bluuebunny/crossref_metadata_2025_split huggingface dataset:
30 Oligoarticular juvenile idiopathic arthritis-associated uveitis
Authors: Guedri Rahma 
Published: October 2022

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.
32 Methotrexate in oligoarticular juvenile idiopathic arthritis: adverse effects and outcome
Authors: Guedri R , Dghaies C , Hrizi H , Fraj H , Rebhi M , Essaddam L , Dahmouni M , Abdelaali A , Fitouri Z , Ben Becher S 
Published: October 2022

Abstract

Background
Methotrexate (MTX) is the anti-rheumatic drug of choice in juvenile idiopathic arthritis (JIA). Its adverse effects such as intolerance occur frequently, potentially hindering its efficacy

Objective
To describe the frequency of MTX intolerance and its various adverse effects (AE).

Results
A retrospective study was conducted on the AEs associated with MTX therapy in children diagnosed with oligoarticular juvenile idiopathic arthritis followed-up at the paediatric emergency and outpatient department of the children's hospital in Tunisia between January 2005 and December 2021. Among 95 cases, only 70 patients were included into the study, of whom 55 (78.5%) were girls. The median age at diagnosis was 5.9 years. Of these, 39/70 (55.7%) had required the use of MTX. The use of MTX was early (within the first 6 months after diagnosis) in 61.5% of cases. The initial median weekly dose was 11.4 mg/m². MTX intolerance was noted in 35.8% of cases. Management of intolerance included change in the dose, education and counselling. Adverse events led to MTX withdrawal in only one patient (2.5%).
The adverse effects noted during the follow-up were mainly transient increased plasma transaminases (17.9%), gastrointestinal symptoms (10.2%) and cytopenia (7.6%). Impairment of kidney function and behavioural problems were not noted in any of our patients.
The medium duration of the treatment was 3.8 years. The evolution was marked by the occurrence of a relapse of the disease after discontinuation of MTX in 14/33 cases (42.4%). The addition of biotherapy was necessary in 10/39 cases (25.6%). Only 7% of children have persistent joint stiffness or deformity.

Conclusion
In JIA, it is important to start effective treatment early to avoid long-term sequelae, such as joint damage. MTX adverse effects such as MTX intolerance occur frequently, potentially hindering its efficacy. To avoid inefficacy, the physician should be timely aware of these adverse events.
33 Oligoarticular juvenile idiopathic arthritis-associated uveitis
Authors: Dghaies C , Hrizi H , Guedri R , Rebhi M , Fraj H , Essaddam L , Dahmouni M , Abdelaali A , Fitouri Z , Ben Becher S 
Published: October 2022

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 associated with disabling morbidity. The uveitis seen in JIA is chronic anterior uveitis which is always asymptomatic in the initial stage.

Objective
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 95 consecutive pediatric patients with oligoarticular JIA in the paediatric emergency and outpatient department of the children's hospital in Tunisia between January 2005 and December 2021. 70 patients were included in this study. 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 had acceptable visual acuity. The presence of antinuclear antibody (ANA) was 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. Multidisciplinary management by the rheumatologist and the ophthalmologist is essential to optimize outcome.
36 Juvenile idiopathic arthritis-associated uveitis: a study of 69 cases
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 rheumatic disease association with uveitis in children. Uveitis is a significant cause of visual morbidity in children with JIA. Geographical variations in the incidence of uveitis in JIA have been reported around the world.

Objectives
The aim of this study was to determine the prevalence of JIA associated uveitis and its features 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 medications, JIA status at the time of enrolment and the latest follow-up.

