Rheumatology (Oxford, England)最新文献

{"title":"44 Pediatric onset systemic lupus erythematosus (p-SLE)","authors":"Sheren Maher","doi":"10.1093/rheumatology/keac496.040","DOIUrl":"https://doi.org/10.1093/rheumatology/keac496.040","url":null,"abstract":"Abstract Background Pediatric onset Systemic lupus erythematosus (p-SLE) is multisystem autoimmune disease with disease onset before the 18th birthday. It is a highly complex disease with marked heterogeneity between patients, causing anything from mild to life-threatening disease. Objective To assess thrombomodulin (TM) and D-dimer levels in pediatric systemic lupus erythematosus patients and their correlation to disease activity. Methods This study included 40 children diagnosed with systemic lupus erythematosus. They were grouped according to the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score into group (A) [Mild activity] included 20 children with SLEDAI score less than or equal to 4, group (B) [Moderate-high activity] included 20 children with SLEDAI score >4. Also, 40 apparently healthy children were included as a control group. They were age and sex matched, with the children having SLE. Results The two studied groups had significantly higher TM level than controls, furthermore group B had significantly higher TM level than group A (p< 0.008 in all). The two studied groups had significantly higher frequency of abnormally high TM level than controls, furthermore group B significantly had higher frequency of abnormally high TM level than group A (p< 0.02 in all). SLE children had significantly higher D dimer level than controls, furthermore group B had significantly higher D dimer level than group A (p≤ 0.006 in all). The two studied groups had significantly higher frequency of abnormally high D dimer level than controls, furthermore group B had significantly higher frequency of abnormally high D dimer level than group A (p≤ 0.03 in all). TM had a significant positive correlation with SLEDAI score as r = 0.342 and p = 0.031. D dimer had a significant positive correlation with SLEDAI score as r = 0.42 and p = 0.007. Conclusion Thrombomodulin and D dimer are potentially valuable markers of the disease activity and thrombotic tendency in p SLE patients. Thus, regular evaluation may be useful for early detection of thrombotic complications.","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":"156 12 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"125621405","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"76 Osteogenesis imperfecta: a case report on 4 cases","authors":"A. Ferhat, F. Benaziez, A. Rahmoune, N. Bahaz, C. Dahou-makhloufi","doi":"10.1093/rheumatology/keac496.072","DOIUrl":"https://doi.org/10.1093/rheumatology/keac496.072","url":null,"abstract":"Abstract Background Osteogenesis imperfecta, or “glass bone disease”, is a rare genetic disorder due to an abnormality in the production of type 1 collagen. It is characterised by bone fragility and low bone mass, leading to repeated fractures. Osteogenesis imperfecta (OI) can be classified into several types ranging from lethal at birth to benign forms that may be discovered incidentally in adulthood. We report four observations of OI that caught our attention. Observation 1: Mr H.A, aged 15 years, with no particular pathological history (atcd), he presented since the age of 4 years old iterative low-energy fractures (06 fractures). The clinical examination finds dorsal scoliosis, deformities of the long bones, blue staining of the sclerae. Radiography showed diffuse bone hypertransparency, thinned cortices and curvature of the long bones. Osteoporosis with a Z-score of -3.8 DS in the spine and -3.1 DS in the femur on bone densitometry (BMD). The patient received 20 courses of pamidronic acid at a dose of 07 mg/kg/year with good clinical (decrease in the number of fractures) and osteodensitometric improvement and good tolerance. Observation 2: Mr D.A, 16 years old, without any particular pathological atcd, the onset of the disorders goes back to the age of 13 years marked by the installation of repeated fractures following minimal trauma. The patient presents with growth retardation, deformities of the long bones and blue sclera. Radiography showed diffuse bone hypertransparency, thinned cortices, curvature of the long bones with bilateral coxa profonda. BMD showed osteoporosis with a Z score of -4.8DS. He was treated with pamidronic acid at 07 mg/kg/year (09 courses) with satisfactory improvement and good tolerance. Observation 3: Mr A.N aged 41 years with no particular pathological