Cervical Spine Involvement Research Articles

Hereditary multiple exostoses with cervical spine involvement: a case report: Retraction.

[This retracts the article DOI: 10.1097/MS9.0000000000002452.].

Clinical characteristics and prognosis of interstitial lung disease in systemic juvenile idiopathic arthritis: a two-center retrospective observational cohort study

BackgroundInterstitial lung disease (ILD) is a serious complication in systemic juvenile idiopathic arthritis (SJIA). This study aimed to identify the clinical characteristics and prognosis of SJIA-ILD.MethodsA two-center retrospective cohort study was conducted on patients newly diagnosed with SJIA in China from October 2010 to December 2021. Clinical characteristics, laboratory parameters, outcomes, and relapse rates were compared between ILD and non-ILD groups.ResultsA total of 176 children with SJIA were included, including 35 in ILD group and 141 in non-ILD group. The median age at onset of SJIA was 5.8 years (range 4.4–9.5) in patients with SJIA-ILD. It exhibited higher incidences of cervical spine (28.6%) and hip involvement (40.0%) in ILD group (P = 0.031 and P = 0.029, respectively). The incidence of macrophage activation syndrome (MAS) in ILD group reached up to 40%, significantly elevated than that in non-ILD group (P = 0.047). Children with ILD demonstrated a stronger inflammatory response and were more prone to developing lymphopenia (P = 0.009), requiring more combination therapy (P = 0.006) to control disease activity. 54.3% of patients received biologic therapies, with only three patient receiving biologics (one with IL-6 blockade, two with TNF inhibitor) prior to ILD onset and none receiving IL-1 blockade. The median follow-up duration was 6.0 years (range 3.9–9.5). The proportions of patients with SJIA-ILD achieving clinical inactive disease without glucocorticoids within 6 to 12 months of the treatment were significantly lower than control group (45.7% vs. 70.2%, P = 0.006). In ILD group, only 54.3% of patients achieved complete remission, and 17.1% were in a non-remission state, among whom two deaths from respiratory failure. There was no significant difference in disease relapse rates between the two groups (P > 0.05).ConclusionsPatients with SJIA-ILD exhibited heightened inflammation, increased hip joint and cervical spine involvement, and were more susceptible to developing lymphopenia and MAS, suggesting a relatively poor prognosis. They required a prolonged time to control inflammation and more aggressive treatment strategies to achieve inactive status. The unsatisfactory rate of complete remission highlighted an urgent need for focused clinical strategies.

Radiological features of dropped head syndrome.

This study aimed to elucidate the specificity of the radiological features of Dropped head syndrome (DHS) from both reginal and global aspects. We enrolled 53 patients with DHS (8 men, 45 women; mean age 73.5 years), and captured their lateral spinopelvic radiographs in standing position. We also selected 21 age- and sex-matched controls with cervical spondylosis. Radiological parameters were measured and compared between two groups. Compensatory and decompensatory sites were also listed for each patient. Radiological factors such as sagittal vertical axis (SVA), clivo-axial angle (CAA), C2-7 angle, C2-7 SVA, anterior slippage of the vertebra, alignment. C1, C2, C3, C4, C5, C6 slopes, and T1 slope-C2-7 angle showed statistically significant differences between the groups. Multivariate logistic regression showed that SVA, C2-7 SVA, T1-slope-C2-7 angle, and C1 slope were the most important factors specific to DHS. Sole cervical spine and involvement of both cervical and thoracic spine accounted for 22% and 29% of the decompensatory sites in DHS respectively. Notably, 24% of the patients did not show decompensation of the cervical spine. While, 93% exhibited compensation at the craniovertebral junction. The thoracic spine contributed 70% to DHS compensation. This study indicated the radiological features of DHS from both regional and global aspects. Compensatory and decompensatory DHS mechanisms varied among individuals. Compensation was likely to be developed at the neighboring sites, with the craniovertebral and thoracic junctions as the proximal and distal parts for DHS, respectively.

