Wide Range of Clinical Manifestations Identified in Chronic Recurrent Multifocal Osteomyelitis

MRI of a knee joint showing the destruction of bone due to advanced osteomyelitis
MRI of a knee joint showing the destruction of bone due to advanced osteomyelitis
A series of reported cases shows that the range of associated clinical manifestations is heterogeneous in chronic nonbacterial osteomyelitis or chronic recurrent multifocal osteomyelitis.

Chronic nonbacterial osteomyelitis (CNO) or chronic recurrent multifocal osteomyelitis (CRMO) can present with a range of associated clinical manifestations. Onset of the conditions is usually during the early teenage years, but CNO or CRMO may also occur in adults, even in the absence of mucocutaneous manifestations, according to a retrospective study published in Rheumatology.

Investigators used a web-based registry, Eurofever, to collect retrospective data on patients affected by CRMO/CNO. The study included both adult and pediatric patients, and the final database extraction was performed in September 2016.

Complete baseline information was available for 486 patients, including 176 men and 310 women. The mean age of disease onset was 9.9 years; adult onset occurred in 6.3% of patients. The mean time from disease onset to final diagnosis was 1 year, with a range of 0 to 15 years. MRI was performed at baseline in 88% of patients, revealing a mean number of 4.1 lesions.

The most frequent disease manifestations not directly related to bone involvement were myalgia (12%), mucocutaneous manifestations (5% acne, 5% palmoplantar pustulosis, 4% psoriasis, 3% papulopustular lesions, and 2% urticarial rash), and gastrointestinal symptoms (8%). A total of 361 patients were treated with nonsteroidal anti-inflammatory drugs (NSAIDs), 112 with glucocorticoids, 61 with bisphosphonates, 58 with methotrexate, 47 with sulfasalazine, 26 with anti-tumor necrosis factor therapy, and 4 with anakinra. Response to these treatments was variable.

The authors note that there has been a long-standing debate whether arthritis is a feature of this disease. In this study, 26.6% of patients were affected by arthritis. Among the treatments used, methotrexate and etanercept were less effective, particularly compared with glucocorticoids and bisphosphonates. In the early phases of the disease, NSAIDs were the most commonly used drugs, leading to complete response in nearly 40% of patients.

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Girschick H, Finetti M, Orlando F, et al. The multifaceted presentation of chronic recurrent multifocal osteomyelitis: a series of 486 cases from the Eurofever international registry [published online March 27, 2018].  Rheumatology (Oxford). doi:10.1093/rheumatology/key058