Paediatric Rheumatology European Society Research Articles

Association between IL2RA and juvenile idiopathic arthritis (JIA) disease severity at first presentation to paediatric rheumatology: results from the Childhood Arthritis Prospective Study (CAPS)

Results 185 children with JIA (median age 7.2 years(IQR 3.6, 11.7), 65% female) were included. Median CHAQ score at presentation was 0.75(IQR 0.13, 1.38). There was a trend towards higher disability with increased number of copies of the rare allele of rs2104286 (Table 1) (OR 8.00 (0.93, 68.79), p = 0.06). An association was also seen between increased disability and homozygosity for the rare allele of rs11594656 (OR 3.37 (0.89–12.75), p = 0.07). There was no association with rs41295061. Conclusion Children homozygous for the rare allele (rs2104286) a SNP associated with JIA susceptibility, showed a trend towards increased disability. Interestingly, a second SNP in the IL2RA region not previously associated with JIA susceptibility also showed a similar trend. Validation of these results in larger cohorts is required. from 15th Paediatric Rheumatology European Society (PreS) Congress London, UK. 14–17 September 2008

Sleep, fatigue and quality of life in juvenile idiopathic arthritis (JIA) and Juvenile Dermatomyositis (JDM)

Fatigue was related to prednisone and methotrexate use as reported by patients and parents. Conclusion Sleep disturbance and fatigue are prevalent among children with different rheumatic diseases. Sleep disturbance and fatigue are strongly related to increases in pain and decreases in quality of life. Strategies aimed at improving sleep should be studied as possible ways of improving quality of life for children with rheumatic illness. from 15th Paediatric Rheumatology European Society (PreS) Congress London, UK. 14–17 September 2008

The use of Etanercept and Adalimumab in the management of JIA: a 5-year follow-up study

Results Safety: Common respiratory tract infections were recorded in 28% of pts (10/34 under ET and 3/14 under AD). Serious infections were recorded in 4.7% (1 ET, 1 AD). No other serious adverse effects were recorded. Efficacy: ACRped 50–70. 1 st yr: 88% of the ET and 68% of the AD group. 2 nd yr: 81% of the ET and 66.7% of the AD group. 3 yr: 83% of the ET and 100% of the AD group. During the 5-yr period, 11/46 pts (28%) switched from ET to AD or vice versa. Of all patients, 32.5% discontinued anti-TNF treatment due to remission and 52.2% had a satisfactory response (ACRped 50–70), while 8.7% had a poor response either to ET or AD. Conclusion Most of the patients with refractory to conventional treatment JIA respond satisfactorily to the long-term administration of anti-TNFs. The first 2 years are critical to predict a good and sustained response. Although serious infections are rare, a systematic vigilance is warranted in order to avoid fatal outcomes. from 15th Paediatric Rheumatology European Society (PreS) Congress London, UK. 14–17 September 2008

Gastroparesis associated with Juvenile Dermatomyositis

Gastroparesis associated with Juvenile Dermatomyositis

Adverse events during anti-TNF therapy in 269 patients with juvenile idiopathic arthritis

Results The total drug exposure was 665 years; etanercept exposure was 354 years, infliximab 287, and adalimumab 24. Of altogether 1057 AEs, 49 (5%) were considered as serious (Table 1). A total of 509 (49%) infections occurred, of which 291 (57%) were upper respiratory tract infections. Dermatological problems were documented in 57 (21%) patients, and hypersensitivity reactions (consisting infusion and injection site reactions) in 52 (19%). Neither malignancies nor tuberculosis appeared, although one patient had a mycobacterium avium pneumonia during adalimumab therapy. Conclusion Infections were the most common AEs in patients with JIA receiving anti-TNF therapy, but the rate of serious infections seemed to be low. from 15th Paediatric Rheumatology European Society (PreS) Congress London, UK. 14–17 September 2008