Hereditary multiple exostoses with cervical spine involvement: a case report

Introduction and Importance: Hereditary multiple exostoses (HME) is a rare genetic disorder characterized by the formation of multiple benign bone tumors known as osteochondromas. Management of HME involves a multidisciplinary approach encompassing surgical expertise and genetic counseling. This case report highlights the significance of such an approach in optimizing outcomes for patients with HME, emphasizing the importance of individualized treatment strategies and future management. Case Presentation: We present the case of a 12-year-old male patient with a history of HME, who presented with symptoms including progressively enlarging and painful mass at the posterior aspect of his neck since last year. Surgical intervention was undertaken to address symptomatic lesions, focusing on preserving joint function and alleviating discomfort. The surgical approach involved careful consideration of lesion characteristics and their impact on surrounding structures. Clinical Discussion: The case shows the importance of a tailored surgical approach in managing symptomatic osteochondromas associated with HME, highlighting the need for careful consideration of lesion characteristics and their impact on joint function. By integrating surgical expertise with genetic counseling, clinicians can optimize outcomes while addressing familial implications and ensuring informed decision-making. Conclusion: This case report illustrates the significance of a multidisciplinary approach encompassing surgical intervention and genetic counseling in the management of hereditary multiple exostoses. Through individualized treatment strategies and informed decision-making, clinicians can optimize outcomes for patients with HME while providing comprehensive support and guidance for future management.

Clinical Features and Surgical Management of Intramedullary Schwannoma of the Spinal Cord

Background contextIntramedullary schwannoma is a kind of relatively rare tumors with only a few literature reports. This study was aimed to report the clinical characteristics of intramedullary schwannoma and discuss imaging findings and treatment strategies. MethodsThe inclusion criterion was consecutive patients with intramedullary schwannomas who were surgically treated in our institution between 2017 and 2022. Data included clinical characteristics, radiological features, surgical management and prognosis. Clinical and follow-up details of all cases were collected and reviewed. ResultsThere were three males and eight females. The mean age of all patients was 45 years old (Range 26-77 years). Cervical spine (4 cases, 36.4%), thoracic spine (4 cases, 36.4%) and lumbarsacral spine (3 cases, 27.3%) involvement were found respectively. Weakness, numbness and pain of limbs were the main symptoms at administration. Pre-operative MRI all demonstrated lesion with spinal cord medullary invasion and well demarcated margins. The post-operative histological examination all showed benign lesions and confirmed the schwannoma. ConclusionsIntramedullary schwannoma is a very rare tumor. This article presented a series of 11 cases of intramedullary schwannoma with sharp margins and well-enhanced feature. Prognosis and functional recovery were good after gross total resection.

Case Report: 14 Years-Old Boy with Paraplegia and Aneurysmal Bone Cysts of Neck who Responded to Anti-Tuberculosis Treatment

This article discusses a rare case of Aneurysmal Bone Cyst (ABC) affecting the cervical spine, presenting with neck pain and progressive weakness in all extremities. A 14-year-old boy experienced these symptoms for three years without any history of trauma. Physical examination revealed weakness and tenderness in the cervical vertebrae. MRI showed cervical compression, paravertebral abscess, spinal nerve swelling, and canal stenosis, suggestive of tuberculosis spondylitis. The surgical intervention involved laminectomy and tumor debulking, confirming the diagnosis of a aneurysmal bone cyst. Despite initial motor deficits, anti-tuberculosis treatment significantly improves motor function and neck mobility over several weeks. This case underscores the rarity of cervical spine involvement in aneurysmal bone cyst and highlights the importance of prompt diagnosis and treatment to prevent neurological complications and achieve favorable outcomes. The study emphasizes considering aneurysmal bone cyst as a potential differential diagnosis for lytic lesions involving the spine in young patients, emphasizing the need for vigilance in clinical practice.

Cervical Disc Hernia Following a Jet-Ski Accident

Authors report the case of an adult man showing cervical spine involvement with radicular pain of acute onset. The happening followed a Jet Ski use for recreational purposes, and the patient did not had any training before the event. MRI of the cervical spine showed a massive disc involvement at the C6-7 level, and a conservative treatment with supportive collar was warranted. Particular attention should be made with the advent of personal watercraft (PWC) engines and devices in our area, unknown before both to users or clients as well as to medical staff. PWC might show a wide range of bodily injuries, some of them even lethal, therefore guidelines and precautions might be needed extensively in the next future.

