Reliability of the tunisian pediatric gait, arms, legs, and spine: toward a valid screening tool for tunisian children with musculoskeletal conditions.

BACKGROUND: Pediatric musculoskeletal disorders account for 10% of first-line consultations in Tunisia. Referral delay and deficiencies in musculoskeletal screening raise a challenge to the early diagnosis and management of rheumatic conditions in children. The pGALS (Pediatric Gait Arms Legs Spine) was developed and translated into many languages to overcome these deficiencies. Our study aimed to adapt and validate pGALS to the Tunisian dialect for school-age children. METHODS: Using the Delphi method, we carried out a cross-cultural adaptation of the pGALS to the Tunisian dialect. This consensual version was validated in a cross-sectional study, in two pediatric centers. RESULTS: Ninety-two patients were enrolled, 43 females (46.7%) and 49 males (53.3%), mean age was 9.4 ± 2.6 years. The mean test duration was 3.4 ± 2.3 min, and the acceptability and comprehension of the test were good. Six patients had a musculoskeletal complaint, 19 had positive pGALS, and 14 were diagnosed with musculoskeletal disease. The internal consistency score (Cronbach's α) was 0.852. The sensitivity of the test was 92.8%, the specificity was 92.3%, the positive likelihood ratio was 2.16, and the negative likelihood ratio was 0.01. CONCLUSION: The pGALS test adapted to the Tunisian dialect is a relevant, quick, and valid tool for screening musculoskeletal abnormalities in school-age children.

Treatment with Rituximab in Rheumatoid Pneumoconiosis: A Case Report.

BACKGROUND: Caplan's syndrome, also known as rheumatoid pneumoconiosis (RP), is a rare disease associating pneumoconiosis with rheumatoid arthritis (RA). This is one of the rare cases evaluating the effect of Rituximab, which was used initially for the treatment of RA, on pneumoconiosis Case Presentation: In this case report, we described a 21-year long-standing history of pneumoconiosis and its association with RA. A 67-year-old man diagnosed with pneumoconiosis presented with morning stiffness and symmetrical polyarthritis. Laboratory investigations showed high titers of rheumatoid factor (RF) and anti-citrullinated protein antibodies. The diagnosis of RA was established and the patient was put on leflunomide. Then, he was treated with Rituximab, as he did not respond to leflunomide. The patient showed marked improvement as pain and swelling decreased. More importantly, Caplan's nodules stabilized on chest-computed tomography. CONCLUSION: The use of rituximab in pneumoconiosis does not alter the evolution of the pulmonary nodules. More trials are needed to establish a treatment consensus for RP.

The concept of telemedicine in pediatric rheumatology in Tunisia: Parents' perceptions.

INTRODUCTION: With the COVID-19 pandemic, health care systems are facing challenges in delivering proper patient care. Children and adolescents with juvenile idiopathic arthritis require specialized and comprehensive attention. In this context, telemedicine is an alternative that has the potential to improve access to healthcare in addition to cost savings. The objective of our study was to evaluate parents' willingness for telemedicine and factors helping to adopt this alternative in the era of COVID-19. METHODS: We undertook a cross-sectional study via structured phone interviews of parents' JIA patients as well as those with no established diagnoses. We evaluated their point of view and willingness to adhere to TM. RESULTS: The study included 40 parents. The main reasons for favoring TM were avoiding hospitals during the pandemic (32.5%), time saving (27.5%) as well as avoiding school absenteeism (27.5%). The main reasons for preferring a live consultation were the fear of a possible discrepancy between physical and distant evaluation (47.5%) and the fear of the trivialization of the disease (38.5%). There was no association between preference for TM and a family history of COVID-19 (p = 0.704) as well as electronic devices afforded (p = 0.263). However, patients who lived away from hospital, not familiar with the concept of TM and with higher income adhered less to TM. CONCLUSION: Unlike the literature data, our study showed the low prevalence of parents willing to accept TM as a model of care. This imply an urgent need for parent and patient education to promote TM especially in pediatric rheumatology.

Denosumab use in osteogenesis imperfecta: an update on therapeutic approaches

Osteogenesis imperfecta (OI) is an inherited skeletal disorder that leads to bone fragility and multiple fractures. Given advances in the genetic understanding of existing phenotypes and newly discovered mutations, therapeutic management of OI has become challenging. Denosumab, a monoclonal antibody that inhibits the interaction between the receptor activator of nuclear factor kappa B ligand (RANKL) and its receptor RANK, has been approved to treat postmenopausal osteoporosis and emerged as an important therapy for malignancies and other skeletal disorders, including pediatric skeletal conditions such as OI. This review summarizes information about denosumab therapy in OI by exploring its mechanisms of action, main indications, and safety and efficacy. Several case reports and small series have been published about the short-term use of denosumab in children with OI. Denosumab was considered a strong drug candidate for OI patients with bone fragility and a high risk of fracture, particularly for patients with the bisphosphonate (BP)-unresponsive OI-VI subtype. The evidence for denosumab's effects in children with OI indicates that it effectively improves bone mineral density but not fracture rates. A decrease in bone resorption markers was observed after each treatment. Safety was assessed by tracking the effects on calcium homeostasis and reporting side effects. No severe adverse effects were reported. Hypercalciuria and moderate hypercalcemia were reported, suggesting that BPs be used to prevent the bone rebound effect. In other words, denosumab can be used as a targeted intervention in children with OI. The posology and administration protocol require more investigation to achieve secure efficiency.

Sarcoidosis and spondyloarthritis: A coincidence or common etiopathogenesis?

BACKGROUND: Sarcoidosis is a multisystem granulomatous disease. Co-existence with spondyloarthritis (SA) has been more described as an adverse effect of anti-TNF α therapy than an association. We report herein a case of a typical sarcoidosis confirmed by histological proofs and an advanced SA with a bamboo column. CASE PRESENTATION: A 48-years-old woman presented with inflammatory back pain for 5 years and ankle swelling for 1 year. On physical examination, she had an exaggerated dorsal kyphosis and disappearance of lumbar lordosis with limitation in motion of the cervical and lumbar spine. Laboratory tests did not show an inflammatory syndrome or hypercalcemia. Plain radiographies of the spine and pelvic revealed a triple ray appearance with sacroiliitis grade 4. Chest radiography and CT confirmed the presence of bilateral hilar lymph nodes and parenchymal nodes. Bronchoscopy and biopsies were performed showing non-calcified granulomatous reaction without cell necrosis. The diagnosis of SA was performed based on 9 points of Amor criteria associated with pulmonary sarcoidosis. She was treated with 15 mg per week of methotrexate and 1mg/kg/day of prednisone for pulmonary disease with good outcomes. CONCLUSIONS: Sarcoidosis may be associated to SA besides paradoxical drug effect. The same physio pathological pathways mediate by TNF α are arguments for association than hazardous coincidence.