If Your Newborn Has CINCA, Then You Need to Read This

According to a study published in Pediatric Rheumatology, treatment with anakinra early in the neonatal period (birth-28 days) was shown to be effective in treating CINCA (Chronic Infantile Neurological Cutaneous Articular Syndrome).

This chronic disease, also known as NOMID (Neonatal Onset Multisystem Inflammatory Disease), has early onset in the neonatal period that affects the central nervous system, bones, and joints. It is characterized by three key symptoms:

  • Cutaneous rash
  • Arthritis
  • Meningitis-like symptoms, involving severe inflammation without infections

CINCA is a genetic mutation that causes increased inflammasome activity that results in increased release of IL-1β (Interleukin 1 beta). Interleukin 1 beta is a group of 11 cytokines, which plays a central role in the regulation of immune and inflammatory responses to infections. This increase in IL-1β leads to symptoms of inflammation, such as fever and joint pain.

A study was done where two preterm infants with severe CINCA were treated with anakinra early in the neonatal period. Anakinra targets interleukin 1 beta activity. A persistent decline of systemic inflammation was observed in both cases suggesting that treatment can be started safely in neonates in order to prevent irreversible organ damage and disability.

Anakinra has a short half-life (only a few hours!), so it is the treatment of choice in patients  with CINCA.

That’s especially true if there’s some question of how they’ll tolerate the drug, such as neonatal and pediatric patients.

In the case study report 1, a baby boy born at 33 weeks was diagnosed with CINCA syndrome based on his symptoms and the absence of infection. After discussion with his parents and their consent, anakinra treatment was initiated at day 17. Already after three subcutaneous injections, the rash disappeared, C reactive protein reached normal values, and the arthritis improved.

In the case study report 2, a baby girl born at 34 weeks was diagnosed with CINCA syndrome based on her symptoms of arthritis, rash, and persistently elevated inflammation markers. Her parents accepted treatment with anakinra at 32 days old.

The treatment suppressed the inflammatory reaction. Normal C reactive protein levels were reached and the patient was free of symptoms!

Of course, for patients with CINA, long term outcomes are dependent on how severe and how long the inflammatory process lasts. However, symptoms, like hearing loss, may be reversed if the treatment is started early.

In fact, as long as the treatment is started before any irreversible lesions appear, other complications may actually be prevented!

If those aren’t enough reasons to treat CINCA, and treat early, then we don’t know what is!

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