NEUROPSYCHIC POLYMORPHISM OF JUVENILE SYSTEMIC LUPUS ERYTHEMATOSUS

Damage to the nervous system (neurolupus) is one of the most common clinical manifestations of systemic lupus erythematosus (SLE) in childhood, and is also considered as an unfavorable prognostic criterion for the course of this disease. Neurolupus is characterized by a wide range of clinical manifestations in both children and adult patients, which is due in most cases to a common pathogenetic mechanism - the formation of systemic microvasculitis. The non-specificity and variability of neuropsychiatric symptoms, which may appear already at the onset of the disease, significantly complicate the early diagnosis of SLE and necessitate a close acquaintance of the pediatrician with neurolupus polymorphism in children.

Kidney Function, Age, and Education as Contributors to Depression and Anxiety in Juvenile Systemic Lupus Erythematosus

Juvenile systemic lupus erythematosus (JSLE) is diagnosed in children younger than 18 years of age. Depression and anxiety are common, but not well understood in JSLE. We investigated the clinical and psychological factors associated with the psychological manifestations of JSLE. Twenty-nine JSLE patients were recruited for the study. Patients completed surveys evaluating their psychological status and perceptions about their health. Medical records were used to obtain laboratory results. The JSLE patient population was compared with adult-onset SLE (ASLE) patients and unaffected controls. Kidney involvement was associated with depression in the JSLE patients. The BUN levels, BUN/creatinine ratio, and leukocyturia were all significantly associated with depressive symptoms. Multivariate analysis found that the BUN/creatinine ratio was the most predictive value for both depression and anxiety. Depressive symptoms in JSLE were less pronounced than in ASLE, although anxiety was not different. Age and education are likely to be protective against depression in the JSLE patients. These findings may indicate that symptomatology is an important indicator of whether the patient needs psychiatric care.

Serum KL-6 as predictive and prognostic marker of interstitial lung disease in childhood connective tissue diseases: a pilot study

This study was aimed to evaluate serum KL-6 levels to determine if this marker can be used for diagnosing and assessing severity of interstitial lung disease (ILD) in children with connective tissue disorders. In total, 40 patients [18 patients with juvenile systemic lupus erythematosus (JSLE), 10 patients with juvenile idiopathic arthritis (JIA), 8 patients with juvenile mixed connective tissue disease (JMCTD), 3 patients with juvenile systemic sclerosis (JSSc), and 1 patient with juvenile dermatomyositis (JDM)] and 20 healthy controls were included in this study. Age, sex, and duration of CTD and ILD (if any) were recorded. Blood samples from all the patients and controls were examined by ELISA. 20 of the 40 patients with CTD (50%) had ILD, 12 were mild and 8 were severe as assessed by spirometry. The median serum KL-6 level was 102.7 U/mL (76.1-180.8) in the CTD with severe ILD group, 72.2 U/mL (58.4- 100.5) in the CTD with mild ILD group, 56.7 U/mL (35.8-68.5) in the CTD without ILD group, and 52.3 U/mL (32.8-62.4) in the control group. KL-6 levels were significantly higher in the CTD with ILD (p<0.05), at a cutoff of 63.4 U/ml identified by ROC curve, serum KL-6 showed a sensitivity of 95.2% and specificity of 89.7%. KL-6 is a valuable biomarker for diagnostic purposes and to detect severity in ILD in childhood CTD.

P048 Juvenile systemic lupus erythematosus, what specificities?