Inflammatory arthritis associated with inflammatory bowel disease in children

Results Nine children aged from 2.8 to 14.9 years presented with a Crohn's disease associated with arthritis. Rheumatic disease occurred 25 to 41 months before the diagnosis of IBD (3 children), 8 to 62 months after the diagnosis of IBD (4 children) or at the same time of IBD (2 children). In the first three children, polyarticular form of juvenile idiopathic arthritis and Still's disease were initially diagnosed. IBD was diagnosed 2 to 7 months after the initiation of etanercept (2 children) and anakinra (1 child). Arthritis developed in children who received corticosteroids, infliximab, methotrexate and/or azathioprine because of IBD. Patients presented with peripheral arthritis, involving knees (7 children), ankles (5 children), and rarely hips, fingers, elbows and shoulders. 12/18 relapse of arthritis occurred at the same time as relapses of IBD. Abdominal manifestations included diarrhea, anal abscess, abdominal pain, vomiting, and loss of weight. Mean C reactive protein value and erythrocyte sedimentation rate were102 mg/L and 58 mm at onset of the disease respectively. Discussion IBD may be diagnosed several years after the occurrence of arthritis and must be searched for in children presenting with arthritis and abdominal involvement and/or weight loss. IBD may develop in children who receive etanercept while infliximab does not prevent the occurrence of inflammatory arthritis. from 15th Paediatric Rheumatology European Society (PreS) Congress London, UK. 14–17 September 2008

Hemorrhagic bullous henoch schonlein purpura: a diagnostic challenge for paediatricians

Patients A 9-year-old girl and 11-year-old boy were admitted to our hospital with abdominal pain, arthralgia, ecchymoses and petechiae at legs, arms and buttocks. Shortly, large, tense hemorrhagic bullae and vesicles (2 to 30 mm) developed over the purpuric rash, while petechiae spreaded over face and neck. Both patients were prostrate and drowsy with a diffuse oedema of periorbital region, hands and feet. Laboratory tests showed increased CRP 5.2 mg/ dl, WBC 23 × 109/L and PTL 623 × 109/L. Serological investigation for viruses (Epstein Barr Virus, Cytomegalovirus, and Hepatitis-C Virus) and bacteria were negative. ANA, pANCA, cANCA absent. Urinalysis showed proteinuria (1 g/24 h) and hematuria (1074 red blood cells/ field) in the girl. Cardiologic evaluation, lung X-ray, and abdominal ultrasound were unremarkable. Due to an aggressive infection of the cutaneous lesions, imipemen and teicoplanine were introduced. Three pulses of methylprednisolone were given to the girl, and then oral prednisone (2 mg/kg/daily) to control renal disease. Bullae faded within the next two weeks and necrotic lesions healed leaving a mild pigmentation and scars. Conclusion In childhood HSP the occurrence of hemorrhagic bullae may be a diagnostic challenge at onset in absence of other typical symptoms as many paediatric diseases including toxic epidermal necrolisis, erythema multiform, pemphigus, bullous impetigo, dermatitis herpetiformis and staphylococcal scalded skin syndrome may present with bullous cutaneous lesions. from 15th Paediatric Rheumatology European Society (PreS) Congress London, UK. 14–17 September 2008

Switching from a first to a second tumour necrosis factor (TNF) alpha antagonist in patients with juvenile idiopathic arthritis

Results A total of 33 patients initially treated with Etanercept were switched to Adalimumab after a mean of 25.9 months (range 3–87 months). Reasons for discontinuation of Etanercept were inefficacy (n = 23, 65.8%), uveitis (n = 6, 17.1%), intolerance (n = 3, 8.6%) and patients' request (n = 6, 17.1%). Follow up data on Adalimumab were obtained from 12 patients for a range of 2 to 26 months (mean 10.9 months). The maximum response rate of the PedACR30/50/70 on Etanercept was 82.4%/73.5%/ 67.6%. The last documented response rate on Etanercept showed a decrease to 47.1%/35.3%/29.4% (PedACR30/ 50/70). After switching to Adalimumab a maximum PedACR30/50/70 of 75%/66.7%/50% compared to start of Etanercept and 33.3/33.3/25 compared to start of Adalimumab was reached. Treatment on both drugs was safe with no report of serious AE. Conclusion Although a number of patients have reached a good PedACR response rate on Etanercept, treatment was unsatisfied and therefore it was switched to Adalimumab. According to the PedACR only minor improvement was observed after switching to Adalimumab. from 15th Paediatric Rheumatology European Society (PreS) Congress London, UK. 14–17 September 2008

Treatment of osteogenesis imperfecta with intravenous pamidronate in pediatric patients: comparison between a single-day infusion twice a year protocol with other regimens described in the literature

Conclusion Despite a lower increase in z-score in our serie, the clinical efficacy is similar to other protocols. There is a tendency towards greater gain in Z-score as the annual dosage increases.

