–Neel Syndrome (BNS) presenting as biopsy-proven GCA. BNS is a rare complication (1%) of Waldenstrom Macroglobulinemia (WM), which results from infiltration of lymph plasmacytoid cells and plasma cells into the central nervous system. A 77-year-old female with a past medical history of glaucoma, hypertension, diabetes, and chronic ocular ischemic syndrome in her right eye presented with progressive left eye vision loss for 5 days. Fundoscopic examination was notable for pseudophakic pseudopallor but no optic disc edema. Intraocular pressure was >40 and normalized after acetazolamide. The patient was started on pulse dose steroids by her neuro-ophthalmologist. She was discharged home on 60 mg of prednisone. At follow up with her neuro-ophthalmologist, new dot blot hemorrhages in the left eye were noted and she was readmitted for pulse dose of intravenous methylprednisolone. Temporal artery biopsy was consistent with GCA spectrum. Work up revealed paraproteinemia and subsequent bone marrow biopsy demonstrated WM. The patient was treated for her WM and her ophthalmic complications stabilized.http://dx.doi.org/10.1155/2024/2043012
spellingShingle Arifa Javed
Sadia Arooj Javed
Barbara Ostrov
Jiang Qian
Khoa Ngo
Bing–Neel Syndrome: An Unknown GCA Mimicker
Case Reports in Rheumatology
title Bing–Neel Syndrome: An Unknown GCA Mimicker
title_full Bing–Neel Syndrome: An Unknown GCA Mimicker
title_fullStr Bing–Neel Syndrome: An Unknown GCA Mimicker
title_full_unstemmed Bing–Neel Syndrome: An Unknown GCA Mimicker
title_short Bing–Neel Syndrome: An Unknown GCA Mimicker
title_sort bing neel syndrome an unknown gca mimicker
url http://dx.doi.org/10.1155/2024/2043012
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AT barbaraostrov bingneelsyndromeanunknowngcamimicker
AT jiangqian bingneelsyndromeanunknowngcamimicker
AT khoango bingneelsyndromeanunknowngcamimicker