Chronic Nonbacterial Osteomyelitis of the Sternocostoclavicular Region in Adults: A Single-Center Dutch Cohort Study.

Autor: Ramautar AI; Centre for Bone Quality, Department of Medicine, Division of Endocrinology Leiden University Medical Center Leiden The Netherlands., Appelman-Dijkstra NM; Centre for Bone Quality, Department of Medicine, Division of Endocrinology Leiden University Medical Center Leiden The Netherlands., Lakerveld S; Centre for Bone Quality, Department of Medicine, Division of Endocrinology Leiden University Medical Center Leiden The Netherlands., Schroijen MA; Centre for Bone Quality, Department of Medicine, Division of Endocrinology Leiden University Medical Center Leiden The Netherlands., Snel M; Centre for Bone Quality, Department of Medicine, Division of Endocrinology Leiden University Medical Center Leiden The Netherlands., Winter EM; Centre for Bone Quality, Department of Medicine, Division of Endocrinology Leiden University Medical Center Leiden The Netherlands., Hamdy NA; Centre for Bone Quality, Department of Medicine, Division of Endocrinology Leiden University Medical Center Leiden The Netherlands.
Jazyk: angličtina
Zdroj: JBMR plus [JBMR Plus] 2021 Apr 10; Vol. 5 (5), pp. e10490. Date of Electronic Publication: 2021 Apr 10 (Print Publication: 2021).
DOI: 10.1002/jbm4.10490
Abstrakt: Sternocostoclavicular hyperostosis (SCCH) is a rare autoinflammatory bone disorder caused by chronic nonbacterial osteomyelitis (CNO), which is associated with sclerosis and hyperostosis primarily affecting the sternum, the medial end of the clavicles, and the first ribs. Other areas of the axial skeleton may also be affected. The more severe synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome is additionally associated with dermatoses and joint manifestations. This Dutch retrospective cross-sectional single-center cohort study characterizes the spectrum of clinical features in adult CNO/SCCH patients at the time of diagnosis. The only inclusion criteria was the availability of complete sets of clinical and imaging data systematically collected over three decades using in-house protocols. Data from 213 predominantly female patients (88%) with a median age of 36 years at presentation were studied. The mean diagnostic delay was 5 ± 5 years. The main symptoms were chronic pain (92%), bony swelling (61%), and restricted shoulder girdle function (46%); 32% had palmoplantar pustulosis and 22% had autoimmune disease. The majority (73%) had isolated SCCH; 59 (27%) had additional localizations in vertebrae (19%), the mandible (9%), or both (2%); 4 had SAPHO. The prevalence of current or past smoking was high (58%), particularly for patients with palmoplantar pustulosis (76%). There was a significant relationship between delay in diagnosis and both the extent of affected skeletal sites ( p = 0.036) and erythrocyte sedimentation rate levels ( p = 0.023). Adult-onset CNO is characterized by distinctive clinical and radiological features, but diverse aspects of its spectrum are currently not fully captured by a comprehensive classification. Delayed diagnosis is still common and potentially associated with irreversible structural changes and debilitating chronic symptoms, increasing the burden of illness and negatively impacting on quality of life. It is hoped that findings from this study will dispel confusion about nomenclature and classification of adult-onset CNO and increase awareness of its distinctive clinical and radiological features, and thus facilitate early diagnosis and referral for treatment, which should positively impact prognosis by preventing disease progression, although this remains to be established. © 2021 The Authors. JBMR Plus published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research.
Competing Interests: All authors state that they have no conflict of interest. Author Contributions Study design: AIER, NMA‐D, and NATH. Study conduct: AIER, NMA‐D, SL, MAS, MS, EMW, and NATH. Data interpretation: AIER, NMA‐D, EMW, and NATH. Drafting manuscript: AIER, NMA‐D, and NATH. Revising manuscript content: AIER, NMA‐D, EMW, and NATH. Approving final version of the manuscript: All authors. AIER and NATH take responsibility for the integrity of the data analysis.
(© 2021 The Authors. JBMR Plus published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research.)
Databáze: MEDLINE
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