Background Juvenile systemic lupus erythematosus (J-SLE) is a chronic autoimmune disease characterized by multi-visceral involvement with an unpredictable prognosis. The diagnosis is usually made in young women between the ages of 20 and 40, however, it can set in at any age and will be classified as juvenile (LESj) when it begins before the age of 16.We report the epidemiological, clinical, therapeutic and evolutionary characteristics of a retrospective series carried out at the level of the pediatric center—CHU de Sétif comprising 13 girls and one boy. Methods The mean age of onset is 12 years and 3 months, the mean time to diagnosis is 7 months. The clinical picture is made of e reached articulaire skin and e fever in 86% respectively 57% and 57% of cases, followed by kidney disease in 57% of cases. Cardiac involvement pulmon area ophtalmologiqu e is referred to in low percentages. The blood reached logic of étectée on blood counts in 85% of patient e s i and the syndrome nflammatoire was almost constant. A positive titer of antinuclear antibodies and anti- AD Nn is objectified, as well as a reduction in the level of complement. The anti-GP 2 and anti- cardio lopine antibodies are positive in 57% of cases. Has the present hue kidney in 42% of cases. A single case of overlap syndrome with dermatomyositis has been reported. As for the neurological form, only one adolescent presented it. With a single case of familial lupus and a single case of Rhupus. Results The diagnosis is based on the American College of Rheumatology (ACR) 1982 classification revised in 1997 and the new SLICC “Systemic Lupus International Collaborating Clinics” criteria. The clinical characteristics of our series are consistent with the overall data in the literature with a predominance of cutaneous and joint involvement. with however some specific characteristics which are individualized by a more advanced age of onset, of 13 years on average in our study vs 10 years and 12 years, the rarity of the familial forms (1 case), a lower percentage of renal damage (42% vs 63% and 80%). The therapeutic management was based on corticosteroids and Hydroxychloroquine in most cases, the use of immunosuppressants was reserved for x severe. Conclusion Lupus is an autoimmune disease with protean clinical manifestations, the prognosis of which is dominated by renal, neurological and thrombotic damage. Corticosteroid treatments and immunosuppressants markedly improved the vital prognosis.

P049 Comparison between the ACR 1997, SLICC 2012 and EULAR/ACR 2019 classification criteria in juvenile systemic lupus erythematosus

Background Juvenile systemic lupus erythematosus is a multisystem inflammatory autoimmune disease affecting patients under 16 years old. Diagnosis may be difficult because of the large clinical heterogeneity. Classification criteria are important to ensure a clear definition and consistent inclusion of patients in clinical trials. The objective of the work: is to Compare the performance of the classification criteria of the American College of Rheumatology (ACR 1997), those of the Systemic Lupus International Collaborating Clinics (SLICC 2012) and the new classification criteria together with the European League Against Rheumatism “EULAR” and the American College of Rheumatology “ACR” (EULAR/ACR 2019). Methods This is a cross-sectional retrospective study carried out in a tertiary care service over a period of one year, from January 1, 2020 to December 31, 2020. The patients were subjected to the different classification criteria ACR 1997, SLICC 2012 and EULAR/ACR 2019. All our patients were matched in age and sex with control cases followed for inflammatory disease (12 control cases). Results Six patients were diagnosed with juvenile systemic lupus erythematosus (JSL) over the period of our study. A clear predominance of women was noted with a sex ratio of 5 girls/1 boy. The mean age at the time of diagnosis was 9 years (extremes: 3 –12 years). The diagnosis was made after an average delay of 5 months (extremes: 1–12 months). All of our patients met the three classification criteria. Sensitivity to all criteria was noted at diagnosis and during the first months of follow-up with higher sensitivity for SLICC criteria. Regarding the control cases, 3 patients met the ACR 1997 criteria. Conclusion In this population of Juvenile systemic lupus erythematosus, the SLICC criteria gave the best results in terms of sensitivity and precision at the time of diagnosis and during the first months of follow-up, while the ACR 1997 criteria were less specific.