Characteristics of patients with psoriatic arthritis in Korea: focusing on axial involvement.

We aimed to clarify the clinical characteristics of psoriatic arthritis (PsA) in Korean patients focusing on PsA with axial involvement. A retrospective medical chart review was performed to identify PsA patients at a single tertiary center. Cases of AS patients with psoriasis were recruited from a prospective AS registry of the same center. Demographics, laboratory findings, and radiologic characteristics were assessed. A total of 69 PsA patients were identified. In PsA patients, spondylitis (46.4%) was the most common form. Compared to AS patients with psoriasis, PsA patients with radiographic axial involvement were older (50.9 vs. 32.4 years; p < 0.001) and showed greater peripheral disease activity (peripheral arthritis 78.1 vs. 12.5%, p < 0.001; enthesitis 50.0 vs. 6.3%, p = 0.003). AS patients with psoriasis presented a higher rate of HLA-B*27 positivity (81.3 vs. 17.2%; p < 0.001) and a more frequent history of inflammatory back pain (100.0 vs. 75.0%; p = 0.039) than PsA patients with radiographic axial involvement. Significant proportions of PsA patients with radiographic axial involvement had cervical spine involvement (10/18, 55.6%) and spondylitis without sacroiliitis (10/23, 43.5%). We demonstrate that axial involvement is common in Korean PsA patients, and its characteristics can be distinct from those of AS.

Penetrating Cervical Injury Due to Throat Slitting in Children: 1 Case Reported at Mopti Hospital (Mali)

The impact of the media and security crises (rebellions, acts of terrorism, intercommunity conflicts) on children often results in the practical reproduction of what they see and hear. Anterior penetrating injuries to the neck pose a life-threatening situation and the causes vary among countries around the world. They include stab wounds, gunshot wounds, vehicle accident injuries, and neck lacerations. They pose a great challenge to treating surgeons because of the important structures in the neck that can be affected. We report a case of knife slitting of a child by one of his classmates during a simulation game in a conflict zone in central Mali. A 5-year-old boy was cut in the throat by a 9-year-old friend with a knife. According to the patient's father who testified, the cut was committed in the play area where the oldest friend placed the patient in a supine position and then cut him in the throat with a knife; as if to imitate adults. On exploration, the lesion assessment found an anterior cervical wound which was horizontal with linear borders located between the sternocleidomastoid muscles without damage to them and the large vessels. A partial section of the esophagus and trachea was noted. There was no neurological or cervical spine involvement on X-ray. Laryngo -tracheal repair with two control tractor wires and then orotracheal re-intubation; Finally a suture of the sternothyroid muscles and skin closure. The postoperative course was simple. Penetrating trauma is rare in children, their management is a challenge. They are serious and put the patient's vital prognosis at stake.

