bluuebunny/crossref_metadata_2025_split huggingface dataset:
24 Scoliosis in children with juvenile idiopathic arthritis: any link?
Authors: Rania Ben Aissa , Hanene Ferjani , Mariam Moalla , Dorra Ben Nessib , Wafa Triki , Kaouther Maatallah , Dhia Kaffel , Wafa Hamdi 
Published: October 2022

Abstract

Background
Juvenile idiopathic arthritis (JIA) is an unusual aetiology of scoliosis in childhood. With few data in literature, the associated factors of scoliosis in JIA are still unknown.

Objectives
To describe the incidence and associated factors of scoliosis in children with JIA.

Methods
A cross-sectional study including children with JIA according to the International League of Associations for Rheumatology (ILAR). The recorded data included anthropometric measurements (weight, size, body mass index (BMI), and leg length), disease characteristics (JIA subtypes and duration), and static foot posture disorders. Regarding scoliosis, we collected clinical and radiographic findings.

Results
Thirty-five patients, forty-three percent of the patients were boys (n = 15). The mean age was 12.2 ± 3.61 years. The mean disease duration was 4.1 ± 3.29 years. The mean patient global assessment and the mean visual analogic scale were 3.4 ± 3.02 and 3.37 ± 2.92, respectively. The patient's distribution of JIA subtypes was oligoarticular (n = 13), enthesitis-related arthritis (n = 9), polyarticular (n = 4), undifferentiated (n = 4), psoriasis-related JIA (n = 4) and systemic-onset (n = 1). The mean CRP and ESR were 7.51 ± 11.85 mg/l and 18.88 ± 15.53 mm, respectively. The mean JADAS was 7.58 ± 6.3. Seventeen percent of the patients had an inactive disease (n = 6). Seven patients (20%) (6 females) had developed scoliosis during their disease, the spine regions of scoliosis were thoracolumbar scoliosis (n = 6) and thoracic scoliosis (n = 1). There was no specific distribution of scoliosis among JIA subtypes (p = 0.464). Most patients with scoliosis had associated leg length discrepancy (71.43% vs 28.57%, p = 0.025). Static foot posture disorders were present in most patients with scoliosis (85.71% vs 14.29%) but this difference was not significant (p = 0.673) compared with patients with no scoliosis. Weight, size and BMI were not associated with scoliosis (p = 0.955, p = 0.922, p = 0.303 respectively).

Conclusion
Our study shows that scoliosis is associated with leg length discrepancy. Indeed, the joint inflammation of the lower limb may explain the postural syndrome in children with JIA, with a prompt impact on the axial skeleton.
25 Impact of early feeding on the occurrence of juvenile idiopathic arthritis
Authors: Rabhi Emna , Triki Wafa , Ferjani Hanene , Nessib Dorra Ben , Maatallah Kaouther , Kaffel Dhia , Hamdi Wafa 
Published: October 2022

Abstract

Background
Juvenile idiopathic arthritis (JIA) is considered to be an autoimmune disease, which is a result of an immune reaction caused or triggered by environmental factors in a genetically susceptible individual. However, interactions between early feeding and risk Juvenile idiopathic arthritis (JIA) remains largely unexplored.

Objectives
To determine the impact of early alimentation on the occurrence of JIA in Tunisia.

Methods
We conducted a case-control study including 26 children with JIA fulfilling the international league against rheumatism (ILAR) 2010 criteria compared with a homogeneous control group of 21 children hospitalized in a paediatric orthopedic unit for traumatic reasons and healthy for any chronic inflammatory rheumatism. Parents of patients were asked after having their consent to participate. We collected the following data: Type of JIA, juvenile disease activity score (JADAS), type of feeding, total duration of breastfeeding, duration of exclusive breastfeeding and age at introduction of gluten. Patients were divided into two groups: group 1 (G1): JIA patients, group 2 (G2): control group. Data were analyzed using the SPSS statistical package. A p-value &lt; 0.05 was considered significant.

