ABSTRACT
Objective:To summarize the clinical features of chronic non-bacterial osteomyelitis (CNO) in children.Methods:Clinical data of 8 CNO patients admitted to the Department of Rheumatology and Immunology, Children′s Hospital Affiliated to Capital Institute of Pediatrics from March 2014 to December 2020 were retrospectively analyzed.The clinical characteristics of 8 children with CNO were summarized and compared with those reported abroad.Results:A total of 8 CNO patients were recruited, involving 3 males and 5 females with the mean age of onset (7.2±3.2)years, and the average diagnosis time 25.9 months, respectively.The common clinical symptoms included bone pain (7 cases, 87.5%), arthritis (4 cases, 50.0%), and fever (3 cases, 37.5%). The main manifestations on X-ray and CT scans were bone destruction and progressive osteosclerosis.Magnetic resonance imaging (MRI) showed bone marrow edema, periostitis, soft tissue swelling, and enhancement.All of them had more than one site of bone involvement.Seven patients(87.5%) had bilateral bone involvement, with the most common site of tibia (22.0%), followed by femur (17.1%) and mandible (9.8%). Bone biopsy was performed in 8 patients, and 4 cases showed osteonecrosis, 4 cases showed bone fibrosis and 2 cases showed osteomyelitis.The etiological examination of the bone was negative.Eight children received non-steroid anti-inflammatory drugs alone or in combination with glucocorticoids, disease-modifying antirheumatic drugs (DMARDs), bisphosphonates or tumor necrosis factor-α(TNF-α) antagonists.After treatment, the patients were followed up for 3 months to 2 years.Eight children improved.Their inflammatory indexes were normal, and had no disability, teratology or multiple organ damage.Conclusions:Pediatric CNO is more common in children of school age, with a long course of disease.The main manifestations are multi-site bone pain and arthritis.Imaging studies indicate multiple bone involvement, which is more common at lower extremities.Non-steroids anti-inflammatory drugs, glucocorticoids, DMARDs, bisphosphonates and TNF-α antagonists are effective to CNO.
ABSTRACT
Chronic recurrent multifocal osteomyelitis (CRMO) is one of the most severe form of chronic non-bacterial osteomyelitis (CNO), which could result in bone and related tissue damage. This autoinflammatory bone disorder (ABD) is very difficult for its clinical diagnosis because of no diagnostic criteria or biomarkers. CRMO in the jaw must be suspected in the differential diagnosis of chronic and recurrent bone pain in the jaw, and a bone biopsy should be considered in chronic and relapsing bone pain with swelling that is unresponsive to treatment. The early diagnosis of CRMO in the jaw will prevent unnecessary and prolonged antibiotic usage or unnecessary surgical intervention. The updated researches for the identification of genetic and molecular alterations in CNO/CRMO should be studied more for its correct pathophysiological causes and proper treatment guidelines. Although our trial consisted of reporting items from Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA), there are very few articles of randomized controlled trials. This article was summarized based on the author's diverse clinical experiences. This paper reviews the clinical presentation of CNO/CRMO with its own pathogenesis, epidemiology, recent research studies, and general medications. Treatment and monitoring of the jaw are essential for the clear diagnosis and management of CNO/CRMO patients in the field of dentistry and maxillofacial surgery.
Subject(s)
Humans , Biomarkers , Biopsy , Dentistry , Diagnosis , Diagnosis, Differential , Early Diagnosis , Epidemiology , Jaw , Osteomyelitis , Surgery, OralABSTRACT
La osteomielitis crónica multifocal recurrente fue recientemente clasificada dentro de las enfermedades autoinflamatorias, caracterizadas por episodios de inflamación sistèmica, que incluyen indicadores serológicos de inflamación, en ausencia de autoanticuerpos o agentes patógenos. La característica clínica es la aparición insidiosa de dolor, tumefacción y sensibilidad localizada sobre el hueso afectado, principalmente, en la metáfisis y epífisis de los huesos largos, clavícula y también vértebras. Son episodios autolimitados y recurrentes. Se presenta a un paciente de 2 años y 2 meses con afectación ósea tipo osteolítica en dos focos aislados con un año de diferencia entre ambos episodios. La biopsia ósea fue compatible con osteomielitis crónica y se descartó patología de origen infeccioso, neoplásico y enfermedad proliferativa. Presentó buena respuesta al tratamiento con antiinflamatorios. El conocimiento de esta entidad como diagnóstico diferencial evita el tratamiento antibiótico prolongado, estudios complementarios y biopsias óseas, considerando los criterios diagnósticos y recurrencia de los episodios.
