Infantile-onset multisystem inflammatory disease: radiologic findings.

Abstract

Infantile-onset multisystem inflammatory disease (IOMID) is a newly recognized disease that is similar to systemic-onset juvenile rheumatoid arthritis (JRA). The clinical symptoms of IOMID include rash, fever, lymphadenopathy, chronic meningitis, uveitis, and a distinct arthropathic condition. Skeletal involvement is surprisingly constant. The long bones become short, bowed, and widened. The metaphyses flare and simulate rickets. Grotesque epiphyses appear fragmented early in the course of the disease but are gradually incorporated into the widened shafts. We observed these and other radiologic findings in the four cases described in the present study. The findings are distinctive and allow for a specific diagnosis.

Tumor Necrosis Factor Receptor–Associated Periodic Syndrome

“…71,72 TRAPS lacks the distinctive deforming arthropathy with unique epiphyseal radiologic findings preferentially affecting large joints present in IOMID. 73 Eye involvement and neurological symptoms, including chronic meningitis, macrocephaly, and developmental delay, are common findings in IOMID and rare in TRAPS. [72][73] PFAPA is characterized by cervical adenitis, pharyngitis, aphthous stomatitis, features that rarely occur in TRAPS.…”

“…73 Eye involvement and neurological symptoms, including chronic meningitis, macrocephaly, and developmental delay, are common findings in IOMID and rare in TRAPS. [72][73] PFAPA is characterized by cervical adenitis, pharyngitis, aphthous stomatitis, features that rarely occur in TRAPS. Although both syndromes are characterized by periodic fever, attacks in PFAPA are shorter (4.7 days) than those in TRAPS (7-21 days).…”

Long-term response after 6-year treatment with anakinra and onset of focal bone erosion in neonatal-onset multisystem inflammatory disease (NOMID/CINCA)

“…Arthropathy of NOMID is a unique feature of this condition, which is believed to be the expression of abnormal endochondral bone growth, mainly aVecting large joints and long bones, beginning in the Wrst infancy and causing changes until skeletal maturity [7,8]. Children might present shortening of distal limbs and growth retardation, but it is still undetermined whether knee deformity is triggered by inXammation early in the skeletal development with IL-1 hypersecretion or by NLRP3 mutations causing abnormal chondrocyte apoptosis.…”

Chronic infantile neurological cutaneous articular syndrome: CD10 over-expression in neutrophils is a possible key to the pathogenesis of the disease

“…Most of the patients with CINCA previously published [2,5,6,8,9,10,13,19] presented with mental retardation. In all our cases psychomotor development has so far been normal.…”

“…Approximately 100 cases have been identified in the world [2,5,6,7,8,9,10,13,19]. The main clinical features consist of persistent urticaria-like skin manifestations, recurrent articular symptoms, fever, neurological manifestations, mental impairment, ocular inflammatory lesions, skeletal deformations and persistently abnormal laboratory findings (increased acute phase reactants, hypergammaglobulinaemia, and anaemia) [15].…”