atcd. The history of the disease shows repeated low-energy fractures (06 fractures) since the age of 11 years. On clinical examination, there is an exaggerated kyphosis, blue sclera and bilateral hearing loss on the right. On imaging, diffuse bone hypertransparency, thinned cortices, curvature of the long bones and right acetabular protrusion were noted. On BMD, osteoporosis with a Z-score of -4.0 DS at the spine and femur. The patient received zoledronic acid 04 mg/06 months. The patient improved well with a decrease in the number of fractures and an improvement in bone densitometry. Observation 4: Mrs B.Z aged 40 years without any particular pathological atcd, having made iterative low energy fractures since the age of 01 month (05 fractures). The patient presents a dorsal scoliosis, deformations of the long bones, growth retardation, blue sclera and translucent teeth. The radiograph shows diffuse bone hypertransparency, thinned cortices and curvature of the long bones. BMD and adequate treatment were not done as the patient was lost to follow-up. Conclusion Osteogenesis imperfecta is a rare disease with a polymorphous clinical expression. Late diagnosis delays th","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":"1 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"125805028","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"87 Intracerebral inflammatory pseudotumor in Behçet’s disease: a pediatric case report","authors":"D. Hadef, S. Slimani, Mouna Nezzar, Issam Tahrour","doi":"10.1093/rheumatology/keac496.083","DOIUrl":"https://doi.org/10.1093/rheumatology/keac496.083","url":null,"abstract":"Abstract Introduction Behçet's disease is a chronic, relapsing, multisystem vasculitis. The diagnosis is essentially clinical, due to the absence of specific biological criteria, which remains very difficult to establish in pediatric age because of often insidious or atypical disease onset. The association of Pseudo Inflammatory Non-Specific Tumors (PTINS) and Behcet's disease (BD) is exceptionally reported. We report the case of a 12-year-old boy with Behçet's disease who presented with an inflammatory pseudotumor during the course of his disease. Methods and results A 12-year-old boy, from Batna (Algeria), with no relevant past medical history, admitted in 2010 for the management of a venous thrombosis of the right lower limb without any obvious cause and which responded well to anticoagulant treatment. A year later, the child was readmitted for polymorphic clinical signs made up of joint damage (inflammatory arthralgia of the large joints), digestive (glairo-bloody diarrhea) and ocular (decreased visual acuity) and skin and mucous membranes (oral-genital apthosis and erythema nodosum of the 4 limbs). Lab work-up showed significant inflammatory syndrome with erythrocyte sedimentation rate (ESR) of 100 mm at the first h, C-reactive protein (CRP) up to 400 mg/l and thrombocytosis (700 000–900 000 elements/mm2). HLA B51 was negative. The patient was treated with Colchicine, Steroids, and Aspirin with partial response. Subsequently, a treatment with azathioprine was started, with clinical improvement (despite occasional digestive crises) but with persistent biologic inflammation. In 2012 the patient presented with a unilateral decrease in visual acuity. A CT scan was performed showing an intracranial tumor compressing the right optic nerve with all the criteria of a malignant tumor (rupture of the cortex, increase in volume on 2 successive CT scans). A transsphenoidal biopsy of the mass revealed a non-specific inflammation. Biological treatment with anti-TNF alpha (Infliximab) was associated with a spectacular tumor regression, allowing retrospectively the diagnosis of an inflammatory granulomatous lesion related to Behçet's disease rather than a malignant tumor. The outcomes were favorable with clinical and biological improvement for the first time since the onset of the disease. Conclusion Behcet is rare in the pediatric age group and it is difficult to diagnose. Nonspecific inflammatory pseudotumors can be associated with Behçet's disease and constitute a real diagnostic and therapeutic challenge. Disclosure of Interest None declared","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":"106 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134414011","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"05 Methotrexate improve the foot functional impairment in juvenile idiopathic arthritis","authors":"Y. Makhlouf, H. Ferjani, L. Ben