Chronic nonbacterial osteomyelitis in children: a retrospective multicenter study

Abstract

BackgroundTo determine the clinical presentation, current treatment and outcome of children with nonbacterial inflammatory bone disease.MethodsRetrospective multicenter study of patients entered into the Swiss Pediatric Rheumatology Working Group registry with a diagnosis of chronic nonbacterial osteomyelitis (CNO) and synovitis acne pustulosis hyperostosis osteitis (SAPHO) syndrome. The charts were reviewed for informations about disease presentation, treatment, course and outcome.ResultsForty-one children (31 girls and 10 boys) from 6 pediatric hospitals in Switzerland diagnosed between 1995 and 2010 were included in the study. The diagnosis was multifocal CNO (n = 33), unifocal CNO (n = 4) and SAPHO syndrome (n = 4). Mean age at onset of CNO was 9.5 years (range 1.4–15.6) and mean follow-up time was 52 months (range 6–156 months). Most patients (n = 27) had a chronic persistent disease course (>6 months), 8 patients had a course with one or more relapses and 6 patients had only one episode of CNO. Forty nine percent had received at least one course of antibiotics. In 57 % treatment with nonsteroidal anti-inflammatory drugs (NSAID) was sufficient to control the disease. Twelve out of 16 children with NSAID failure subsequently received corticosteroids, methotrexate, TNF α inhibitors, bisphosphonates or a combination of these drugs.ConclusionsIn a multicenter cohort of 41 children 22 % started with unifocal lesion with a significant diagnostic delay. A higher proportion presented with chronic persistent disease than with a recurrent form. An osteomyelitis in the pelvic region is significantly associated with other features of juvenile spondylarthritis.

SAPHO and CRMO: The Value of Imaging

“…The clinical course in both adults and children/adolescents is usually characterized by repeated episodes of active inflammation and remission but may present as single flares or have a chronic inflammatory pattern. 4,10,12,[26][27][28] Involvement of additional sites during the disease course is frequent, 7,10,11,16 but the disease progression, at least in adults, is usually minimal and slow, and SAPHO is not a particularly debilitating disorder. 9,11,12,29 Low-grade fever may be present during periods with disease activity, especially in children/adolescents, but other systemic manifestations are rare.…”

“…15,21,[33][34][35][36] WBMRI may visualize most of the osteoarticular changes of SAPHO/CRMO in one examination, especially when using coronal T1-weighted and STIR sequences in addition to axial STIR images of the whole body supplemented by sagittal sequences of the spine and/or dedicated MRI of inadequately visualized peripheral bone involvement. 28,[35][36][37] WBMRI is valuable for assessing total disease activity as well as for monitoring therapeutic response or the spontaneous course of the disease. 36 This radiation-free assessment of the entire body by WBMRI is particularly important in children/adolescents where repeated follow-up is often necessary because clinical remission does not necessarily mean radiologic remission.…”

Physicians’ Perspectives on the Diagnosis and Treatment of Chronic Nonbacterial Osteomyelitis

“…A prospective study of thirty-seven children showed good response to NSAIDs during the first 3–6 months of illness [9]. Other studies of CNO cohorts have documented the effectiveness of biologic and nonbiologic disease modifying antirheumatic drugs and bisphosphonates in treating CNO not controlled by NSAIDs [7, 8]. Understanding pediatric rheumatologists' practices in diagnosing and treating CNO would inform future refined diagnostic criteria and disease activity monitoring and enable development of standardized treatment regimens (consensus treatment plans) to allow for comparative effectiveness studies.…”

Clinical Images: Chronic Nonbacterial Osteomyelitis

“…Because the clinical presentation may mimic infectious osteomyelitis or bone malignancy, it is important to perform a bone biopsy. Chronic nonbacterial osteomyelitis may also present with fever and arthritis (seen in this patient), skin lesions (e.g., acne, palmoplantar pustulosis, and psoriasis), and/or autoimmune disease such as Crohn's disease and ulcerative colitis . Our patient was started on daily loxoprofen, the mainstay treatment, which partially relieved her pain and improved laboratory indicators of inflammation.…”