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TitreBirdshot-like chorioretinopathy in lymphoid interstitial pneumonia due to hypogammaglobulinopathy
Abstract Nr.134
ButTo describe a patient with a birdshot-like choroidoretinopathy secondary to a hypogammaglobulinopathy, complicated with lymphoid interstitial pneumonia and persistent lymphadenopathy.
MéthodesAn 18 - year old girl presented with an asymptomatic bilateral papillary oedema and chorioretinal infiltrates. These findings were noted during routine follow-up for hydroxychloroquin (HCQ) use. She underwent a full ophthalmological and systemic work-up, including electroretinography and fluorescein and ICG angiographies.
RésultatsThis patient was diagnosed with a lymphoid interstitial pneumonia due to a hypogammaglobulinopathy at the age of 13. Since then she’s treated with monthly intravenous immunoglobulines and oral HCQ. Annual visits to exclude hydroxychloroquine toxicity revealed no abnormalities until 2 years ago.
One year ago visual acuity was 12/10 without correction in both eyes. Slit-lamp examination was unremarkable. However, fundoscopy revealed bilateral papilloedema and chorioretinal infiltrates with a birdshot-like appearance. On fluo-angiography a bilateral hot disc was present, whilst the chorioretinal infiltrates were hypofluorescent on ICG. ERG and colour vision were normal. MRI brain revealed no signs of raised intracranial pressure.
HLA- A29 analysis was negative, as were the blood levels of ACE.
ConclusionHypogammaglobulinopathy complicated with lymphoid interstitial pneumonia may present with an HLA-A29 negative phenocopy of Birdshot choroidoretinopathy.
Auteur 1
NomDE MAEYER
InitialesV
InstitutDept of Ophthalmology, Ghent University Hospital
VilleGhent
Auteur 2
NomKESTELYN
InitialesP
InstitutDept of Ophthalmology, Ghent University Hospital
VilleGhent
Auteur 3
NomHAERYNCK
InitialesF
InstitutDept of Paediatrics, Ghent University Hospital
VilleGhent
Auteur 4
NomDE BAETS
InitialesF
InstitutDept of Paediatrics, Ghent University Hospital
VilleGhent
Auteur 5
NomLEROY
InitialesBP
InstitutDept of Ophthalmology, Ghent University Hospital
VilleGhent
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