Chronic recurrent multifocal osteomyelitis: what is it and how should it be treated?

Girschick, Hermann; Zimmer, C.; Klaus, Guenter; Darge, Kassa; Dick, Anke; Morbach, Henner

doi:10.1038/ncprheum0653

Cited by 136 publications

(121 citation statements)

References 18 publications

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“…Every child examined using MRI had confirmed bone lesions. CNO diagnosis can be almost definite if bone lesions on MRI are accompanied by typical skin lesions (9). In such cases, biopsy appears to be unnecessary (27).…”

Section: Discussionmentioning

confidence: 99%

“…Patients are usually in good general condition. CNO can appear as a mono-/oligo-focal disease, as well as in chronic recurrent polyfocal stages with a risk of late effects, such as vertebral fractures and severe hyperostotic bone lesions (9). CNO primarily affects the metaphyses of long bones, although lesions can occur in any part of the skeleton (10).…”

Section: Introductionmentioning

confidence: 99%

“…CNO primarily affects the metaphyses of long bones, although lesions can occur in any part of the skeleton (10). Other organs, including the skin, eyes, gastrointestinal tract, and lungs, can also be affected by inflammation (9,11,12). Skin inflammation manifests as palmoplantar pustulosis, acne, psoriasis, and rarely as pyoderma gangrenosum (1,2).…”

Section: Introductionmentioning

confidence: 99%

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Characteristics and outcomes of chronic non-bacterial osteitis in children

Wintrich¹,

Horneff²

2015

Eur J Rheumatol

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Objective: To determine patient characteristics, clinical presentation, pattern of involvement, treatment, and outcome of patients with chronic non-bacterial osteitis (CNO).Material and Methods: Consecutive cases of CNO were analyzed at a single center for pediatrics and adolescent medicine from 2006 to 2013 in terms of patient characteristics, clinical presentation, pattern of involvement, treatment, and outcome. Results:We identified 32 children aged 1.5-15 years who were diagnosed with CNO between 2006 and 2013. A maximum of 12 bones per patient were affected in a total of 114 documented locations. The pelvis and clavicle (affecting 34% of patients each) were the most frequently affected bones. The foot skeleton was the most commonly affected region in 60% of patients. Skin manifestations were found in 7 (21%) patient. Increased inflammatory signs at presentation were detected in 18 patients. Pathological findings were found in all 30 children examined using magnetic resonance imaging (MRI), in 10 of 11 children examined using radiography, and in 8 of 10 patients examined using skeletal scintigraphy. Bone biopsy was performed in 9 patients. For initial treatment, non-steroidal anti-inflammatory drugs (NSAIDs) or coxibs were used in 28 (87.5%) patients. Remission or satisfactory follow-up was achieved in all patients.Conclusion: Today, CNO is increasingly diagnosed using MRI and rarely through histological examinations. Therapeutic strategies include NSAIDs, which are often highly effective. All patients in the present study showed good clinical outcomes. Keywords: Chronic recurrent multifocal osteomyelitis, bone diseases, chronic disease IntroductionChronic non-bacterial osteitis (CNO) is a benign noninfectious autoinflammatory disease of the bone tissue with an incomplete etiology (1, 2). The incidence of CNO is 0.4/100.000 children (3). It was first reported by Giedion et al. (4) in 1972. There are several synonyms for CNO, such as chronic recurrent multifocal osteomyelitis (CRMO); non-bacterial osteitis; or synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome, all describing the same disease entity (5, 6). CNO generally occurs in children and adolescents, with a peak age of onset of 7-12 years (7). Localized bone pain is the leading symptom, often with local swelling and warmth (8). Patients are usually in good general condition. CNO can appear as a mono-/oligo-focal disease, as well as in chronic recurrent polyfocal stages with a risk of late effects, such as vertebral fractures and severe hyperostotic bone lesions (9). CNO primarily affects the metaphyses of long bones, although lesions can occur in any part of the skeleton (10). Other organs, including the skin, eyes, gastrointestinal tract, and lungs, can also be affected by inflammation (9,11,12). Skin inflammation manifests as palmoplantar pustulosis, acne, psoriasis, and rarely as pyoderma gangrenosum (1, 2). The most important differential diagnosis is bacterial osteomyelitis, although it is not usually multifocal (13). Even...