Results
Sixty-nine cases of JIA were included. The female to male ratio was 1.8 :1. The median age at diagnosis was 9 years (range 1–16). At the latest follow-up, the median disease duration onset was 1 year (range 1–8 years). There were 34(49.3%) oligoarthritis, 9(13%) rheumatoid factor (RF) negative polyarticular arthritis, 8(11.6%) rheumatoid factor (RF) positive polyarticular arthritis, 6(8.7%) systemic onset JIA, 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. Sixty-three patients (91.3%) have benefited from the slit lamp examination. Uveitis was found in 5 patients (7.2%). three of these patients presented with oligoarticular JIA and two presented Polyarticular JIA. There was male predominance (4 boys and 1 girl). All these patients were ANA negative.

Conclusion
The observed phenotypic variabilities in JIA associated uveitis underscore the existence of true diversities in disease characteristics across geographical areas. Prospective multicentre studies are necessary to better identify the JIA associated uveitis peculiarities in our country.

Disclosure of Interest
None declared
52 Use of corticosteroids in childhood systemic lupus erythematosus: experience from a pediatric rheumatology center
Authors: Gacem Ourida , Abba Kahina , Mansouri Nawal , Ayad Nadia , Zeroual Zoulikha , Arrada Zakia , Achir Moussa , Ladj Mohamed Samir 
Published: October 2022

Abstract

Background
Childhood-onset Systemic Lupus Erythematosus (cSLE) is a rare but severe multisystem autoimmune/inflammatory disease that can affect any organ system and cause significant damage, disability and/or death. Corticosteroids are one of the important therapeutic weapons for childhood Systemic Lupus Erythematosus (SLE). Prednisone doses are increased during flare-ups of lupus disease, and then progressively reduced to reach a threshold dose in order to avoid the harmful effects of this treatment in growing children. The oral route is by far the most used over prolonged periods, however corticosteroid boluses are recommended in the treatment of severe acute attacks of the disease.

Objective
Assess the use of corticosteroids in childhood systemic lupus erythematosus (SLE) and the risk of steroid-related damage.

Methods
Patients with childhood -onset SLE (n = 83) treated at paediatric rheumatology centre were followed for 36 months. Information on medication use, disease activity as measured by SLEDAI was collected.

Results
Eighty-three (83) patients were studied. Female: male ratio was 1:49. Mean ages at lupus onset and diagnosis were respectively: 10, 12 (±3,88_ and 11, 3 (±3,62) years. Corticosteroids were used in 92% of cases in oral form and on 37% of cases as a bolus of high dose intravenous methylprednisolone. The indication of corticoids bolus was significantly related to severe forms (p = 0,001), high disease activity (p = 0, 00008), and macrophage activation Syndrome (p = 0, 0006). The doses varied between 0.5–1 g/1.73m2. The mean initial disease activity in these patients was 30 ± 10, while that found after taking the treatment was 12.9 ± 15. The decrease was statistically significant (p = 0, 000001). More than half of our patients were on high dose oral corticosteroids (2 mg/kg/d) and only 1/5 of the patients benefited from a dose of (1 mg/kg/day). The use of corticosteroids has been associated with significant side effects, including growth delay (22%), cushingoid obesity (58%), stretch marks (49%), osteoporosis (33%), femoral head osteonecrosis (1%), infections (54%). The association of certain side effects with a high dose of oral corticosteroids (2 mg/kg/day) was significant in this case cushingoid obesity (p = 0, 0003), stretch marks (p = 0.014).

Conclusion
Corticosteroids are the mainstay of therapy for prompt control of acute disease related inflammation in patients with SLE; they remain the first-line treatment for significant lupus manifestations, especially severe forms and life-threatening situations Higher dosage and longer duration of therapy, which are common in SLE, result in greater risk of adverse effects. Their use wisely is the guarantee of a better response and the reduction of deleterious effect.
53 Nakajo-Nishimura syndrome: the first African case
Authors: Ghodbane Nacif Eddine , Mecibah Ali , Haddouche Assia , Zerguine Halima , Akakba Zineb , Slimani Samy 
Published: October 2022

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.
70 Comparative study between PIMS and Kawasaki disease
Authors: Jalal M , Sakhi A , Ansari G. Benbrahim , Aboufaris H , Bouayed K , Ameayou S , Hassoun S , Nani S 
Published: October 2022