P29 A rare case of complex multi-system treatment resistant rheumatoid arthritis

Abstract Introduction Rheumatoid arthritis (RA) is a chronic progressive systemic inflammatory disorder with multi-organ involvement. The introduction of biologics has revolutionised the disease management. However, we still have 5-20 percentage of patients who pose clinical challenge with treatment resistant and multi-organ involvement. Cervical spine involvement among RA patients is well noted with a prevalence of 24 percent, however involvement of thoracic and lumbar spine is not commonly known. We present one such case which is treatment resistant, multiple organ involvement disease, associated with the axial involvement, complicated by infection posing clinical challenge for management. Case description 58-year-old Chinese origin woman, initially diagnosed with RA and Sjogren’s syndrome in 2018. RF and Anti-CCP positive with active synovitis of her hands confirmed on ultrasound. Initially treated with hydroxychloroquine, sulfasalazine, and methotrexate of which she failed to tolerate hydroxychloroquine and sulfasalazine. A year later she developed progressive SOB. Pulmonary function test and CT-thorax confirmed RA related progressive interstitial lung disease. The respiratory team offered anti-fibrotic therapy as a part of clinical trial but she opted for conservative management. Given the high disease activity score, she had 2 cycles of rituximab along with methotrexate, but this failed to control symptoms. During this period, she had multiple intramuscular and intravenous methylprednisolone infusions to alleviate the symptoms. In 2020, due to high disease activity (DAS-6.93) and lower infection risk, abatacept was administered for 12 months but had partial response. Following year, she presented with abdominal pain, deranged liver functions and back pain to hospital with raised inflammatory markers. MRCP suggested biliary ductal dilatation secondary to distal biliary stricture and was managed conservatively. MRI of the whole spine confirmed inflammatory changes within the spinal column mainly involving lumbar and sternoclavicular joints, consistent with inflammatory arthropathy. HLA B 27 was negative. Her disease continued to progress (DAS28 7.7, CRP 76mg/L). Her mobility status reduced drastically by this point leading to bed sores with risk of infection. Hence switched to sarilumab with weekly methotrexate and tapering steroids. Unfortunately, 6 months later she developed pneumonia requiring hospitalization and she refused to go back on sarilumab. Since early 2023 she has been on tocilizumab, methotrexate and oral prednisolone. Tocilizumab was given fortnightly as she developed neutropenia and haematology input was taken. In the last few months, she developed red, gritty eyes and was diagnosed with scleritis. Currently on steroid eye drops with advice on further immunosuppression. Discussion This case focuses on the multiple challenges in managing treatment resistant rheumatoid arthritis. Even with the availability of a wide range of biologics to manage RA, the patient continues to have SOB, pressure sores, active synovitis, inflammatory back pain with lumbar involvement, scleritis and neutropenia. There are times when it is difficult to achieve remission despite treatment and that is referred to as resistant or difficult-to-treat rheumatoid arthritis (D2T RA). The above case highlights one such scenario where resistant RA was further complicated with multi system involvement. Patients with interstitial lung disease are at high risk of developing pneumonia and immunosuppression increases that risk further. This can be seen in our case where she required hospitalisation with temporary suspension of her immunosuppressant leading to further worsening of the disease activity. Along with peripheral joints, spine involvement led to significant reduction in her mobility status resulting in development of bed sores. There is literature extensively discussing the impact of rheumatoid arthritis (RA) on the cervical spine. However, there has been limited focus on the rarer occurrence of rheumatoid involvement in the thoracic and lumbar regions. In addition, there was patient’s altered perception of rheumatoid arthritis and its symptoms, influencing the medication adherence and coping mechanism, which added to the management dilemmas. Moreover, one cannot escape from the impact of COVID pandemic and isolation measure that may have created more anxiety among our patients. Overall, now patient continues to be with high disease activity, infection risk, reduced mobility, multi-biologic resistance in the background of neutropenia which has posed real challenge in her management. Patient specific biologic treatment would help in such situation, and we need more evidence to introduce this in clinical practice. Key learning points Cervical spine involvement is well known among patients with RA but it is important to be aware that other areas of the axial skeleton may also be affected. Recognizing these symptoms and initiating early investigations are crucial steps towards prompt diagnosis and appropriate management, ultimately leading to improved outcomes for individuals with RA. There is a need for more evidence in developing a patient specific treatment for patients with difficult-to-treat rheumatoid arthritis (RA) who are unable to attain the treatment goal with current biologics strategies. Patients perception, belief about the disease and adherence to medication influence the disease management and thus clinicians need to prioritize patient education about the disease to enhance the effectiveness of treatment early in the treatment.

Cervical Spine Involvement: A Rare Entity in Psoriasis

Cervical Spine Involvement: A Rare Entity in Psoriasis

Advances in surgical treatment for atlantoaxial instability focusing on rheumatoid arthritis: Analysis of a series of 67 patients.

