Antonella Petaccia

Chorea, a little-known manifestation in systemic lupus erythematosus: short literature review and four case reports

Chorea is a movement disorder that may be found in children due to several causes. Here we focus especially on Systemic Lupus Erythematosus associated chorea. First we outline its epidemiology, hypothesized pathogenesis, clinical presentation and treatment, then we report four significant clinical cases, which represent well the extreme variability of set of symptoms that may accompany lupus chorea. Our experience, according to literature, suggests that choreic movements in a child should alert the pediatrician and lead him to investigate a potential neurological involvement of Systemic Lupus Erythematosus.

Wegener's granulomatosis presenting with life-threatening lung hemorrhage in a 7-year-old child

Wegener’s granulomatosis (WG) is an idiopathic systemic disease that usually onsets in adolescence and is rare in young children. Its diagnosis is usually based on the presence of fever with arthralgia and weight loss, associated with symptoms of upper and/or lower respiratory tract involvement and renal disorders. We describe the appearance of a life-threatening lung hemorrhage in the absence of hemoptysis in a 7-year-old girl with a completely negative previous clinical history, who was subsequently diagnosed as having WG. The teaching message is that immediate bronchoscopy with bronchoalveolar lavage seems to be advisable in the presence of severe respiratory distress and bilateral lung as well as renal involvement. When a diffuse alveolar hemorrhage syndrome is demonstrated, WG should be considered among the main etiologies even in a relatively young child without a clinically suggestive history.

Neuropsychiatric manifestations in juvenile systemic lupus erythematosus: what’s the weight of headache?

Juvenile Systemic Lupus Erythematosus (JSLE) is an autoimmune disease characterized by multiple organ involvement. The nervous system is often affected, with a higher frequency in children than in adults.

Paediatric acute rheumatic fever in developed countries: Neglected or negligible disease? Results from an observational study in Lombardy (Italy)

Introduction Acute Rheumatic Fever (ARF) is a multisystemic disease that results from an autoimmune reaction due to group A streptococcal infection. The disease affects predominantly children aged 5 to 15 years and although its incidence in developed Countries declined since the early 1900s, to date there is a paucity of data that confirm this epidemiological trend. Objective The study aimed to assess the burden of ARF in term of hospitalization and to describe the characteristics of acute rheumatic fever (ARF) in the paediatric population of Lombardy. Study design The study was carried out by analyzing hospital discharge records of patients resident of Lombardy and aged 0–17 years old who, from 2014 to 2016, were hospitalized with the diagnosis of ARF. The following variables have been studied: age, sex, municipality of residence, date of diagnosis of each patient, hospital of admission, and presentation of the disease. Results From 2014 to 2016, 215 patients were found to meet the inclusion criteria and diagnosed as affected from Acute Rheumatic Fever. The rate of hospitalization showed a slightly increasing trend from 3.42 in 2014 to about 5.0 in 2016. Moreover, ARF presented a typical seasonal trend with lower cases in the autumn and a peak of hospitalization in the spring. Conclusion To date, ARF seems to be a rare but not negligible disease in southern central European countries, and in Lombardy we estimated an annual hospitalization rate of 4.24 cases per 100,000 children. The increasing trend found in our study suggests that the burden of the disease could be reduced by involving multidisciplinary health professionals who, in addition to the paediatrician of free choice, would promote evidence based medicine management of the disease during all its clinical phases.

Longterm Safety and Efficacy of Adalimumab and Infliximab for Uveitis Associated with Juvenile Idiopathic Arthritis

Objective. Anti-TNF-α agents have significantly changed the management of juvenile idiopathic arthritis (JIA). We evaluated the safety and efficacy of adalimumab (ADA) and infliximab (IFX) for the treatment of JIA-associated uveitis in patients treated for ≥ 2 years. Methods. Patients with JIA-associated uveitis treated with IFX and ADA were managed by a standardized protocol and data were entered in the ORCHIDEA registry. At baseline, all patients were refractory to standard immunosuppressive treatment or were corticosteroid-dependent. Data recorded every 3 months were uveitis course, number/type of ocular flares and complications, drug-related adverse events (AE), and treatment switch or withdrawal. Data of patients treated for ≥ 2 years were analyzed by descriptive statistics. Results. Up to December 2014, 154 patients with ≥ 24 months followup were included in the study. Fifty-nine patients were treated with IFX and 95 with ADA. Clinical remission, defined as the absence of flares for > 6 months on treatment, was achieved in 69 patients (44.8%), with a better remission rate for ADA (60.0%) as compared to IFX (20.3%; p < 0.001). A significant reduction of flares was observed in all patients without difference between the 2 treatment modalities. The number of new ocular complications decreased in both groups but was lower for ADA (p = 0.015). No serious AE were recorded; 16.4% of patients experienced 35 minor AE and the incidence rate was lower with ADA than with IFX. Conclusion. At the 2-year followup, ADA showed a better efficacy and safety profile than IFX for the treatment of refractory JIA-associated uveitis.

