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Post-inflammatory retinal dystrophy in CINCA syndrome
Donato Rigante1, Achille Stabile, Angelo Minnella
1Department of Pediatric Sciences, Center of Periodic Fevers, Università Cattolica Sacro Cuore, Largo A. Gemelli no. 8, 00168 Rome, Italy. drigante@gmail.com
Insights
Anakinra effectively treated a child with CINCA syndrome, resolving meningitis and improving vision despite developing retinal dystrophy. Long-term treatment stabilized vision, highlighting anakinra
Area of Science:
- Pediatric Rheumatology
- Ophthalmology
- Neurology
Background:
- Neonatal aseptic chronic meningitis can be associated with CINCA syndrome.
- Early diagnosis and treatment are crucial for managing autoinflammatory conditions.
Observation:
- A 9-month-old presented with meningitis and later diagnosed with CINCA syndrome.
- Ocular findings included chorioretinitis and pseudopapilledema.
- Initial treatments showed inconsistent responses.
Findings:
- Anakinra initiated at age 7 led to immediate clinical and laboratory improvement.
- Severe post-inflammatory retinal dystrophy was diagnosed at age 10.
- Vision remained stable after one year of anakinra treatment.
Implications:
- Anakinra is a promising therapeutic option for CINCA syndrome.
- Early intervention may mitigate severe ocular complications.
- Long-term monitoring is essential for patients with CINCA syndrome and ocular involvement.
Abstract:
Right chorioretinitis and bilateral pseudopapilledema were firstly appreciated in a 9-month-old child with neonatal findings of aseptic chronic meningitis, framed in the context of CINCA syndrome at 1 year. Therapeutical response to various combinations of drugs was inconsistent until 7 years, when anakinra was started with immediate clinical and laboratory improvement. A state of severe retinal dystrophy of post-inflammatory origin became evident on funduscopy, optical coherence tomography and visual electrophysiology tests at the age of 10 years, which remained stationary after 1 year of anakinra treatment.