Ammar, D. B. Nessib, W. Triki, K. Maatallah, D. Kaffel, W. Hamdi","doi":"10.1093/rheumatology/keac496.001","DOIUrl":"https://doi.org/10.1093/rheumatology/keac496.001","url":null,"abstract":"Abstract Background Foot involvement in juvenile idiopathic arthritis (JIA) is common and affects about 60% of children [1]. Often overlooked by patients and practitioners, ankle and foot disability has been poorly studied and there are no accepted clinical practice guidelines for the diagnostic approach, as well as for the therapeutic management. Objectives Our study aimed to evaluate the effect of medical treatments on functional disability related to foot involvement in JIA. Methods We conducted a cross-sectional study including patients with JIA according to the revised ILAR criteria, collected from the rheumatology department of the Kassab National Institute. Sociodemographic and disease data (activity assessed by the Juvenile Arthritis Disease Activity Score 10 (JADAS-10) and therapeutic modalities) were recorded. Ankle and foot involvement were investigated by questioning and physical examination. We used the Oxford ankle foot questionnaire for children (OxAFQ-C); a validated, simple and reproducible score to assess the impact on the quality of life of children with foot problems [2]. This questionnaire encompasses 14 items corresponding to three dimensions: physical, school and play, and emotional. Higher scores represent better functioning. We searched for an association between the different drug treatments for JIA and the OxAFQ-C score. Results The study included 23 patients. The mean age was 13 ± 4 [6–18] years. The sex ratio was 0.42 with a female predominance. The age of the disease was 49 ± 40 months [6–180 months]. The distribution of the different forms of JIA was as follows: oligoarticular (n = 7), enthesitis-related arthritis (n = 6), polyarticular FR + (n = 1), polyarticular FR- (n = 3), psoriatic arthritis (n = 3), systemic (n = 1) and undifferentiated (n = 1). Pain on walking and limitation of the talocrural joint was found in 39% and 30% respectively. The mean JADAS-10 score was 6.72 ± 6.1 [0–20]. Ten patients had high activity. Eighteen patients (78%) were taking level I analgesics and 14 patients (61%) were on non-steroidal anti-inflammatory drugs, 12 of them on demand. Naproxen was the most commonly used drug, followed by diclofenac. Eleven patients (48%) were on a disease-modifying anti-rheumatic drug (csDMARD). Methotrexate (MTX) was prescribed in 30% of cases with a mean dose of 7.91 mg/week [7.5–10]. Only one patient was on sulfasalazine. Four patients (17%) were treated with biologics: Etanercept (n = 3) and Tocilizumab (n = 1). The mean scores of the different domains of (OxAFQ-C) were as follows: 73.52 ± 35.89 [0—100] in the physical domain, 84.2 ± 30 [6.25—100] in the school and play domain, 88.75 ± 24.71 [12.5 – 100] in the emotional domain. There was no statistically significant association between the different domains of the Oxford score and the use of analgesics or NSAIDs (p> 0.05). Similarly, there was no statistically significant association between the different domains of the Oxford score and treatment w","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":"2 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"117325778","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"53 Nakajo-Nishimura syndrome: the first African case","authors":"Nacif Eddine Ghodbane, Ali Mecibah, A. Haddouche, Halima Zerguine, Zineb Akakba, S. Slimani","doi":"10.1093/rheumatology/keac496.049","DOIUrl":"https://doi.org/10.1093/rheumatology/keac496.049","url":null,"abstract":"Abstract Nakajo-Nishimura syndrome is a hereditary autoinflammatory disorder caused by an autosomal recessive homozygous mutation of the PSMB8 gene, which encodes the immunoproteasome subunit beta 5i. The clinical manifestations of NNS are mainly pernio-like skin rashes, nodular erythema, lipodystrophy, clubbed fingers, remittent fever, hepatosplenomegaly, and basal ganglia calcifications. Here we are reporting a case of NNS in an 11-year-old girl, who lives in eastern Algeria, born from a first-degree consanguineous marriage, she presented with erythematous patches on her face and her back, nodular erythema on her neck, swollen and painful fingers with acrocyanosis and recurrent fever that mainly occurred in cold weather. The patient received long-term treatment with low-dose glucocorticoids, along with immunomodulatory drugs (hydroxychloroquine with methotrexate), partial improvement clinically and biologically was observed. Colchicine was added to her treatment, with increased prednisone