Abstract

Background
Kawasaki disease (kDa) is a multisystemic vasculitis affecting medium and small vessels with a predilection for the coronary arteries. It appears to be very similar to the pediatric multisystemic inflammatory syndrome (PIMS), which is a post-infectious inflammatory condition occurring after SARS-COV2 infection. Although these two entities have clinical and biological similarities, marked differences are identified. The aim of this study is to compare the two conditions in order to highlight the specific criteria of each of these related entities and to describe their demographic, clinical, biological and therapeutic characteristics in our context.

Method
Single center prospective observational study from January 2021 to March 2022 was conducted, comparing children admitted for PIMS on the basis of persistent fever, inflammatory syndrome, and positive COVID-19 IgG serology to patients hospitalized for kDa according to American Heart Association criteria.

Results
42 cases were recruited, among them 24 PIMS and 18 kDa, with a male predominance. The average age was 5 years for PIMS and 2 years and 7 months for kDa. All had a persistant fever with an average duration of 8 days as reason for consultation. Conjunctivitis was found in 88% of kDa vs 75% of PIMS and cheilitis in 94% of kDa vs 75% of PIMS. Skin rash, extremity involvement, cervical adenopathy were reported in both pathologies with respective percentages of 62.5%, 17.4% and 25% for PIMS vs 61.1%, 33.3% and 33.3% for kDa. Abdominal pain was reported in 54.2% of PIMS patients vs 5.6% of kDa. All patients had an inflammatory syndrome. The mean sedimentation rate was 67 mm at the first h in PIMS and 99 mm at the first h in kDa. The mean CRP was 147 mg/l in PIMS vs 132 mg/l in kDa. Lymphopenia was predominant in PIMS with 33% vs 11% in kDa. Cardiac enzymes were higher in PIMS with 29% of myocarditis and 15% of coronary dilatation vs 16.7% of coronary involvement in kDa. All PIMS patients received immunoglobulin infusion IV-IG, corticosteroid therapy, and an antiplatelet agent, whereas patients with kDa received IV-IG and acetyl salicylic acid in anti-inflammatory doses. Apyrexia was obtained at day 1 of treatment in the majority of patients, whereas 5.6% of kDa had persistent aneurysm.

Conclusion
PIMS cases predominated over kDa cases during this pandemic period. The distinction between the two entities could be difficult given the clinico-biological similarities. Abdominal pain was significantly more frequent in PIMS patients, whereas lymphopenia and myocarditis were not. The prognosis was better in PIMS.
71 Estimation of incidence of MIS-C in Cape Town, South Africa
Authors: Butters Claire , Abrahams Deepthi , Stander Raphaella , Spraklen Timothy , Facey-Thomas Heidi , Zühlke Liesl , Rabie Helena , Scott Christiaan , Webb Kate 
Published: October 2022

Abstract

Background
Multisystem inflammatory syndrome is a severe manifestation of SARS-CoV-2 in children. The incidence of MIS-C after SARS-CoV-2 infection is poorly understood. There are very few cohorts describing MIS-C in Africa despite MIS-C being more common in Black children worldwide.

Methods
A cohort of children with MIS-C and healthy children was recruited from May 2020 to May 2021 from the two main paediatric hospitals in Cape Town, South Africa. Clinical and demographic data were collected, and serum was tested for SARS-CoV-2 antibodies. The incidence of MIS-C was calculated using an estimation of population exposure from seroprevalence in the healthy group. Summary data, non-parametric comparisons and logistic regression analyses were performed.