Results
Forty-seven children (26 diagnosed with JIA and 21 controls) with a mean age of 10.07 years ± 4.96 were enrolled. JIA subtypes were in decreasing order of frequency: oligoarticular (45.8%), enthesitis-related arthritis (20.8%), undifferentiated JIA (12.5%), seronegative polyarticular JIA (8.3%), juvenile psoriatic arthritis (8.3%), and seropositive polyarticular JIA (4.2%). The mean JADAS was 6.966 ± 5.38. There was no significant difference between the G1 and G2 with regard to the duration of breastfeeding (12.23 ± 3.8 months vs 8.14 ± 4.1 months, p = 0.09), exclusive breastfeeding (3.8 ± 3.6 vs 4.1 ± 2.62, p = 0.40) and age at introduction of gluten (G1: 8.48 ± 5.30 months vs G2: 7.48 ± 1,2 months, p = 0.35)

Conclusion
Our study didn’t show any impact of early alimentation on the occurrence of JIA in Tunisia. However, more studies are necessary to conclude these issues.
37 Subtype frequencies and demographic characteristics of juvenile idiopathic arthritis in Batna, Algeria
Authors: Hadef Djohra , Slimani Samy , Khamari Mohamed Choukri , Mekaoussi Walid , Belot Alexandre , Quartier Pierre 
Published: October 2022

Abstract

Background
Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood. There is a disparity in the prevalence of Juvenile idiopathic arthritis (JIA) subsets between different geographical areas or ethnic groups. In Arabic and African populations, data describing JIA are scarce. However, the epidemiological studies remain the best tool to understand the disease and to improve its management.

Objectives
To determine subtype, frequency, demographic and clinical features of JIA in Batna -Algeria- and to compare the findings with other JIA populations worldwide.

Methods
A multicentre retrospective descriptive study was conducted in Batna health centers (public and private sectors), over a seven-year period from January 2013 to December 2019, based on data collected on JIA patients. As public sector source, we referred to the department of pediatrics of the university hospital center (CHU Benflis Touhami Batna), and as private sector source, we referred to private adult rheumatologists based in Batna. The studied variables were: gender, age at the initial symptoms, age at diagnosis, JIA subtype based on International League of Associations for Rheumatology (ILAR) criteria, symptoms at onset, disease duration at the latest follow up, presence of uveitis, auto antibodies (antinuclear antibodies, Rheumatoid Factor and anti-CCP) pattern, joint imaging results, JIA status at the time of enrolment and the latest follow-up.

Results
The study included a total of 69 cases of JIA that were being followed in Batna health centers over the study period. The female to male ratio was 1.83. The median age at diagnosis was 9 years (range 1–16). Forty-six patients (72%) were diagnosed within the first year after disease onset. At the latest follow-up, the median disease duration onset was 1 year (range 1–8 years). There were 34 oligoarthritis (49.3%), 9(13%) rheumatoid factor (RF) negative polyarticular JIA, 8(11.6%) RF positive polyarticular JIA, 6(8.7%), systemic arthritis, 6(8.7%) enthesitis-related arthritis, 3(4.3%) psoriatic arthritis and 3(4.3%) undifferentiated arthritis. Nine patients (18.7%) were anti-nuclear antibody (ANA) positive, and 21 patients (30.4%) had indeterminate ANA status.

Conclusion
Oligoarthritis was the most common JIA subtype in our study. The RF positive polyarthritis frequency was higher than in literature. Prospective multicentre studies are necessary to better identify the JIA peculiarities in our country.

Disclosure of Interest
None declared
39 Pediatric monoarthritis of the elbow: atypical onset of a common disease
Authors: Ibrahim Rehab Ali 
Published: October 2022

Abstract

Background
Oligoarticular type of juvenile idiopathic arthritis (oligoJIA) is considered the most common type of JIA. It usually affects one or &lt; 4 joints. The knee is the most common affected joint followed by the ankle and wrist. Oligoarticular JIA presenting with erosive elbow monoarthritis as an initial sign of the disease is a rare condition which needs prompt diagnosis and management.

