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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
Rheumatology International
|May 9, 2009
Summary
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.