An estimated 88% of rheumatoid arthritis (RA) patients experience various degrees of cervical spine involvement. The excessive movement of the atlantoaxial joint, which connects the occiput to the upper cervical spine, results in atlantoaxial instability (AAI). AAI stabilization is usually achieved by C1 lateral mass-to-C2 pedicle screw-rod fixation (LC1-PC2 fixation), which is technically challenging in RA patients who often show destructive changes in anatomical structures. This study aimed to analyze the clinical results and operative experiences of C1-C2 surgery, with emphasis on the advancement of image-guided surgery and augmented reality (AR) assisted navigation. We presented our two decades of experience in the surgical management of AAI from April 2004 to November 2022. We have performed surgery on 67 patients with AAI, including 21 traumatic odontoid fractures, 20 degenerative osteoarthritis, 11 inflammatory diseases of RA, 5 congenital anomalies of the os odontoideum, 2 unknown etiologies, 2 movement disorders, 2 previous implant failures, 2 osteomyelitis, 1 ankylosing spondylitis, and 1 tumor. Beginning in 2007, we performed LC1-PC2 fixation under C-arm fluoroscopy. As part of the progress in spinal surgery, since 2011 we used surgical navigation from presurgical planning to intraoperative navigation, using the preoperative computed tomography (CT) -based image-guided BrainLab navigation system. In 2021, we began using intraoperative CT scan and microscope-based AR navigation. The technical complexities of C1-C2 surgery can be mitigated by CT-based image-guided surgery and microscope-based AR navigation, to improve accuracy in screw placement and overall clinical outcomes, particularly in RA patients with AAI.

E13 Cervical spine involvement in juvenile idiopathic arthritis: frequency and associated factors

Abstract Background Juvenile idiopathic arthritis (JIA) is a pediatric rheumatism including several subgroups. The cervical spine involvement is poorly studied in JIA compared with adult rheumatism. Objectives This study aimed to assess the frequency and associated factors with cervical spine involvement in JIA. Methods We conducted a retrospective study including adults with long-standing JIA according to International League of Associations of Rheumatology (ILAR) criteria over a 28-year period (1994 – 2022). We retrieved radiographs of the cervical spine in anteroposterior, lateral, and lateral flexion views in search of an atlantoaxial dislocation (AAD). Demographic, clinical, biological, and radiological data were compared according to the presence or absence of AAD. Results A total of 29 Patients were enrolled (12 men and 17 women), the mean age was 35.69 ± 11.72 [18–61] years. The mean age of disease onset was 11.10 ± 4.25 [2–16] years. The average diagnostic delay was 52.96 ± 95.97 [0–336] months. The average disease duration was 24.48 ± 12.76 [1–47] years. The mean body mass index (BMI) was 21.20 ± 4.88 [14.17–27.55] kg/m2. Most patients had a polyarticular form (55.2%, n = 16). Mean C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were 42.74 ± 63.37 [2–218] mg/l and 69.67 ± 36.23 [36–108] mm/h. A biological inflammatory syndrome was present in 19 cases. Rheumatoid factor, ACPA and anti-nuclear antibodies were observed in 12, 7 and 5 cases respectively. At least one extra-articular involvement was noted in 16 cases. Bone erosions and hip involvement were noted in 18 and 14 cases, respectively. AAD was noted in 4 patients (13.8%). It was diagnosed on average 4.28 ± 10.07 [0–27] years after disease onset. AAD was only associated with extra-articular involvement in our study (75% vs 13.6%; P = 0.047). On the other hand, our study showed that age, gender, age at onset, diagnostic delay, duration of symptoms, BMI, JIA sub form, inflammation markers, rheumatoid factor, ACPA, antinuclear antibodies, bone erosions, and hip involvement were not associated with AAD. Conclusion The cervical spine involvement was not negligible in our study and was associated with extra-articular involvement. This condition is serious and can be life threatening. Therefore, it is important to perform more studies to better evaluate its frequency, clinical presentation, and imaging.