Objective
To recognize that isolated chronic elbow monoarthrits is an atypical presentation for oligoJIA.
Case presentation: A 14-year-old volleyball player female child presented with chronic recurrent left elbow swelling of six months’ duration. No history of morning stiffness, constitutional symptoms, other joint swelling, uveitis, or skin manifestations were reported. On examination there was mild cubitus valgus with elbow effusion associated with limited last degree elbow extension with no other signs of inflammation or infection (redness, tenderness, hotness). Hypermobility was noted in both elbows and knees. Patient was advised to rest the elbow and symptomatic treatment was recommended. On follow up there was mild improvement of the swelling, so further investigations were ordered. Erythrocyte Sedimentation Rate (ESR), C-reactive protein (CRP), Rheumatoid factor (RF) and antinuclear antibody (ANA) showed average normal values. X-ray of the elbow showed degenerative changes. Musculoskeletal ultrasound showed mild elbow effusion and magnetic resonance imaging (MRI) was ordered revealing effusion and early joint erosion at the ulnar articular surface with no synovial hypertrophy. Arthroscopy was recommended to detect the underlying pathology and histopathology revealed chronic active synovitis suggesting JIA. Non-steroidal anti-inflammatory drugs (NSAIDs) were recommended but one month later knee arthritis was noted, so methotrexate was started and the condition started to improve.

Results
We report a 14-year-old female child who presented with atypical isolated chronic swelling of the elbow and was diagnosed by arthroscopy and histopathology. Non-steroidal anti-inflammatory drugs (NSAIDS) were tried as a first line treatment followed by methotrexate.

Conclusion
Chronic elbow monoarthritis is an atypical onset of oligoJIA and its diagnosis is important to prevent irreversible joint damage. Synovial biopsy can have a major role in the diagnosis of atypical monoarticular JIA and can help in early diagnosis and management improving the outcome.
The implication to policy, practice, research, and advocacy: Monoarthritis of the elbow as initial sign of oligoJIA is an atypical onset of this disease and in the absence of systemic signs or other clinical indicators determining the exact cause of monoarthritis in children might be difficult. Synovial biopsy can have an important role in the diagnosis of monarticular arthritis in pediatric patients.
44 Pediatric onset systemic lupus erythematosus (p-SLE)
Authors: Maher Sheren 
Published: October 2022

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 &gt;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&lt; 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&lt; 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.
45 Juvenile systemic lupus erythematosus: about a series of 35 cases
Authors: El Qadiry R , Nassih H , Bourrahouat A , Ait Sab I 
Published: October 2022

Abstract

Background
Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease that can involve any organ system with a wide range of disease manifestations, and can lead to significant morbidity and even mortality.

Objectives
To describe the epidemiology, common clinical features, complications, and management of SLE in children.

Methods
A single center retrospective descriptive study of 35 children with SLE.

Results
There were 27 (77%) girls and 8 (22.9%) boys. The mean age at diagnosis was 9 ± 2 years, with extremes of 3 and 15 years. Systemic involvement was in the form of protracted fever in 62.8%, nephrotic syndrome in 37%, nephritic syndrome in 40%, thrombotic microangiopathy in 8.6%, isolated proteinuria in 25.7%, acute kidney injury in 25.7%, malar rash in 48.6%, arthritis in 57%, neurolupus in 17%, serositis in 11.4%, and hemolytic anaemia in 28.6%. Coexisting autoimmune conditions were systemic sclerosis, antiphospholipid antibodies syndrome and Gujerat-Sjogren syndrome. Two cases had severe pancreatitis as a revealing pattern. Posterior uveitis was present in one case. Meanwhile, pulmonary involvement was seen in two cases (interstitial lung disease and recurrent pneumonia). Proliferative lupus nephritis was found in kidney biopsy in 48.6%. Four cases had end stage kidney disease and had hemodialysis sessions. Management consisted of hydroxychloroquine at 5 mg/kg/day and oral steroids at a starting dose of 2 mg/kg/day in all cases, while pulses of methylprednisolone were prescribed in 57%. Other immunomodulatory treatments used were IV cyclophosphamide in 34.4%, mycophenolate mofetil in 51.4%, azathioprine in 8.6%, tacrolimus in 5.7%, rituximab in 11.4%, and methotrexate in 2.8%. Evolution was marked by stabilization in 42.8% and recurrent flares in 28.6%. Meanwhile six cases were lost to follow-up and four cases died. The reasons for mortality were severe disease activity (seen in two cases), infection and macrophage activation syndrome (in one case each).