Chronic recurrent multifocal osteomyelitis has recently been classified as an autoinflammatory disorder characterized by episodes of systemic inflammation including serological signs of inflammation occurring in the absence of autoantibodies or pathogen agents. The insidious onset of pain with swelling and tenderness localized over the affected bones are the main manifestations. The metaphysis and epiphyses of the long bones, clavicle and vertebrae are affected. We report a male patient aged 2 years and 2 months with osteomyelitis with lytic destruction in two different single sites with a year difference between the episodes. Histological examination of the bone showed inflammation and chronic osteomyelitis, excluding the existence of infectious osteomyelitis, neoplasm and myeloproliferative disease. Clinical symptoms improved under treatment with nonsteroidal anti-inflammatory drugs. Considering chronic recurrent multifocal osteomyelitis may shorten time to diagnosis in order to avoid potentially unnecessary prolonged courses of intravenous antibiotics, complementary studies and multiple biopsies.
Subject(s)
Humans , Male , Child, Preschool , Osteomyelitis/diagnosis , Anti-Inflammatory Agents, Non-Steroidal/therapeutic use , Inflammation/diagnosis , Osteomyelitis/pathology , Osteomyelitis/drug therapy , Time Factors , Treatment Outcome , Diagnosis, Differential , Inflammation/pathology , Inflammation/drug therapyABSTRACT
Los síndromes autoinflamatorios son un grupo de enfermedades caracterizadas por episodios espontáneos, recurrentes o persistentes de inflamación multisistémica. En ellos no se evidencia una etiología infecciosa, neoplásica o autoinmune. Están causadas por alteraciones de la inmunidad innata, lo que ocasiona una desregulación del sistema inflamatorio a nivel del inflamosoma. Estos síndromes se subdividen en dos grandes grupos los síndromes hereditarios de fiebre periódica y las enfermedades autoinflamatorias persistentes, dentro de este último se ubican las enfermedades inflamatorias óseas donde se incluye la osteomielitis multifocal crónica recurrente. Presentamos un caso de una niña de 9 años que ingresa en nuestro hospital por un síndrome febril prolongado y después de múltiples investigaciones se concluye como una osteomielitis multifocal crónica recurrente.
Autoinflammatory syndromes are a group of diseases characterized by spontaneous, recurrent or persistent episodes of multisystem inflammation. They do not show an infectious, neoplastic or autoimmune etiology. They are caused by alterations of the innate immunity, which causes a dysregulation of the inflammatory system at the level of the inflammasome. These syndromes are subdivided into two major groups, the hereditary syndromes of periodic fever and the persistent autoinflammatory diseases; within the latter are the inflammatory bone diseases which chronic recurrent multifocal osteomyelitis. We present a case of a 9-year-old girl who enters our hospital due to a prolonged febrile syndrome and after multiple investigations, it concludes as a Chronic Recurrent Multifocal Osteomyelitis.
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Introducción: La osteomielitis crónica multifocal recurrente (OCMR) es una enfermedad rara de causa desconocida, que afecta principalmente la metáfisis de los huesos largos; se caracteriza por un cuadro clínico insidioso de exacerbaciones y remisiones; la cronicidad de los síntomas, los hallazgos imagenológicos y la falta de respuesta al manejo con antibióticos, orientan al diagnóstico. Objetivo: Presentar los datos clínicos, resultados de laboratorio, imagenológicos y estudios histo- patológicos que permiten realizar el diagnóstico de OCMR. Caso clínico: Paciente de sexo femenino de 9 años, con artralgias en ambas rodillas y tobillo izquierdo de 1 mes de duración. Los estudios de gammagrafía ósea y resonancia corporal total evidenciaron compromiso óseo multifocal; con estos hallazgos, se realizó biopsia que fue negativa para infección y malignidad. Por la evolución, estudios de laboratorio, radiológicos e histo-patológicos se diagnosticó OCMR. Se inició tratamiento con anti-inflamatorios no esteroides con adecuada respuesta. Conclusiones: La OCMR es una patología poco frecuente de difícil diagnóstico, por lo que se requiere de una alta sospecha clínica para realizar un adecuado enfoque y tratamiento oportuno.
Introduction: Chronic recurrent multifocal osteomyelitis (CRMO) is a very rare disease, of unknown origin that affects primarily the metaphysis of long bones. It is characterized by an insidious onset of symptoms and multiple remissions. The chronicity of symptoms, the diagnostic imaging and the lack of response to first line antibiotic treatment, should be helpful for diagnostic. Objective: Present a clinical case, based on clinical, laboratory, radiologic imaging and histopathological results, that ultimately led to the diagnostic of CRMO. Case report: 9 year old, female patient, with one month of bilateral knee and left ankle arthralgia. Bone Gammagraphy and full body MRI, showed multifocal bone inflammation. These findings led to a biopsy, that turned negative for malignancy and infection. Given all the information available from the laboratory test results, radiologic imaging and histopathological findings, CRMO diagnosis was made. NSAID treatment was order, with good results. Conclusions: CRMO is a rare disease that even to date and with cutting edge technology, still represents a diagnostic challenge that primarily relies on a high level of suspicion, for a timely and correct treatment.