E10 Temporo-mandibular joint involvement risk factors in juvenile idiopathic arthritis

Abstract Introduction Recent recommendations suppose temporomandibular joint (TMJ) involvement in juvenile idiopathic arthritis (JIA) is a risk factor of poor outcomes and severe disease course. Objectives To compare the clinical characteristics of patients with juvenile idiopathic arthritis with and without TMJ involvement. Methods We analyzed the data of 753 patients with juvenile idiopathic arthritis aged 2–17 years, depending on TMJ arthritis (n = 43; 5.7%) or no TMJ arthritis (n = 710; 94.3%). Clinical, laboratory characteristics and treatment regimens were compared. Odds ratio (OR) analysis of sensitivity (Se) and specificity (Sp) was performed to obtained the predictors of TMJ involvement. Results Despite the similar age of onset, TMJ arthritis was associated with longer course of the disease, polyarticular JIA category, use of systemic corticosteroids and longer achievement of remission with similar rate of biologic administration. TMJ arthritis was associated with involvement of cervical spine, hip and shoulder arthritis. The main risk factors, associated with TMJ involvement in JIA were active joints &amp;gt; 8 (OR = 14.9 (5.8; 38.3), P = 0.0000001), delayed remission &amp;gt; 7 years (OR = 3.1 (1.6; 5.8), P = 0.0004), delayed hip involvement (OR = 4.6 (0.9; 22.6), P = 0.041), hip osteoarthritis (OR = 4.0 (1.2; 13.0), P = 0.014), and avascular necrosis of the hip. Cervical spine arthritis (OR = 10.3 (5.4; 19.8), P = 0.000001), and corticosteroid treatment (OR = 2.3 (1.2; 4.4), P = 0.0007) were associated with TMJ arthritis. Patients with TMJ arthritis required more biologics (OR-3.2 (1.6; 6.2), P = 0.0006). Oligoarticular JIA category and uveitis were protective against TMJ involvement. Data are in the table. Conclusion TMJ involvement is marker of poor disease prognosis. Early initiation of biologics and avoidance of corticosteroids might improve the risk of TMJ involvement. Ethics Written consent was obtained according to the declaration of Helsinki. This retrospective study's protocol was approved by the local Ethical Committee of Saint Petersburg State Pediatric Medical University (protocol number 11/10 from 23.11.2020).

E17 Juvenile idiopathic arthritis: are girls less lucky?

Abstract Introduction Juvenile idiopathic arthritis (JIA) is one of the most common chronic diseases in children. ∼3 million children and young adults worldwide have JIA, with higher prevalence rates in girls. Objectives Our objective was to assess the relationship between gender and disease characteristics. Methods A retrospective study including adults with long-standing JIA according to International League of Associations of Rheumatology (ILAR) criteria over a 28-year period from 1994 to 2022. We collected clinical, biological, and radiological disease characteristics from medical records. These parameters were compared according to gender. Results We have included 29 patients (17 women and 12 men). The mean age was 35.69 ± 11.72 [18–61] years. Symptoms began at an average age of 11.10 ± 4.25 [2–16] years and have been evolving since 24.48 ± 12.76 [1–47] years. The average diagnostic delay was 52.96 ± 95.97 [0–336] months. The mean body mass index (BMI) was 21.20 ± 4.88 [14.17–27.55] kg/m2, 8 patients had a normal BMI. Growth delay was noted in 3 cases. Sixteen patients (55.2%) had a polyarticular form. The presence of rheumatoid factor (RF), ACPA and anti-nuclear antibodies was noted in 12, 7 and 5 cases, respectively. At least one extra-articular manifestation was noted in 16 patients: ophthalmologic (n = 6), skin (n = 4), cardiac (n = 4), pulmonary (n = 3) and renal (n = 2) involvement. Osteoporosis was noted in 7 cases. Mean C-reactive protein (CRP), erythrocyte sedimentation rate (ESR) and haemoglobin value level 42.74 ± 63.37 [2–218] mg/l, 69.67 ± 36.23 [36–108] mm, and 11.31 ± 2.10 [6.5–15] g/dl. An elevation of inflammation markers was noted in 19 cases. Anaemia was noted in 20 cases. Bone erosions were noted in 18 cases. Hip and cervical spine involvement were noted in 14 and 4 cases, respectively. Our comparative study showed that rheumatoid factor positivity was significantly associated with female gender (73.3% vs 16.7%; P = 0.018). No other significant gender differences were identified when comparing the rest of the clinical, biological, and radiological variables. The correlation study concluded the following correlations of gender with haemoglobin level (r = -0.574; P = 0.001), rheumatoid factor positivity (r = 0.517; P = 0.016) and JIA subform (r = -0.482; P = 0.008). Conclusion A relationship of gender to rheumatoid factor positivity, haemoglobin level, and JIA subform was identified in our study. Larger-scale longitudinal studies would better investigate the influence of gender on JIA.