Conclusion
Management of juvenile SLE can be challenging especially when there is severe systemic involvement. Disease damage, side effects of drugs and frequent hospitalization have major effect on the quality of life of pediatric patients.
49 Autoimmune hemolytic anemia revealing a familial systemic lupus erythematosus
Authors: Hatem Rouag , Seif Zayani , Alaa Akid , Emna Sfar , Tesnime Ghedira , Radhia Hadj Salem , Chokri Chouchane , Farah Thabet , Slah Chouchane 
Published: October 2022

Abstract

Background
Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease; whose pathogenesis remains poorly understood. The genetic susceptibility to lupus is supported by the high concordance rate of the disease in monozygotic twins (29–57%) compared with dizygotic twins (2%), and by the existence of familial lupus (10–12%). Furthermore, SLE shares genetic and environmental risk factors, as well as pathophysiologic mechanisms with autoimmune hemolytic anaemia (AIHA), which is clearly over-represented in patients with SLE and often occurs before the diagnosis of the underlying disease.

Objective
We report a case of familial SLE in two siblings, in whom, the main symptom was AIHA.

Case report
A twelve-year-old girl was referred to our Pediatric department for polyarthralgia, asthenia and anorexia developing over the previous 4–6 months. Regarding family history, she is born of a non-consanguineous marriage and has a 7-year-old brother who has been followed for 2 years for AIHA of undetermined aetiology. The examination upon admission objectified a splenomegaly and jaundice. The laboratory workup revealed an autoimmune hemolytic anaemia: haemoglobin: 7.2 g/dl, positive Coombs' test with specific anti globulin IgG and C3d; unconjugated bilirubin (45 µmol/l) and reduced haptoglobin (0.01 g/l). These findings being very evocative of SLE, an antinuclear antibody (AAN) assay was positive at 1/400. The formal SLE diagnosis was established according to the EULAR/ACR 2019 criteria; with a score of 12 (AIHA: 4; positive antiphospholipid antibodies: 2; positive anti-Sm antibodies: 6). The therapeutic management based on corticosteroids at a dose of 1 mg/kg/day, with a favorable clinical and biological outcome. In light of this diagnosis, the case of the aforementioned brother was reviewed, and a familial lupus was suspected. A physical examination and a several biological explorations were done, the results of which allowing to retain the diagnosis of SLE with EULAR/ACR 2019 score of 10 (ANA &gt; 1/80, AIHA : 4, positive anti-phospholipid antibodies: 2, proteinuria: 4)

Conclusion
Since the new criteria EULAR/ACR 2019 have made it possible to diagnose incomplete forms of lupus, the challenge remains to detect autoimmune thyroiditis, antiphospholipid syndrome, complement deficiencies and other entities that are associated with familial lupus according to scientific literature.
56 Prevalence and severity of infections in patients with systemic autoimmune diseases on biotherapy
Authors: Hiba Ben Ayed , Sarra Ben Yacoub , Manel Boudokhane , Rym Bourguiba , Zeineb Teyeb , Mariem Ayari , Taieb Jomni , Imen Abdelaali , Syrine Belakhal , Hedi Dougui Mohamed 
Published: October 2022

Abstract

Introduction
The prognosis of chronic inflammatory diseases has been transformed by the use of biologic drugs. However, there is evidence of an increased risk of infection in patients undergoing biotherapy. The objective of our study was to investigate the frequency and the severity of infections in patients on biotherapy.

