Does sacroiliitis is a mandatory criterion for enthesitis-related arthritis diagnosis?

INTRODUCTION AND OBJECTIVES: Magnetic resonance imaging (MRI) sensitivity and specificity seem to be less studied in enthesitis-related arthritis (ERA). We aimed to determine the ability of sacroiliac MRI to diagnose ERA patients. MATERIALS AND METHODS: We conducted a retrospective study including 44 patients with juvenile idiopathic arthritis (JIA). Each patient had a sacroiliac joint MRI. We divided patients into two groups: G1 patients with ERA and G2 patients with non-ERA subtype. RESULTS: ERA was noted in 61% of the cases. Sacroiliac joints were painful in 15 patients (34%). MRI was normal in 25 patients (57%) (G1:11 versus G2:14) and showed bone marrow edema in the sacroiliac joints in 19 patients (34%) (G1=16 versus G2=3, p=0.005). Sacroiliac joints MRI's sensitivity and specificity in the ERA diagnosis were 61.54% and 82.35%, respectively. Positive and negative predictive values were 84.21% and 58.33%, respectively. Furthermore, sacroiliac joint pain in the clinical examination was able to predict sacroiliac bone edema in MRI with an odds ratio of 6.8 (95% CI 1.68-28.09; p=0.006). CONCLUSION: Our study showed that sacroiliac joint MRI has good specificity and positive predictive value in the diagnosis of ERA patients among JIA patients. This underlines the usefulness of sacroiliac joint MRI in the early diagnosis of ERA patients.

Management of Blau syndrome: review and proposal of a treatment algorithm.

Blau syndrome is a rare genetic granulomatosis affecting children. It could be responsible for vision-threatening complications and articular deformation. Due to the rarity of this disease, there are no standardized guidelines for its management. This work aimed to provide an updated overview of the different therapeutic options for Blau syndrome. We conducted research in the PubMed database for the different treatments used in Blau syndrome patients, and we proposed a therapeutic algorithm for disease management. High doses of corticosteroids are considered as a bridging therapy in Blau syndrome. Methotrexate should be initiated if the patient has articular or ocular involvement. An anti-tumor necrosis factor α should be added for patients with uveitis or residual arthritis. If the patient remains symptomatic, a switch to another anti-tumor necrosis factor α is the best option. In non-responders to the first- and second-line biotherapies, a switch to an anti-interleukin 1, an anti-interleukin 6, or tofacitinib is necessary. CONCLUSION: This article suggested an algorithm for the treatment of Blau syndrome. Other studies are necessary to confirm the efficacy of these treatments. WHAT IS KNOWN: • Blau syndrome is a rare but severe granulomatosis that could be responsible for vision-threatening complications and articular deformation. • Blau syndrome seems to be refractory to treatments. WHAT IS NEW: • High doses of corticosteroids are usually insufficient and should be considered only as a bridging therapy. • Blau syndrome could be considered as a poor factor for uveitis, thus, an anti-tumor necrosis factor α should be initiated for patients with uveitis or with residual arthritis.

Sarcopenia: a new issue in juvenile idiopathic arthritis. A study Protocol.

INTRODUCTION: The decrease in muscle function and mass is defined as sarcopenia. Known for a long time as an age-related disorder, sarcopenia is nowadays well recognized in childhood. Juvenile idiopathic arthritis (JIA), a chronic inflammatory joint disease may be associated with loss of skeletal mass. AIM: This protocol aims to evaluate the prevalence rate of sarcopenia and its associated factors in JIA. METHODS: To evaluate the prevalence rate and factors associated with sarcopenia in juvenile idiopathic arthritis, we are enrolling 30 children with JIA and 30 healthy children aged between 4-and 16 years. Clinical data will report: age, sex, body mass index, disease duration, and therapeutic management. All participants will undergo the Whole-body Dual-energy X-ray absorptiometry to assess the skeletal muscle mass. The muscle strength will be measured using the handgrip dynamometer and adjusted to the body mass index. Data will be analyzed and compared to age and sex reference curves. RESULTS: This study aims to detect sarcopenia in JIA children and identify subsequently the main associated factors. By collecting anthropometric data and extracting the main features of the disease, specific metrics will be extracted. Body composition will be obtained using the DXA scans, including appendicular lean mass and skeletal muscle mass. Muscle strength will also be assessed. CONCLUSION: This study aims to assess sarcopenia in JIA patients, using the sarcopenia update definition. If we will provide conclusive results, it will be possible to better identify the associated factors of sarcopenia and to prevent children from this complication. Clinical trials registration NCT05291416.

Foot-related impairment in children with juvenile idiopathic arthritis.

AIM: Foot involvement is present in approximately 60%-90% of children with Juvenile idiopathic arthritis (JIA). It is a major cause of disability, which can lead to deterioration in daily activities and quality of life. However, it is often overlooked and can compromise patient management. PURPOSE: Our objective was to describe the ankle and foot involvement in JIA and to appraise the functional impact of this damage on the child's performance and quality of life by using validated scores. METHODS: The cross-sectional study included patients with JIA. Functional impairment was assessed through the Juvenile Arthritis Functionality Scale (JAFS) and the Oxford Ankle and Foot Questionnaire for children. RESULTS: Twenty-three patients aged 12.75 ± 3.9 and with 41 months of disease duration. After completing the Oxford score, the physical domain appeared to be the most altered. A long delay in diagnosis was associated with an alteration in the emotional domain of the Oxford score. Higher disease activity was significantly associated with impairment in both the physical activity and the footwear domains of the Oxford score. As regards clinical examination data, the presence of foot pain as well as the presence of tendinopathy were associated with an alteration of all Oxford score domains. The presence of flat feet significantly affected all domains of the Oxford score. The JAFS was reported to affect the child's performance ability and was associated with impairment in physical activity, school and play, and emotional domains. CONCLUSION: Ankle and foot involvement was common in our study. Functional ability was most impaired in the lower limbs. High disease activity, foot and ankle pain, tenosynovitis, and flat feet were associated with poorer quality of life and higher functional impact.

