Long-term response after 6-year treatment with anakinra and onset of focal bone erosion in neonatal-onset multisystem inflammatory disease (NOMID/CINCA).
The exact elucidation of skeletal and cartilagineous involvement in neonatal-onset multisystem inflammatory disease (NOMID) is still poorly known, and there are few data providing the long-term response to treatment with the available interleukin-1 inhibitors. We present here a 13-year-old boy with NOMID treated with anakinra and low-dose methylprednisolone since he was 7 years old for an overall period of 6 years. Every clinical manifestation was highly responsive to interleukin-1 blockade, with the exception of his bone abnormalities. At the comparison of radiography and magnetic resonance imaging of his knees made respectively at 7 and 13 years, we noticed a bone erosion on the posterior surface of the patella combined with the progression of distal femoral overgrowth and endosteal thinning of both meta-epiphyses. This report must encourage clinicians in a precocious institution of interleukin-1 antagonists to thwart the occurrence of irreversible bone changes.
Department of Pediatric Sciences, Università Cattolica Sacro Cuore, Largo A. Gemelli, 8, 00168 Rome, Italy. drigante@gmail.com
Donato
D
Rigante
Antonio
A
Leone
Raffaella
R
Marrocco
Maria Elena
ME
Laino
Achille
A
Stabile
D000293:Adolescent; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D001862:Bone Resorption / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000517:prevention & control; D002352:Carrier Proteins / Q000235:genetics; D056587:Cryopyrin-Associated Periodic Syndromes / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics; D004359:Drug Therapy, Combination; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D007375:Interleukin-1 / Q000037:antagonists & inhibitors; D007717:Knee / Q000000981:diagnostic imaging; D008279:Magnetic Resonance Imaging; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D020125:Mutation, Missense; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; D011859:Radiography; D016896:Treatment Outcome
D018501:Antirheumatic Agents; D002352:Carrier Proteins; D053590:Interleukin 1 Receptor Antagonist Protein; D007375:Interleukin-1; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; C453881:NLRP3 protein, human; D008775:Methylprednisolone
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