Methods
We conducted a retrospective descriptive study including all patients followed in the internal medicine department of the ISF hospital and treated with biotherapy over a period of 14 years [2009–2022].

Results
We identified 50 patients treated with biologic therapy among 76 patients. The patient’s mean age was 48 years± 12.5 [23–76] and male to female ratio (M: F) was 1 :6. The mean age at initiation of biotherapy was 41.3 ± 11.6 [17–68] years. The molecules used were TNFα inhibitors in 82% of cases, rituximab in 14% of cases and tocilizumab in 4%. Infectious complications were identified in 20 patients. Infections occurred after a mean duration of 12 months [0.5–36] after the initiation of biotherapy. They affected the skin in nine cases, the bronchopulmonary tract in seven cases, the central nervous system in four cases, the gastrointestinal tract in three cases, and the upper respiratory tract in two cases. The infection was bacterial in 16 cases (64%), viral in five cases (20%) and fungal in four cases (16%). We report five cases of tuberculosis (TB), including four cases of pulmonary TB and one case of neuro-meningeal TB. The infection was severe, requiring hospitalization in only eight cases (32%). The median duration of hospitalization was 12 days [2–58]. The infectious episode led to temporary interruption of biotherapy in six cases and definitive interruption in two cases. No deaths were noted.

Conclusion
Our study confirms the increased susceptibility to infections in patients treated with biologics. These infections, often bacterial, are in the majority of cases benign. However, tuberculosis remains frequent.
57 Ocular tuberculosis: a differential for chronic panuveitis
Authors: Thakkar Monali , Migowa Angela 
Published: October 2022

Abstract

Background
Mycobacterium tuberculosis is an organism responsible for causing chronic granulomatous inflammation leading to active or latent tuberculosis (TB). Both pulmonary and extrapulmonary infections; including the skin, the eye, the cardiovascular, genitourinary and gastrointestinal systems can occur. Within the eye, it can cause global disease ranging from anterior or posterior uveitis to retinal TB. Within this abstract we demonstrate a case of chronic panuveitis with a diagnosis of ocular TB.

Methodology
Retrospective chart review

Results
We reviewed a 9-year-old- male in the rheumatology outpatient clinic who was on treatment for ocular TB with methotrexate 2.5 mg once a week, prednisone 5 mg once a day, maxitrol eye drops once a day and prednisolone eye drops twice a day.
He presented with a red, painless left eye with deteriorating vision 2 years ago. Functional enquiry was unremarkable. He was noted to have a fixed dilated left pupil at 3-4mm but other examinations were normal. Extensive investigations were carried out, including an Interferon-gamma release assay (IGRA)- which was positive. Toxoplasma antibody test, Treponema pallidum Haemagglutination test, and chest X-ray were all negative. He was then started on the above-mentioned therapies with marked improvement.
A decision on increasing the methotrexate dose if steroid tapering showed no improvement was undertaken and a plan on reassessing the patient with ANA, ACE and HLA B51 was made, to ensure complete workup.

Discussion and conclusion
Intra or extra ocular TB (OTB) manifestations may result as a direct inoculation of the organism in ocular fluid or as a systemic immune response initiated by an infection from a distant site. There is a myriad of ocular manifestations of OTB which make forming a clinical diagnostic criterion difficult. The gold standard for diagnosis is an ocular fluid culture showing organisms but the yield may be low and the risk of damage to surrounding structures may be high. PCR tests done on the sample may yield clinical information but non ocular tests, such as the IGRA and tuberculin tests may also be employed. Therapy depends on active vs latent disease and mainly involves anti-inflammatory agents and anti-TBs.
Ocular TB may lead to sudden and irreversible blindness with high socioeconomic implications and hence, an index of suspicion in areas with a high disease burden should be kept as diagnosis poses a challenge.