P044 Have we been missing cases of Juvenile Systemic Lupus Erythematosus in Nigeria? The experience of a paediatric rheumatology trainee in Lagos

Background Juvenile Systemic lupus erythematosus (JSLE) is a chronic multisystem autoimmune disease of childhood, which accounts for 10% to20% of systemic lupus erythematosus (SLE). It was initially thought that systemic lupus erythematosus (SLE), including JSLE, was rare in Blacks, this was eventually debunked with increasing reports from Africa. However, it is now known that SLE is more common among patients of African descent in western countries. While the estimated prevalence of JSLE in the developed countries is 0.36–2.5 per 100 000, data in Black Africans is scarce due to missed diagnosis, poor diagnostic capacity and under-reporting. JSLE has protean manifestations similar to common paediatric conditions such as severe malaria, overwhelming septicaemia, hyper-haemolytic crisis in sickle cell anaemia etc., which often cause delayed or missed diagnosis. The objective is to describe the demographic and clinical characteristics, including outcome of children with JSLE, thus raising awareness on their occurrence and management in Nigerian children. Methods Retrospective review of records of children diagnosed with SLE at the Adult/paediatric Rheumatology Clinic and Paediatric Wards of Lagos State University Teaching Hospital (LASUTH) from May 2018 to May 2021. Results Twenty-two children, nineteen (n = 19) girls and three (n = 3) boys, aged 5–17 years, fulfilled the American College of Rheumatology (ACR)’s diagnostic criteria for JSLE out of 45 children newly diagnosed with paediatric rheumatic diseases during this period. The duration of symptoms before diagnosis ranged from two weeks to three years. The presentations included recurrent severe anaemia (n = 16), arthritis (n = 17), arthralgia (n = 17), malar rash (n = 17), neurologic symptoms (n = 5) oral ulcers (n = 17), cardiopulmonary symptoms (n = 5), photosensitivity (n = 10) and renal disease (n = 14). Laboratory findings included elevated ESR with a mean (±SD) of 99.68 ± 44.44, positive ANA (n = 22), positive anti-dsDNA (n = 12), low C3 & C4 (n = 2), positive anti-Smith antibody (n = 8) and massive proteinuria (n = 14). All patients were treated with steroids and disease modifying anti-rheumatic drugs (synthetics and biologics) based on disease severity and organ manifestations. Sepsis (n = 4) was the most common preliminary diagnosis before a final diagnosis of JSLE was made, other preliminary diagnosis were pulmonary tuberculosis (n = 1), dermatitis (n = 1), acute glomerulonephritis (n = 1), Typhoid fever (n = 1), malaria (n = 1), deep vein thrombosis (n = 1), seizure disorder (n = 1), leukaemia (n = 1), meningitis (n = 1), meningoencephalitis (n = 1), hyper-haemolytic crisis in sickle cell anaemia (n = 1), Steven Johnson syndrome (n = 1), Juvenile Idiopathic Arthritis (n = 2), Eczema (n = 1), unexplained anaemia (n = 1) and acute rheumatic fever (n = 1). One boy and three girls defaulted from clinic after commencement of treatment due to severe financial constraints of their parents and religious beliefs, however six girls died, four from an acute flare and two from end stage renal disease. Conclusion Our study has shown that JSLE has protean manifestations with a tendency to miss its diagnosis due to similarity of signs and symptoms with common childhood diseases in our environment. JSLE may not be as rare as commonly thought, thus its prompt diagnosis and treatment require a high index of clinical suspicion.

Complete resolution of juvenile neuropsychiatric lupus with low dose rituximab

Nervous system involvement leads to high mortality in juvenile systemic lupus erythematosus. We present the case of a 12-year-old girl who was admitted with a history of recurrence of fever, cervical swellings, seizure, and delirium. Approximately 6 months back, she was started on antitubercular therapy (ATT) for fever and granulomatous lymphadenitis. She developed a recurrence of fever, new cervical swellings, seizures, and delirium while she was continuing ATT. Magnetic Resonance Imaging brain revealed multiple hyperintensities in bilateral basal ganglia, temporal, and hippocampal regions. The patient was diagnosed to have lupus based on clinical features, positive antinuclear antibody, positive double-stranded DNA antibody, and hypocomplementemia. To avoid cyclophosphamide-related gonadotoxicity in this young girl, Rituximab was given. She was treated with two doses of Rituximab and there was complete resolution of her neuropsychiatric SLE. Rituximab can be considered over cyclophosphamide in children to avoid gonadotoxicity.