Imaging in early-onset juvenile idiopathic arthritis: ultrasonography (US) and MRI favours conventional radiography in detecting early inflammatory changes in joints

Results Synovitis in affected joints was detected by US and MRI but not by X-ray. Conclusion US and MRI favours conventional radiography in detecting early inflammatory changes in smaller children with EO-JIA. Although detailed information is given by MRI, the use of MRI is limited to one anatomical region and the smaller children it cannot be performed without general anaesthesia. Thus, US seem most helpful in detecting early inflammatory changes in EO-JIA. from 15th Paediatric Rheumatology European Society (PreS) Congress London, UK. 14–17 September 2008

Henoch–Schönlein purpura

Henoch–Schonlein purpura (HSP) is the commonest vasculitis of childhood. The first description of this disorder was probably that of a young boy with “bloody points” over the shins of his...

Why should pediatric rheumatology be recognized as a separate subspecialty: an open letter to medical councils and government agencies

Some pediatric rheumatologists in the West may take for granted that pediatric rheumatology (PR) is a recognized subspecialty. Yet pediatric rheumatology has been accepted as a subspecialty in the United States only since 1990. There are still countries where many pediatric subspecialties are not given official recognition and support, including PR. This lack of recognition delays and impedes the development of PR, appropriate musculoskeletal and rheumatic teaching in medical schools, and optimal diagnosis and treatment for children with these illnesses. In the opinion of editorial staff, each country where pediatric rheumatology is reasonably well developed as a subspecialty has an obligation to help our pediatric rheumatologists elsewhere gain recognition, support, and respect. The Pediatric Rheumatology European Society (PReS) and the Pediatric Rheumatology International Trial Organization (PRINTO) have been leaders in this effort, but in many countries, pediatric rheumatology is still not recognized. This editorial offers rationales and justifications for medical and governmental entities accrediting pediatric rheumatology as a separate subspecialty that may aid in these efforts.

Childhood systemic sclerosis

Juvenile systemic sclerosis has a variety of clinical manifestations, sometimes different from the adult form. Early recognition, proper classification and treatment may improve the long-term outcome. A large multicenter study coordinated by the Pediatric Rheumatology European Society has yielded important information on the epidemiology and clinical manifestations of systemic sclerosis in childhood. An ad-hoc Committee on Classification Criteria for Juvenile Systemic Sclerosis developed the new classification criteria to help improve patient care by enabling earlier, more definite diagnoses and standardizing the conduct of clinical, epidemiologic, and outcome research for this rare disease. The overall outcome of children with systemic sclerosis is better than in adults but, in those cases with a fatal course, disease progression is rapid and an early involvement of internal organs is associated with poor outcome. Studies over the past few years have highlighted the peculiar clinical features and the better outcome of juvenile systemic sclerosis compared with the adult form and propose new pediatric classification criteria. Efforts have recently been made to address the definition of evidence-based recommendations for the treatment of adult and pediatric onset systemic sclerosis.

Localized scleroderma

Localized scleroderma, also known as morphoea, has a variety of clinical manifestations that can include systemic involvement. Early recognition, diagnosis and treatment may improve the long-term outcome. A large multicentre study coordinated by the Pediatric Rheumatology European Society has yielded important information on the epidemiology and clinical manifestations of juvenile localized scleroderma, especially as it pertains to systemic manifestations. Previous results using methotrexate and corticosteroids have been confirmed. Studies on phototherapy have also demonstrated efficacy. A new immunomodulator, imiquimod, has shown promise in an initial case series. Studies over the past year highlight the wide range of extracutaneous manifestations and different forms of localized scleroderma and suggest that treatment may be beneficial.

VII European Paediatric Rheumatology Congress: Annual Scientific Meeting of PRES

23–27 September 2000, Geneva, Switzerland Organised by: : Swiss Working Group for Paediatric Rheumatology Centre Multisite Romand de Rhumatologie Pediatrique Under the Patronage of: : Swiss Society of Paediatry Swiss...