The conundrum of juvenile spondyloarthritis classification: Many names for a single disease? Lesson learned from an instructive clinical case

Abstract

Pediatric rheumatic diseases are often characterized by an evolving phenotype, resulting in diagnostic dilemma for physicians involved in their management. Although several classification criteria are used in childhood to uniform patients’ diagnoses, several conditions share similar clinical features and therefore their classifications may overlap or be ambiguous. This is particularly paradigmatic for the classification of juvenile spondyloarthritis (JSpA), as the currently available criteria do not encompass their complexity. The differential diagnosis of sacroiliitis is often challenging for clinicians and requires considering several conditions, which include infective, neoplastic and rheumatic diseases. We report the case of a 13‐year‐old boy with an evolving clinical phenotype; its progression shows the wide differential diagnosis required in pediatric rheumatic diseases and emphasizes the issues of the actual classification system.

Comment on: The conundrum of juvenile spondyloarthritis classification: Many names for a single disease? Lesson learned from an instructive clinical case

“…Moreover, chronic recurrent multifocal osteomyelitis (CRMO) was suggested to be an unusual form of SpA by several authors and Vittecoq et al 9 showed that some of the patients with CRMO developed SpA. The insightful report by Maniscalco et al 1 also supports this perspective.…”

“…We read with great interest the paper titled “The conundrum of juvenile spondyloarthritis classification: Many names for a single disease? Lesson learned from an instructive clinical case” by Maniscalco et al 1 which emphasizes the complexity of the clinical phenotypes of juvenile spondyloarthropathies (JSpA). All pediatric rheumatologists face these evolving phenotypes of these groups of diseases in their daily practice.…”

A case of mistaken identity: an enthesitis-related arthritis case report

“…As mentioned previously, ErA is a special case in that it is also a jSpA. 5 Spondyloarthropathies (SpAs) are oligoarticualar, seronegative rheumatological disorders characterized by enthesitis, HLA-B27 positivity, and axial skeletal involvement. These conditions range from ErA, which is an undifferentiated disease seen exclusively in children, to differentiated adult disease such as ankylosing spondylitis.…”

“…The wide range of clinical manifestations of rheumatic diseases make the diagnosis a difficult one. 5 While sacroiliitis in addition to inflammatory bowel disease can lead to a diagnosis of ErA, a diagnosis of chronic nonbacterial osteomyelitis (CNO) syndrome can be reached with the addition of multiple foci of painful swollen bone. If psoriasis were also present in addition to the previous symptoms, conditions to be considered may include CNO syndrome, synovitis, acne, pustulosis, hyperostosis, osteomyelitis (SAPHO) syndrome, and undifferentiated JIA.…”

“…The overlap in diagnostic criteria among these conditions in addition to the complication added as the diseases progress illustrates the need for a revision in the classification system. 5,7 Martini et al proposed a new classification system to identify homogenous chronic disorders that fall under the current term JIA using clinical and routine laboratory measures.…”