doses when she recently developed an AA amyloidosis. Our patient was diagnosed clinically with NNS because she exhibited six of the eight characteristics. To the best of our knowledge, this is the first case of NNS in Africa.","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":"1 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"130729937","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"15 Fertility in juvenile idiopathic arthritis","authors":"Ahlem Ben Ammou, S. Rahmouni, S. Boussaid, H. Tbini, S. Jemmali, S. Rekik, K. Zouaoui, H. Sahli, Mohammed Elleuch","doi":"10.1093/rheumatology/keac496.011","DOIUrl":"https://doi.org/10.1093/rheumatology/keac496.011","url":null,"abstract":"Abstract Background Juvenile idiopathic arthritis (JIA) is a chronic rheumatic disease seen in children below 16 years of age. However, it may persist into adulthood. The damage caused by the disease can influence the sex life and fertility of adults with long-standing JIA. Objectives To study the fertility of patients with JIA. Methods We conducted a retrospective study including adults with long-standing JIA according to the International League of Associations for Rheumatology (ILAR) criteria. A detailed questionnaire was completed for each participant by interviewing them as well as by information obtained from their medical records. We identified among the adult patients, those who are married, their age at marriage, the number of gestation and parity, the age of the first child, miscarriages, and occurrence of menopause. Results A total of 29 Patients were enrolled, the M/F sex ratio was 0.71. 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 disease duration was 24.48 ± 12.76 [1–47] years. The mean BMI was 21.20 ± 4.89 [14.17–27.55] kg/m², 36.9% of patient had a BMI ≥ 25 kg/m². Fourteen patients had hip involvement. Seven patients (6 women, 1 man) were married (24%). The mean age at marriage was 23.75 ± 7.5 [20–35] years. The mean delay between disease onset and marriage was 10 ± 6.68 [6–20] years. Six patient had children, the mean number of gestation and parity were 2.56 ± 2.88 [0–6] and 2 ± 2.29 [0–6] respectively. Miscarriage was noted in 2 patients. Menopause was observed in 3 patients, the mean age at menopause was 46.67 ± 1.53 [45–48] years. Conclusion Most of our patients were not married. This reflects the significant impact of the disease on the social and sexual life of JIA patients at adulthood.","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":"36 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133411896","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"91 Systemic lupus erythematosus and infectious complications: a pediatric case report","authors":"D. Hadef, Badereddine Salhi, Meriem Haddadi, Khaoula Benbrahim, Rimah Belaala, Leila Bouderdaben, Norhane Bensacia, Ibtissam Kahlane, Abdarahmane Abdallaoui, Yacine Khenferi, N. Khernane","doi":"10.1093/rheumatology/keac496.087","DOIUrl":"https://doi.org/10.1093/rheumatology/keac496.087","url":null,"abstract":"Abstract Introduction Infection is one of the leading causes of morbidity and mortality in Systemic Lupus Erythematosus (SLE). Bacterial infections are the most frequent, followed by viral and fungal infections. Therapeutic factors as well as disease- and genetic related factors, all contribute to a lupus patient's increased susceptibility to infections. This report describes a case of a 12-year-old female who presented with features of systemic lupus erythematosus with inaugural infectious joint involvement. Methods and results An 11- year- old girl, from Biskra (Algeria), from a non-consanguineous marriage and the 7th of eight children. She had no relevant past medical history. She was referred to our department for the diagnostic and therapeutic management of a suspected systemic lupus erythematosus. The girl had a fever with a malar erythema and oral ulcerations. Arthralgia of the right hip was reported. Laboratory tests showed a positive CRP (C-reactive protein) and a positive Procalcitonin. The immunological assessment revealed Anti DNA, Anti Sm and Anti RNP positive. Ultrasound of the hip showed a very large arthro-synovial cyst of the anterior face of the right hip of 07 cm compressing the psoas muscle. The cyst was drained and antibiotic therapy initiated. Hydroxychloroquine was started while waiting for the rest of the exploration. Conclusion Joint involvement in systemic lupus erythematosus is frequent but an infectious complication is possible even at the disease onset and should not be missed. No conflicts of