Temporomandibular Joint Involvement in Juvenile Idiopathic Arthritis: The Results from a Retrospective Cohort Tertial Center Study.

Our study aimed to evaluate the clinical and laboratory features of juvenile idiopathic arthritis (JIA) children with temporomandibular joint (TMJ) arthritis. In the retrospective cohort study, we analyzed data of 753 patients with JIA aged 2-17 years, depending on TMJ arthritis or not. TMJ arthritis can to be diagnosed in the presence of at least two of the following clinical signs of inflammation: pain in TMJ, jaw opening limitation, jaw opening deviation, and micrognathia. We compared clinical, laboratory, and treatment features in JIA patients depending on the involvement of TMJ. TMJ arthritis was detected in 43 (5.7%) of our patients and associated with a longer course of the disease, polyarticular JIA category, treatment with systemic corticosteroids, and longer achievement of the remission and involvement of cervical spine, hip, and shoulder. Active joints >8 (OR = 14.9, p = 0.0000001), delayed remission >7 years (OR = 3.1; p = 0.0004), delayed hip involvement (OR = 4.6; p = 0.041), hip osteoarthritis (OR = 4.0; p = 0.014), cervical spine arthritis (OR = 10.3, p = 0.000001), and corticosteroid treatment (OR = 2.3, p = 0.0007) were associated with TMJ involvement. Patients with TMJ arthritis require more biologics (OR = 3.2, p = 0.0006, HR = 2.4, p = 0.005) and have decreased probability of remission achievement (p = 0.014). Consequently, TMJ arthritis was associated with a severe disease course. Early biologic treatment and corticosteroid avoidance might decrease TMJ involvement.

Clinical features of atlantoaxial involvement in patients with rheumatoid arthritis using 18F-fluorodeoxyglucose-positron emission tomography with computed tomography.

Cervical spine involvement is one of the most serious complications in rheumatoid arthritis (RA). The study aim was to assess the clinical significance of atlantoaxial (AA) joint involvement detected by 18F-fluorodeoxyglucose (FDG)-positron emission tomography (PET) with computed tomography (CT) in patients with RA. A prospective cross-sectional study was conducted to evaluate AA joint involvement detected by FDG-PET/CT in consecutive RA patients from December 2017 to February 2022. We investigated the relationship between AA joint involvement and clinical data, including disease activity and patients' cervical symptoms. Among 48 patients enrolled, abnormal FDG uptake at AA joint was detected in 13 (27%). Rheumatoid factor titre, initial disease activity score 28-erythrocyte sedimentation rate and total standardized uptake value were significantly higher in the 13 patients than in the others (P = 0.004, P < 0.001 and P = 0.001, respectively). All patients with abnormal FDG uptake at AA joint had some cervical symptoms regardless of cervical spine X-ray abnormalities. Neck pain on movement and at rest were more frequent in the 13 patients than in the others (P = 0.001 and P = 0.004, respectively). The most sensitive symptom associated with AA joint involvement was neck pain on movement (sensitivity, 69%), and the most specific symptom was neck pain at rest (specificity, 100%). AA joint involvement was commonly observed by FDG-PET/CT in patients with active RA, independent of radiographic findings. Specific cervical symptoms can be important surrogate markers for detection of potential AA synovitis associated with active RA. Key Points • AA joint involvement was frequently seen in RA with high disease activity independent of radiographic findings. • Neck pain was a hallmark of AA joint involvement reflecting disease activity, and resting pain was highly specific.

Facet Articular Irregularity Is the Most Relevant Risk Factor for Rapidly Progressive Degenerative Cervical Myelopathy.