Results
Sixty-eight children with MIS-C were recruited with a median age of 7 years and 97 healthy children were recruited with a 30% seroprevalence. The estimated incidence of MIS-C was 22/100 000 SARS-COV-2 infections in children under 14 years old in the city at that time. Black children were over-represented in the MIS-C group (62% vs 37%, p = 0.002). The most common clinical features in MIS-C were fever (100%), tachycardia (98.5%), rash (85.3%), conjunctivitis (77.9%), abdominal pain (60.3%) and hypotension (60.3%). Median levels of haemoglobin, sodium, CRP, ferritin, cardiac (pro-BNP, trop-T) and coagulation markers (D-dimer and fibrinogen), neutrophil and white cell count were markedly deranged in MIS-C. Cardiac, pulmonary, central nervous and renal organ systems were involved in 71%, 29.4%, 27.9% and 27.9% respectively. Ninety-four point one per cent patient received intravenous immune globulin, 64.7% received methylprednisolone and 61.7% received both. ICU admission was required in 39.7% patient while 38.2% required inotropic support, 38.2% required oxygen therapy, 11.8% required invasive ventilation and 6% required peritoneal dialysis. The median hospital stay duration was 7 days with no deaths.

Conclusion
The lack of reports from Southern Africa does not reflect a lack of cases of MIS-C. The clinical manifestations and outcomes of MIS-C in this region highlight the need for improved surveillance, reporting and data to inform diagnosis and treatment.

Implications
To our knowledge, these are the first data on MIS-C in Africa. This shows that children in Africa are indeed presenting with MIS-C which will increase surveillance around the continent.
80 Case series of juvenile scleroderma at Aga Khan University Hospital, Nairobi
Authors: Nzisa Irene , Mercy Chemutai , Migowa Angela 
Published: October 2022

Abstract

Background
Juvenile scleroderma includes a range of conditions presenting with skin fibrosis. It’s classified into two major categories, localized and systemic. Juvenile localized scleroderma (morphea) is limited to the skin and forms majority of pediatric presentations (&gt;95%). Though rare, juvenile systemic sclerosis involves the skin and other internal organs and is associated with a poorer prognosis.

Objective
To characterize the clinical profile of two cases of scleroderma at Aga Khan university hospital, Nairobi.

Methods
Retrospective review of medical records of two patients with Juvenile scleroderma managed at Aga Khan university hospital, Nairobi.

Results
The first patient was a 7-year-old boy presenting with joint pains for 2 months, restricted activities and limping. He also had a non-itchy lesion on the right thigh for about 6 years. On examination, there was an 8 by 18 cm, flat, shiny skin lesion with ill-defined margins and skin thickening around it. There were 2 punched out scars from previous skin biopsy sites with latest biopsy showing atrophic epidermis, dermal collagen homogenization with thick sclerosed fibers in keeping with morphea. Pulmonary function tests revealed mild restrictive lung disease. There were no other systems involved on further evaluation. He was started on weekly methotrexate, daily prednisone, daily folic acid and omeprazole to be followed up in clinic.
The second case is a 5-year-old girl who presented with 3-year history of inability to walk. Physical examination revealed areas of skin hypopigmentation and hyperpigmentation on the neck and back with active lesions on the left flank and posterior right leg. She had left lower limb atrophy and extreme wasting of thigh and calf muscles, a contracture of the left knee, ankle and limb length discrepancy. Her investigations revealed positive rheumatoid factor, positive antinuclear antibody (ANA), microcytic hypochromic anaemia and skin biopsy with features of morphea. She received six courses of high dose methyl prednisone over six months, weekly methotrexate, daily folic acid, daily prednisone that was tapered off gradually, calcium supplementation and iron supplementation. In addition, she received isoniazid for tuberculosis prophylaxis with pyridoxine. Her management was multidisciplinary involving orthopedics, physiotherapy, occupational therapy, and dieticians. She developed a new skin lesion on the left forearm about five months after initiation of treatment but no worsening of symptoms or other new lesions thereafter.

Conclusion
Juvenile scleroderma though rare has a high risk of misdiagnosis with delay in initiation of treatment. It is associated with significant morbidity and early diagnosis, treatment and follow up is imperative for good outcomes.