interest to declare.","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":"423 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"132147373","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"30 Oligoarticular juvenile idiopathic arthritis-associated uveitis","authors":"R. Guedri","doi":"10.1093/rheumatology/keac496.026","DOIUrl":"https://doi.org/10.1093/rheumatology/keac496.026","url":null,"abstract":"Abstract Background Oligoarticular juvenile idiopathic arthritis (JIA) is a rare inflammatory disease that occur in children under the age of 16. JIA associated uveitis is the most frequent extra-articular manifestation. The uveitis can be sight-threatening and may be burdened with disabling morbidity. The uveitis seen in JIA is chronic anterior uveitis which is always asymptomatic in the initial stages. Aim The purpose of our survey is to report the incidence of uveitis in oligoarticular JIA and to determine the potential risk factors for the occurrence of uveitis in children with oligoarticular JIA. Results We retrospectively evaluated 70 consecutive pediatric patients with oligoarticular JIA in the emergency and outpatient paediatric departement at the children's hospital in Tunisia between January 2005 and December 2021. A total of 12 cases (17.1%) with oligoarticular JIA associated uveitis where identified. The incidence of uveitis in these children was 0.7 cases per year. The uveitis had occurred before the joint manifestations in only one patient. The average time between the onset of symptoms and the occurrence of uveitis was 1.7 years with a maximum time interval of 5 years. Management of JIA-associated uveitis involved use of topical agents in half of our patients and systemic agents in the other half. JIA-associated uveitis has led to ocular complications such as cataracts (n = 5), glaucoma (n = 6), anterior/posterior synechiae (n = 8), and ultimately a visual impairment and blindness (n = 1). Eleven of the twelve patients with uveitis retained acceptable visual acuity. The presence of antinuclear antibody (ANA) has been identified as a risk factor for the occurrence of uveitis (p = 0.03). Conclusion Collateral damage of oligoarticular JIA include growth failure, muscle atrophy and intraocular damage. The main challenge during the management of oligoarticular JIA is the early detection of uveitis. Close cooperation between the rheumatologist and the ophthalmologist is essential to optimize outcome.","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":"32 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127420401","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"66 Optic neuritis in children","authors":"S. Melzi, R. Aboura, A. Ladjouze, N. Bouhafs, K. Boulesnane, R. Bouadjar, L. Mebrouki, Z. Beddek, N. Haddad, M. Tabti, Z. Bouzerar","doi":"10.1093/rheumatology/keac496.062","DOIUrl":"https://doi.org/10.1093/rheumatology/keac496.062","url":null,"abstract":"Abstract Introduction Optic neuritis (ON) are rare in children. They are due to inflammation of the optic nerve which results in a sudden drop in visual acuity, or amputation of the visual field. They are often secondary to an infectious disease or vaccination, but they can be part of auto-inflammatory, demyelinating or systemic diseases. The features of optic neuritis in children are different from adults as regards high rate of bilateral involvement, poor visual acuity, and papillitis. Aim Our aim is to describe the clinical and therapeutic outcomes of patients diagnosed with optic neuritis in the general pediatrics department at the CHU of Bab el Oued in Algiers. Methods It is a retrospective, descriptive study of patients who were treated for optic neuritis in our hospital over a period of three years (January 2018 to April 2021). Results There were 7 cases, 4 girls and 3 boys with F: M of 1.3:1, mean age of 10 years and age range of 6–13 years. The first manifestation was a decrease in visual acuity in all cases, 12 eyes affected, with bilateral involvement in 5 patients. Visual acuity decrease was severe < 3/10 in 80% of cases. The visual field was pathological in 2 cases (narrow, central scotoma). All patients had headaches. Retrobulbar involvement was confirmed by visual evoked potentials in all patients, they showed demyelinating involvement. Three patients presented papillitis. The diagnostic work-up included cerebral imaging which showed old nodular abnormalities of signal of the subcortical white matter in one patient. CSF analysis revealed anti-MOG antibodies in only one case. The infection investigations were negative