Primary pachydermoperiostosis associated with pigmented villonodular synovitis: An unknown association?

Primary pachydermoperiostosis is a rare genetic disease affecting the skin and musculoskeletal system. In contrast to secondary hypertrophic osteoarthropathy, primary pachydermoperiostosis is considered a benign condition. While a variety of associated abnormalities have been described in this form, any association with tumors was previously reported in the literature. We hereby describe the first case of a 20-year-old man with primary pachydermoperiostosis revealed by a knee synovial tumor.

Management of chronic recurrent multifocal osteomyelitis: review and update on the treatment protocol.

INTRODUCTION: Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory disorder primarily affecting children. It is characterized by a peripheral involvement of the metaphysis of long bones rather than axial involvement. Due to the scarcity of the disease, there are no guidelines regarding its management. AREAS COVERED: This review aims to provide an overview of the different therapeutic alternatives and recent protocols. For this reason, first-line and second-line treatment, as well as the impact of new therapies, are discussed in depth. We conducted a search through PubMed on the different aspects of CRMO. Outcomes were categorized as first and second-line treatments. EXPERT OPINION: Non-steroidal anti-inflammatory drugs remain the keystone of CRMO management and are proposed as the first-line treatment. In the case of vertebral involvement, bisphosphonate should be considered, even as a first-line treatment. Several case series and retrospective studies highlight the efficacy of anti-TNF agents. Their use could be an optimal treatment choice for CRMO with comorbid immune-mediated diseases. The potentially favorable effect of interleukin-1 antagonists remains to be determined.

Enthesitis-related arthritis: monitoring and specific tools.

OBJECTIVES: In this article, the authors aimed to review the different tools used in the monitoring of enthesitis-related arthritis. SOURCES: The authors performed a literature review on PubMed, Google Scholar, and Scopus databases. The dataset included the original research and the reviews including patients with enthesitis-related arthritis or juvenile spondylarthritis up to October 2020. SUMMARY OF FINDING: Enthesitis-related arthritis is a category of juvenile idiopathic arthritis. It is characterized by the presence of enthesitis, peripheral arthritis, as well as axial involvement. The only validated tool for disease activity measurement in juvenile idiopathic arthritis is the Disease Activity Score: It has proven its reliability and sensitivity. Nevertheless, due to an absence of validated evaluation tools, the extent of functional impairment, as well as the children and parents' perception of the disease, could not be objectively perceived. Despite the great progress in the field of imaging modalities, the role they play in the evaluation of disease activity is still controversial. This is partially due to the lack of validated scoring systems. CONCLUSIONS: Further work is still required to standardize the monitoring strategy and validate the outcome measures in enthesitis-related arthritis.

Radiculopathies caused by spontaneous pneumorrachis: Two case reports and review of literature.

Pneumorarchis is a rare condition of low back pain. It is usually asymptomatic and secondary to trauma. Spontaneous pneumorrachis is scarce.

Intra-articular hip neurofibromas in von Recklinghausen's disease.

Neurofibromatosis is a genetic disorder with osteo-articular manifestations. The intra-articular location of neurofibroma is scarce.

Progressive pseudorheumatoid dysplasia: A rare entity mimicking juvenile idiopathic arthritis.

Progressive pseudorheumatoid dysplasia can be confused with juvenile idiopathic arthritis. Treatment is mainly symptomatic and the prescription of immunosupressive agents is unnecessary. Surgery may be indicated at advanced stages of the disease.

A rare cause of lumbar nerve root compression: Extramedullary hematopoiesis in a patient with thalassemia major.

Extramedullary hematopoiesis (EMH) can rarely involve the epidural space, leading to spinal cord compression syndrome. No treatment guidelines have been established yet.

Sleep disturbances in chronic rheumatic diseases: Is disease activity the major determinant factor?

INTRODUCTION: Sleep disturbances are closely related to chronic pain processes, especially in patients with inflammatory and mechanical joint diseases. OBJECTIVES: This study aims to report sleep characteristics in patients with rheumatoid arthritis (RA) and knee osteoarthritis also to determine the responsibility of disease activity in the occurrence of sleep disturbances during chronic rheumatic diseases. METHODS: We conducted a comparative study between two groups of patients: the first with RA, the second with primary knee osteoarthritis. We reported sociodemographic and medical data (clinical and biological inflammatory syndrome data). Then, we assessed depression, anxiety, and sleep disturbances with respectively Beck Depression Inventory, Beck Anxiety Inventory and Medical Outcome Study Sleep Scale (MOS-SS). RESULTS: Seventy RA patients aged 51.9 years, with an average of 77.1% female were studied. The mean disease duration was 6.9 years. All sleep domains were altered in these patients. Forty patients with knee osteoarthritis aged 57.5 years with an average of 70% female were included. The mean disease duration was 4 years. The most impaired domains in this group were somnolence and sleep disturbance. Multivariate analysis concluded that risk factors independently related to RA were:  disease activity score, functional disability, depression, anxiety, and body mass index. Disease activity score were the only parameter to influence all domains of sleep. CONCLUSION: So we result that disease activity is a risk factor independently related to sleep disturbances in rheumatoid arthritis. Furthermore, mood disorders and obesity also deteriorate several sleep domains. These factors must be considered in the management of chronic rheumatic disorders.