Advanced Cases of Birdshot HLA-A29 Retinochoroiditis: Prevalence and Characteristics

Abstract

Purpose To establish the prevalence, morphological, and functional characteristics and evolution of advanced cases of birdshot retinochoroiditis (BRC).

Methods A retrospective review of all BRC cases seen at the Centre for Ophthalmic Specialized Care, Lausanne, Switzerland, with at least 3 years of follow-up since the onset of symptoms. The following parameters were evaluated: demographic characteristics, best-corrected visual acuity (BCVA), fundus photographs, fluorescein (FA) and indocyanine green (ICGA) angiography frames, and visual field evaluation from initial visit to last follow-up.

Results Out of the 1920 patients with uveitis who were followed from 1995 until 2018, 32 (1.67%) were diagnosed with BRC; 20 of these 32 patients had sufficient data to be included. Three of these twenty patients (15.0%) had severe forms of the disease. Two patients had received insufficient treatment and one progressed despite attempted maximal therapy. The mean BCVA was 0.62 (± 0.42) at presentation, which was already at an advanced stage of the disease, and 0.51 (± 0.37) at last follow-up. The average visual field mean defect (VFMD) was 19.65 (± 4.71 dB) at presentation and 21.40 (± 2.45 dB) at last follow-up. Choroidal inflammatory activity monitored by ICGA decreased from 10.67 (± 9.18) at presentation to 6.67 (± 5.61) at last follow-up. FA revealed mild retinal vasculitis at 6.50 (± 4.76), which remained stable to 4.67 (± 3.20). EDI-OCT estimated choroidal thickness (EDI-OCT-CT) measurements were 246.36 (± 116.93 µm) at presentation and 231.30 (± 120.70 µm) at the last follow-up.

Conclusions In our setting, 15% of BRC cases had a severe course and suffered extensive irreversible chorioretinal damage, obviously due to insufficient treatment in two cases. This underlines the importance of correctly diagnosing BRC and initiating therapy as promptly as possible to avoid such outcomes in an otherwise possibly controllable disease.

Zusammenfassung

Ziel Das Ziel war es, die Prävalenz, morphologische und funktionelle Eigenschaften und die Entwicklung fortgeschrittener Fälle von Birdshot-Retinochoroiditis (BRC) zu bestimmen.

Methode Eine retrospektive Überprüfung aller BRC-Fälle eines Zentrums für Augenheilkunde, Lausanne, Schweiz, mit mindestens 3 Jahren Follow-up seit dem Beginn von Symptomen. Die folgenden Parameter wurden bewertet: demografische Eigenschaften, bestkorrigierte Sehschärfe, Fundusbilder, Fluorescein- und Indocyaningrün-Angiografie sowie Gesichtsfeldauswertung vom 1. Besuch bis zum letzten Follow-up.

Ergebnisse Von den 1920 Patienten mit Uveitis, die von 1995 bis 2018 beobachtet wurden, wurde bei 32 (1,67%) Patienten eine Birdshot-Diagnose gestellt; 20 dieser 32 Patienten hatten ausreichende Daten, um eingeschlossen werden zu können. Drei dieser 20 Patienten (15,0%) hatten schwere Formen der Krankheit. Zwei Patienten hatten eine unzureichende Behandlung erhalten und bei 1 Patienten schritt die Entzündung trotz des Versuchs der maximalen Therapie fort. Der mittlere Visus betrug bei Präsentation 0,62 (± 0,42) bei Patienten, die bereits in einem fortgeschrittenen Stadium der Krankheit waren, und 0,51 (± 0,37) bei der letzten Nachuntersuchung. Der durchschnittliche Gesichtsfeldmittelwertdefekt (VFMD) betrug bei Präsentation 19,65 (± 4,71 dB) und 21,40 (± 2,45 dB) bei der letzten Nachuntersuchung. Der Aderhautentzündungsscore, durch ICGA monitoriert, nahm von 10,67 (± 9,18) bei Präsentation auf 6,67 (± 5,61) ab bei der letzten Nachuntersuchung. Die FA zeigte eine leichte Netzhautvaskulitis, deren Score leicht abnahm unter Therapie: 6,50 (± 4,76) vs. 4,67 (± 3,20). Die mit EDI-OCT gemessenen Dicken der Aderhaut (EDI-OCT-CT) betrugen 246,36 (± 116,93 µm) bei Präsentation und 231,30 (± 120,70 µm) bei der letzten Nachuntersuchung.

Schlussfolgerungen In unserem Krankengut erlitten 15% der BRC-Fälle schwere, umfangreiche, irreversible, chorioretinale Schäden, offensichtlich aufgrund unzureichender Behandlung in 2 Fällen und schlechter Compliance in 1 Fall. Dies unterstreicht, wie wichtig es ist, bei  BRC eine schnelle Diagnose zu stellen und eine prompte Therapie zu starten, um eine solche schädliche Entwicklung zu vermeiden bei einer ansonsten möglicherweise kontrollierbaren Krankheit.

Key words

stromal choroiditis - birdshot - birdshot HLA-A29 retinochoroiditis - immunosuppressive therapy - severe disease

Schlüsselwörter

stromale Choroiditis - Birdshot-HLA-A29-Retinochoroiditis - immunosuppressive Therapie - schwerwiegende Erkrankung

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