The Challenge of Treating the Severest Forms of Chronic Noninfectious Posterior and Panuveitis

Abstract

Background Noninfectious posterior and panuveitis may exhibit a chronic relapsing clinical course and are challenging to treat. Most affected patients are continuously treated with systemic immunosuppressive therapy, which is potentially associated with significant adverse side effects.

Methods A cohort of 18 patients presenting with severe noninfectious posterior or panuveitis were evaluated with respect to the clinical course of the disease, with particular focus on best-corrected visual acuity (BCVA), treatment duration, remission rates, reported negative side effects, and the necessity for switching medication.

Results The mean follow-up was 27.8 months. Although BCVA improved significantly, complete or partial remission was observed in only 66.7% of patients. Of the patients, 72.2% underwent a change in medical treatment due to either adverse events or inefficacy of medication.

Conclusion Despite new immunosuppressive therapies, effective treatment of severe noninfectious posterior and panuveitis remains a major challenge. We discuss the urgent need for novel treatment strategies in order to prevent systemic adverse effects, and to improve visual outcome and quality of life.

Zusammenfassung

Hintergrund Nicht infektiöse posteriore Uveitis und Panuveitis verursachen häufig chronisch rezidivierende Verläufe bis hin zu irreversibler Erblindung. Eine effektive Behandlung ist nach wie vor schwierig. Die meisten Patienten erhalten eine systemische immunsuppressive Medikation, die häufig mit schwerwiegenden Nebenwirkungen einhergeht.

Methodik Eine Kohorte mit 18 Patienten mit gesicherter Diagnose einer nicht infektiösen posterioren oder Panuveitis wurde bez. des Krankheitsverlaufs ausgewertet. Unser Fokus lag dabei vor allem auf der Entwicklung der visuellen Funktion, der Krankheitsdauer, Remissionsraten, Nebenwirkungen der Therapie und erforderlichen Umstellungen der Medikation.

Ergebnisse Die mittlere Beobachtungszeit lag bei 27,8 Monaten. Obwohl der Visus sich insgesamt signifikant verbesserte, kam es nur in 66,7% der Fälle zu einer kompletten oder teilweisen Remission. Bei 72,2% der Patienten musste die Therapie aufgrund von Nebenwirkungen oder mangelnder Effektivität umgestellt werden.

Schlussfolgerung Trotz neuer immunsuppressiver oder -modulatorischer Therapieoptionen und gutem Zugang zu diesen ist eine effektive Behandlung schwerer nicht infektiöser posteriorer oder Panuveitis eingeschränkt. Wir diskutieren den dringenden Bedarf an neuen Therapieoptionen für diese Erkrankung, um systemische Nebenwirkungen zu vermeiden und die Visusfunktion und Lebensqualität zu verbessern.

Conclusion

• Noninfectious posterior and panuveitis may cause a chronic relapsing clinical course resulting in blindness and are challenging to treat. Most affected patients are continuously treated with systemic immunosuppressive therapy potentially associated with significant adverse side effects.

• Due to rare occurrence, heterogeneous disease courses, and individual treatment, current data regarding treatment success in noninfectious posterior and panuveitis are poor. We therefore evaluated an available patient cohort in a longitudinal manner.

• Rates of remission are unsatisfactory despite novel treatment strategies and excellent access to treatment in our cohort.

• Changes in medication due to inefficacy or severe adverse effects are frequent.

• We see an urgent need for novel treatment strategies to prevent systemic adverse effects and improve visual outcome in noninfectious posterior and panuveitis.

Key words

noninfectious uveitis - birdshot chorioretinopathy - serpiginous choroiditis - Behçets syndrome - Vogt-Koyanagi-Harada syndrome

Schlüsselwörter

nicht infektiöse Uveitis - Birdshot-Chorioretinopathie - serpiginöse Choroiditis - Behçet-Syndrom - Vogt-Koyanagi-Harada-Syndrom

Publication History

Received: 26 January 2023

Accepted: 06 April 2023

Article published online:
12 July 2023

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