Subject(s)
Humans , Female , Child , Osteomyelitis/diagnosisABSTRACT
Chronic Recurrent Multifocal Osteomyelitis (CRMO) is a non-bacterial inflammatory disorder of unknown cause occurring in children and adolescents. It is characterized by the insidious onset of pain and swelling to multifocal involved bones, recurring over months to years. Non-steroid anti-inflammatory drugs (NSAIDs) and steroids are the first choice for the initial and relapse treatment. However, multifocal and frequent relapses might require more intensive anti-inflammatory treatment. Here, we report that an adolescent with CRMO refractory to antibiotics, NSAIDs and steroids over a two-year responded well to bisphosphonate. To our knowledge, this is the first case using bisphosphonate in adolescent refractory CRMO in Korea.
Subject(s)
Adolescent , Child , Humans , Anti-Bacterial Agents , Anti-Inflammatory Agents, Non-Steroidal , Diphosphonates , Korea , Osteomyelitis , Recurrence , SteroidsABSTRACT
Chronic recurrent multifocal osteomyelitis (CRMO) is a non-infectious inflammatory bone disease that occurs mainly in children and adolescents. The clinical presentation shows imprecise bone pain, while radiologic findings and histologic exam are similar to those of infectious osteomyelitis. It is a rare disease, which is difficult to distinguish from other diseases, therefore it could be misdiagnosed. We report on a case of CRMO on the distal tibia and fibula in an 11-year-old boy.
Subject(s)
Adolescent , Child , Humans , Male , Bone Diseases , Fibula , Osteomyelitis , Rare Diseases , TibiaABSTRACT
La osteomielitis crónica multifocal recurrente es una rara enfermedad ósea de niños y adolecentes que afecta las metáfisis de los huesos tubulares. Clínicamente se manifiesta por un inicio insidioso y un curso con exacerbaciones y remisiones, los hallazgos de laboratorio y de patología son inespecíficos. En los estudios de imágenes simula una osteomielitis infecciosa y el diagnóstico se hace después de descartar otras enfermedades. El curso crónico, los hallazgos en imagen, los cultivos negativos y la falta de respuesta a la terapia antibiótica ayudan a su diagnóstico.
Chronic recurrent multifocal osteomyelitis is anunusual inflammatory bone disease; it is a disease of children and young adults. Clinically, the disease is characterized by an insidious onsetand its course is one of recurrent periods of exacerbation and remission. Histopathological,laboratory findings and bacterial culture results are usually negative or no conclusive. The diagnosisis ultimately one of exclusion after morecommon entities have been rule out. The definitive diagnosis is made by combination of a prolongedcourse, characteristic imaging findings,negative cultures and lack of response to an antimicrobial therapy.
Subject(s)
Humans , Child , Adolescent , Bone Diseases , Osteomyelitis , RadiologyABSTRACT
Tubercular osteomyelitis of mandible is an extremely rare condition, particularly in comparison to pyogenic infections and neoplastic diseases involving the mandible. We report a case of tubercular osteomyelitis of mandible in an old chronic smoker woman initially suspected of neoplasia, proved later on as primary tubercular osteomyelitis in absence of a primary focus.
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Chronic recurrent multifocal osteomyelitis (CRMO) is a rare and recently recognized disease of unknown etiology, characterized by remission and exacerbation of multiple bone lesions which radiologically and pathologically have the appearance of hematogenous osteomyelitis. The natural history appears to be slow and spontaneous resolution of the osseous lesions without specific treatment. And antimicrobial agents seem to have no beneficial effect. In proper clinical setting, CRMO should be considered, because recognition of this entity would help us to avoid costly and potentially harmful diagnostic and therapeutic interventions. We report a case of a 48-year-old adult who had chronic recurrent multifocal osteomyelitis.
Subject(s)
Adult , Humans , Middle Aged , Anti-Infective Agents , Natural History , OsteomyelitisABSTRACT
Chronic recurrent multifocal osteomyelitis is rare, its etiology is unknown, and it most commonly occurs during childhood or adolescence. It is characterized by an insidious onset of fever, local swelling and pain in affected bones, and radiological abnormalities suggestive of osteomyelitis. We experienced one case of chronic recurrent multifocal osteomylitis. The lesions were in tibia, radius.