Titre | Birdshot-like chorioretinopathy in lymphoid interstitial pneumonia due to hypogammaglobulinopathy |
Abstract Nr. | 134 |
But | To describe a patient with a birdshot-like choroidoretinopathy secondary to a hypogammaglobulinopathy, complicated with lymphoid interstitial pneumonia and persistent lymphadenopathy. |
Méthodes | An 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. |
Résultats | This 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. |
Conclusion | Hypogammaglobulinopathy complicated with lymphoid interstitial pneumonia may present with an HLA-A29 negative phenocopy of Birdshot choroidoretinopathy. |
Nom | DE MAEYER |
Initiales | V |
Institut | Dept of Ophthalmology, Ghent University Hospital |
Ville | Ghent |
Nom | KESTELYN |
Initiales | P |
Institut | Dept of Ophthalmology, Ghent University Hospital |
Ville | Ghent |
Nom | HAERYNCK |
Initiales | F |
Institut | Dept of Paediatrics, Ghent University Hospital |
Ville | Ghent |
Nom | DE BAETS |
Initiales | F |
Institut | Dept of Paediatrics, Ghent University Hospital |
Ville | Ghent |
Nom | LEROY |
Initiales | BP |
Institut | Dept of Ophthalmology, Ghent University Hospital |
Ville | Ghent |