Facet articular irregularity is associated with rapidly progressive degenerative cervical myelopathy (DCM). However, its significance compared with other known risk factors remains unknown. Therefore, this retrospective study aimed to clarify the potential impact of facet articular irregularity as a risk factor for rapid DCM progression. This study included 141 consecutive patients with DCM who underwent surgical treatment at our institution. Clinical variables and radiological findings related to DCM progression were collected. Imaging findings were analyzed at the segmental level of myelopathy in each case. The patients were divided into 2 groups based on the presence or absence of rapid DCM progression, and independent risk factors were determined using logistic regression analyses. Overall, 131 patients with a mean age of 63.9 ± 12.6 years were analyzed; 27 patients (20.6%) were classified into the rapid DCM progression group. The mean age was significantly higher in the rapid progression group than in the slow progression group (72.4 ± 9.6 vs. 61.7 ± 12.4, p < 0.001). According to univariate analysis, facet articular irregularity, dynamic segmental translation (≥ 1.6 mm), upper cervical spine involvement above C4-5, history of cerebrovascular events, preceding minor trauma, local lordotic angle (≥ 4.5°), diabetes, hypertension, ligamentum flavum hypertrophy, and age were independent risk factors. Additionally, multivariate analysis showed that facet articular irregularity was the highest risk factor for rapid DCM progression (p = 0.001). Facet articular irregularity is the most clinically significant finding among the known risk factors in patients with rapid DCM progression.

Predictors factors of atlanto-axial subluxation in Tunisian patients with rheumatoid arthritis.

The aim of this study was to describe clinical and imaging features of atlantoaxial subluxation (AAS) and the associated risk factors in patients with rheumatoid arthritis (RA). We conducted a retrospective and comparative study including 51 RA patients with AAS and 51 RA patients without AAS. Atlantoaxial subluxation was defined by the presence of an anterior C1C2 diastasis on the cervical spine radiograph in hyperflexion and/or an anterior, posterior, lateral or rotatory C1C2 dislocation on MRI with/without inflammatory signal. In G1, clinical presentations revealing AAS were mainly neck pain (68.7%) and neck stiffness (29.8%). MRI revealed: diastasis C1C2 (92.5%), periodontoid pannus (92.5%), odontoid erosion (23.5%), vertical subluxation (9.8%) and spinal cord involvement (7.8%). A collar immobilisation and corticosteroid boluses were indicated in 86.3% and 47.1% of cases. C1-C2arthrodesis was performed in 15.4% of cases. Atlantoaxial subluxation was significantly associated with: age at disease onset (p=0.009), history of joint surgery (p=0.012), disease duration (p=0.001), rheumatoid factor (p=0.01), anti-cyclic citrullinated peptide (p=0.02), erosive radiographic status (p<0.005), coxitis (p<0.001), osteoporosis (p=0.012), extra-articular manifestations (p<0.001), and high disease activity (p=0.001). Multivariate analysis identified RA duration (p<0.001,OR=1.022 CI[1.01-1.034]) and erosive radiographic status (p=0.01, OR=21.236 CI[2.05-219.44]) as predictive factors of AAS. Our study showed that longer disease duration and joint destruction are the major predictive factors of AAS. Early treatment initiation, tight-control and regular monitoring of cervical spine involvement are required in these patients.

Cervical spine involvement in pediatric mucopolysaccharidosis patients: Clinical features, early diagnosis, and surgical management.

Mucopolysaccharidosis (MPS) is a progressive genetic disease that causes a deficiency in lysosomal enzymes, which play an important role in the degradation pathway of glycosaminoglycans. As a result of enzyme defects, mucopolysaccharides cannot be metabolized and thus accumulate. The cervical spine is one of the most commonly involved sites; thus, prompt surgical management before the onset of severe neurological deterioration is critical. However, because of the rarity of the disease, there is no standard treatment. In this review, we characterize the cervical spinal involvement in pediatric patients with MPS, describe the useful imaging technologies for diagnosis, and provide screening procedure for children with MPS. Surgical managements, including indications, surgical methods, possible difficulties, and solutions, are reviewed in detail.