in all cases except positive COVID 19 serology in one case The diagnosis retained in our patients were 2 cases of Behcet's disease, 1 monophasic neuritis, 1 optic neuritis with anti-MOG, 1 CRION, 1 case linked to COVID 19 in the absence of another plausible cause. In one case, no diagnosis was retained. Seventy percent of the patients benefited from treatment with corticosteroids pulse, only 2 did not receive treatment, the short-term evolution was favorable in 5 patients with a total recovery of visual acuity, one patient has evolved into atrophy of the left optic nerve and kept visual acuity low to 1/10 of the left eye. The medium-term evolution was marked by recurrence in 3 patients, 5 episodes in the 2 patients with Behcet's disease and 8 episodes in CRION which required the use of immunosuppressive treatment and biotherapy for Behcet's disease and plasmapheresis for CRION. Conclusion Despite the fact that our small series of patients is not representative, we found similar characteristics described in children in other studies. The average onset age was 10 years, with a female predominance, the severe decrease in visual acuity with frequent bilateral involvement. The diagnosis of ON is clinical and must be considered in any sudden drop in visual acuity. The diagnosis of the underlying pathology is often difficult ","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":"342 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"124311922","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"26 Kawasaki’s disease vs systemic juvenile idiopathic arthritis, a diagnostic dilemma","authors":"Monali Thakkar, A. Migowa","doi":"10.1093/rheumatology/keac496.022","DOIUrl":"https://doi.org/10.1093/rheumatology/keac496.022","url":null,"abstract":"Abstract Background Both these conditions are separate inflammatory conditions whose clinical presentation overlap and thus make their distinction difficult. Each has a criterion that allows diagnosis but since features overlap, diagnoses may be missed. Recent research also shows that both disease processes have similar underlying pathogenesis and involve interleukin 1; which have now raised the question of whether both these disease processes are distinct entities or conditions of a similar spectrum. Methods Medical records of from the inpatient admission at current and referring hospitals which included clinical history and examination, laboratory and radiological findings. Results 13-year-old boy referred from Isiolo county with complains of unremitting fevers for 9 days with associated hip pains. Was initially treated for malaria, and started on broad spectrum antibiotics and antivirals. On further examination, was noted to have cracked lips, strawberry tongue, non-suppurative conjuctivitis and an anterior chest wall wheal like rash. Examination also revealed a left sided non tender submandibular lymph node. Initial laboratory investigations showed neutrophilia of 12.89, thrombocytosis of 431, CRP of 364 and normal joint radiographs. An ECHO done showed left ventricular enlargement with corresponding mild Mitral regurgitation. Blood cultures showed no organisms. Autoimmune markers and tropical fever markers were normal as well. He was then placed on prednisolone challenge and reported to be fever free for the first time in 12 days. Discussion and conclusion A persistent fever unremitting with parenteral antipyretics can have a multitude of differentials. In a tropical nation the likelihood of an infectious cause is more common than other aetiologies. Hence, it should always be investigated for thoroughly. In the case highlighted above, malaria, TB, osteomyelitis, septic arthritis and other infectious causes were investigated. Childhood malignancy can also present with the same. But of note is autoimmune conditions need to be considered. With history of arthritic like pain, quotidian pattern of fevers, evanescent salmon coloured chest wall rash and cervical lymphadenopathy, a diagnosis of Systemic Juvenile Arthritis needs to be considered. Often this may be overlooked or misinterpreted as Kawasaki’s; which is a close differential; or Still’s disease. There is a criterion for SJIA by the International League of Associations for Rheumatology, however, there can be an incomplete presentation as well. This adds on to the diagnostic challenge and hence, SJIA may remain undiagnosed for a long time.","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":"21 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"114963466","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}