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Characteristics and outcomes of chronic non-bacterial osteitis in children

Wintrich¹,

Horneff²

2015

Eur J Rheumatol

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“…Chronic bacterial osteomyelitis should be differentiated from chronic recurrent multifocal osteomyelitis (CRMO), a nonbacterial inflammatory disease involving multiple osseous structures, typically the metaphysis of long bones and the clavicle [39]. Whole-body MRI is a useful modality for the evaluation of this disease; symmetric involvement is highly suggestive of this condition [40], which responds to antiinflammatory agents rather than antibiotics.…”

Section: Imaging Of Complicationsmentioning

confidence: 99%

Infection: musculoskeletal

Jaramillo

2011

Pediatr Radiol

102

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The imaging approach to osteomyelitis has evolved in the past two decades. Advances in MRI allow for whole body imaging, decreasing the need for scintigraphy when symptoms are not localized or the disease may be multifocal. There is an increasing clinical need for depiction of abscesses in the soft tissues and subperiosteal space, particularly because methicillin-resistant Staphylococcus aureus infections constitute more than one-third of all the infections. The increasing emphasis on radiation dose reduction has also led away from scintigraphy and computed tomography. MR imaging has become the advanced imaging modality of choice in osteomyelitis. There is an increasing understanding of the appropriate role for gadolinium enhancement, which is not indicated when the pre-gadolinium images are normal. Other related infections, including pyomyositis, are best imaged with MRI.Keywords Infection . Musculoskeletal . Children . MRI EpidemiologyAcute hematogenous osteomyelitis is the most common form of musculoskeletal infection [1]. Its incidence is difficult to determine, but it has been reported to be 1 per 5,000 [2]. There is conflicting data regarding the change of the disease over time. A study in 1998 found that the incidence in Norway ranged between 70 and 160 per million and that the incidence had remained stable between 1965 and 1994 [3]. An American study, however, found that in the last 20 years the incidence of osteomyelitis had increased 2.8-fold, whereas that of septic arthritis was stable. Methicillin-resistant Staphylococcus aureus (MRSA) was the causative organism in 30% of the children [4]. More than half of the cases occur before 5 years of age, and it appears to be more frequent in boys. Approximately 70% of infections involve the lower extremities and onethird affect the knee [2]. PathophysiologyHematogenous osteomyelitis results from the seeding of bacteria, usually Staphylococcus aureus (S. aureus) in the metaphysis of a long bone (Fig. 1) or in the metaphyseal equivalents of the axial skeleton (Fig. 2). S. aureus bacteria, which are present in the skin or mucosal membranes, enter the bloodstream and lodge in the bone, usually at sites where blood flow is abundant and slow. The metaphyses are richly vascularized and the valveless sinusoidal loops of the metaphyseal venules have sluggish flow and discontinuous endothelium which facilitates bacterial invasion. During childhood, the metaphyses that grow the fastest, such as those around the knee, are the most richly vascularized in order to produce new bone at a rapid rate. These rapidly growing metaphyses also tend to be affected more frequently by infection. S. aureus has the capacity to attach to the bony matrix and produce proteolytic enzymes that destroy the surrounding tissues, allowing the spread of the infection. From the bone, the infection extends through the porous metaphyseal cortex into the subperiosteal space. The periosteum in children is loosely attached to the bone and separates easily from it, creating a space that allows ...

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“…Currently, corticosteroids are recommended as "bridging" therapy only when NSAIDs have failed, and use of disease modifying drugs is initiated. One recommended schedule consists of oral glucocorticoids for 1 week at 2 mg prednisone/kg/day, followed by discontinuation stepwise by 25% every 5 days (Girschick, Zimmer et al 2007). Intravenous methylprednisolone pulses have been reported to be effective in selected refractory cases but no uniform treatment protocol exists (Holden and David 2005).…”

Section: Corticosteroidsmentioning

confidence: 99%

Chronic Non-Bacterial Osteitis/Chronic Recurrent Multifocal Osteomyelitis

Miettunen¹

2012

Osteomyelitis

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Chronic recurrent multifocal osteomyelitis: what is it and how should it be treated?

Cited by 136 publications

References 18 publications

Characteristics and outcomes of chronic non-bacterial osteitis in children

Characteristics and outcomes of chronic non-bacterial osteitis in children

Infection: musculoskeletal

Chronic Non-Bacterial Osteitis/Chronic Recurrent Multifocal Osteomyelitis

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