May biscuits contribute to iron balance? An observation in children with juvenile idiopatic arthritis.

Abstract Within an observational open study on the effects of a scheduled dosage of biscuits with iron, children with juvenile idiopathic arthritis were either supplemented with biscuits supplying iron fumarate (median 3.6 mg per day) or left to their customary dietary habits. After 4 months, supplemented children showed a more favourable percentage change of blood haemoglobin, while ferritin levels (markers of inflammation) remained stable. We conclude that the supply of iron with available dietary products may contribute to an adequate iron status in children with chronic inflammatory disorders in a stable situation.

Does ultrasound agree with parent's perception of joint disease in juvenile idiopathic arthritis?

Ultrasound (US) is a powerful tool for the assessment of joint disease in children with juvenile idiopathic arthritis (JIA) and has been shown to be more accurate than clinical examination in detecting synovitis. Parent’s proxy-report of joint involvement is potentially usefulto obtain information on parent’s perception of the burden of child’s arthritis and may serve as surrogate for physician’s articular examination. However, it is unclear whether parents are reliable reporters of their children’s disease.

THU0306 Predictors of outcome in a cohort of italian children/adolescents with primary raynaud’s phenomenon: A multicenter study

Background Raynaud’s phenomenon (RP) is a benign reversible vasospasm of the extremities that may occur in the absence of any underlying connective tissue disease (CTD), mostly systemic lupus erythematosus, juvenile dermatomyositis and systemic scleroderma. Predictors of a favorable outcome are still unraveled in primary RP (pRP): the causative role of various autoantibodies remain to be elucidated mostly for pRP starting in childhood or adolescence. Objectives To identify the possible predictors of outcome in a cohort of children and adolescents with pRP. Methods We performed a prospective data collection of demographic, clinical, laboratory and treatment characteristics of 76 Italian children/adolescents with pRP (54 females, 22 males, median age at disease onset: 13.3 years, median age at diagnosis: 14.7 years), managed in 4 pediatric rheumatologic centres and 1 transition clinic during the last three years. Demographic characteristics included sex, age and ethnicity. The evaluation included clinical pictures, eventual disease associations, pubertal status, laboratory data and nailfold videocapillaroscopy (NVC) at baseline and at regular 6-month-follow-up. Laboratory examinations included erythrosedimentation rate, C-reactive protein, transaminases, creatinine, hemoglobin, complement fractions C4 and C3, renal and thyroid function and specific serum autoantibodies (anti-nuclear antibodies [ANA], anti-DNAds, anti-ENA, anti-cardiolipin, anti-Scl-70 and anti-centromere antibodies). Screening for coeliac disease was performed at the first evaluation. Treatment details included the eventual specific drug used, its dosage and overall treatment duration. Out of 76, 14 patients were treated with hydroxicloroquine, 10 with calcium blockers, 1 with low-dose aspirin, 3 with iloprost, while the remaining 48 did not receive any drug.A forward stepwise multiple logistic regression analysis was used to find any association among sex, pubertal status, inflammatory parameters, NVC abnormalities, all serum autoantibodies and the risk of developing a CTD at baseline and at 36-month-follow-up. The software used was STATA 10. A p-value <0.05 was considered significant Results ANA positivity at baseline was significantly associated with the risk of developing a CTD (p<0.05). No NVC abnormalities was related to specific patients’ outcome. No patient resulted positive at the screening for coeliac disease. Conclusions Our data show that only ANA positivity is the potential predictor of poor outcome and progression to CTD in children and adolescents with pRP. References Nigrovic PA, Fuhlbrigge RC, Sundel RP. Raynaud’s phenomenon in children: a retrospective review of 123 patients. Pediatrics 2003;111:715-21. Disclosure of Interest None Declared