Lupus nephritis (LN) occurs in 50%-60% of patients with childhood-onset systemic lupus erythematosus (cSLE), leading to significant morbidity. Timely recognition of renal involvement and appropriate treatment are essential to prevent renal damage. The Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) initiative aimed to generate diagnostic and management regimens for children and adolescents with rheumatic diseases including cSLE. Here, we provide evidence-based recommendations for diagnosis and treatment of childhood LN. Recommendations were developed using the European League Against Rheumatism standard operating procedures. A European-wide expert committee including paediatric nephrology representation formulated recommendations using a nominal group technique. Six recommendations regarding diagnosis and 20 recommendations covering treatment choices and goals were accepted, including each class of LN, described in the International Society of Nephrology/Renal Pathology Society 2003 classification system. Treatment goal should be complete renal response. Treatment of class I LN should mainly be guided by other symptoms. Class II LN should be treated initially with low-dose prednisone, only adding a disease-modifying antirheumatic drug after 3 months of persistent proteinuria or prednisone dependency. Induction treatment of class III/IV LN should be mycophenolate mofetil (MMF) or intravenous cyclophosphamide combined with corticosteroids; maintenance treatment should be MMF or azathioprine for at least 3 years. In pure class V LN, MMF with low-dose prednisone can be used as induction and MMF as maintenance treatment. The SHARE recommendations for diagnosis and treatment of LN have been generated to support uniform and high-quality care for all children with SLE.

• Klíčová slova
• corticosteroids, disease activity, lupus nephritis, systemic lupus erythematosus, treatment,
• MeSH
• antirevmatika terapeutické užití   MeSH
• azathioprin terapeutické užití   MeSH
• cyklofosfamid terapeutické užití   MeSH
• dítě MeSH
• hormony kůry nadledvin terapeutické užití   MeSH
• imunosupresiva terapeutické užití   MeSH
• indukce remise metody   MeSH
• kyselina mykofenolová terapeutické užití   MeSH
• lidé MeSH
• management nemoci MeSH
• medicína založená na důkazech normy   MeSH
• nefritida při lupus erythematodes diagnóza   farmakoterapie   MeSH
• směrnice pro lékařskou praxi jako téma * MeSH
• věk při počátku nemoci MeSH
• výsledek terapie MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Evropa MeSH
• Názvy látek
• antirevmatika MeSH
• azathioprin MeSH
• cyklofosfamid MeSH
• hormony kůry nadledvin MeSH
• imunosupresiva MeSH
• kyselina mykofenolová MeSH

Childhood-onset systemic lupus erythematosus (cSLE) is a rare, multisystem and potentially life-threatening autoimmune disorder with significant associated morbidity. Evidence-based guidelines are sparse and management is often based on clinical expertise. SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) was launched to optimise and disseminate management regimens for children and young adults with rheumatic diseases like cSLE. Here, we provide evidence-based recommendations for diagnosis and treatment of cSLE. In view of extent and complexity of cSLE and its various manifestations, recommendations for lupus nephritis and antiphospholipid syndrome will be published separately. Recommendations were generated using the EULAR (European League Against Rheumatism) standard operating procedure. An expert committee consisting of paediatric rheumatologists and representation of paediatric nephrology from across Europe discussed evidence-based recommendations during two consensus meetings. Recommendations were accepted if >80% agreement was reached. A total of 25 recommendations regarding key approaches to diagnosis and treatment of cSLE were made. The recommendations include 11 on diagnosis, 9 on disease monitoring and 5 on general treatment. Topics included: appropriate use of SLE classification criteria, disease activity and damage indices; adequate assessment of autoantibody profiles; secondary macrophage activation syndrome; use of hydroxychloroquine and corticosteroid-sparing regimens; and the importance of addressing poor adherence. Ten recommendations were accepted regarding general diagnostic strategies and treatment indications of neuropsychiatric cSLE. The SHARE recommendations for cSLE and neuropsychiatric manifestations of cSLE have been formulated by an evidence-based consensus process to support uniform, high-quality standards of care for children with cSLE.

• Klíčová slova
• Autoantibodies, Corticosteroids, Disease Activity, Systemic Lupus Erythematosus, Treatment,
• MeSH
• dítě MeSH
• lidé MeSH
• medicína založená na důkazech normy   MeSH
• mezinárodní spolupráce MeSH
• mladiství MeSH
• mladý dospělý MeSH
• směrnice pro lékařskou praxi jako téma * MeSH
• systémový lupus erythematodes diagnóza   terapie   MeSH
• věk při počátku nemoci MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Evropa MeSH

BACKGROUND: In 2012, a European initiative called Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. Juvenile dermatomyositis (JDM) is a rare disease within the group of paediatric rheumatic diseases (PRDs) and can lead to significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physicians' experience. Consequently, treatment regimens differ throughout Europe. OBJECTIVES: To provide recommendations for diagnosis and treatment of JDM. METHODS: Recommendations were developed by an evidence-informed consensus process using the European League Against Rheumatism standard operating procedures. A committee was constituted, consisting of 19 experienced paediatric rheumatologists and 2 experts in paediatric exercise physiology and physical therapy, mainly from Europe. Recommendations derived from a validated systematic literature review were evaluated by an online survey and subsequently discussed at two consensus meetings using nominal group technique. Recommendations were accepted if >80% agreement was reached. RESULTS: In total, 7 overarching principles, 33 recommendations on diagnosis and 19 recommendations on therapy were accepted with >80% agreement among experts. Topics covered include assessment of skin, muscle and major organ involvement and suggested treatment pathways. CONCLUSIONS: The SHARE initiative aims to identify best practices for treatment of patients suffering from PRD. Within this remit, recommendations for the diagnosis and treatment of JDM have been formulated by an evidence-informed consensus process to produce a standard of care for patients with JDM throughout Europe.

• Klíčová slova
• Autoimmune Diseases, Dermatomyositis, Treatment,
• MeSH
• cyklosporin terapeutické užití   MeSH
• dermatomyozitida diagnóza   terapie   MeSH
• glukokortikoidy terapeutické užití   MeSH
• imunosupresiva terapeutické užití   MeSH
• kyselina mykofenolová terapeutické užití   MeSH
• lidé MeSH
• medicína založená na důkazech MeSH
• methotrexát terapeutické užití   MeSH
• prednisolon terapeutické užití   MeSH
• přípravky chránící proti slunci terapeutické užití   MeSH
• rituximab terapeutické užití   MeSH
• směrnice pro lékařskou praxi jako téma * MeSH
• společnosti lékařské MeSH
• terapie cvičením * MeSH
• týmová péče o pacienty organizace a řízení   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• konsensus - konference MeSH
• přehledy MeSH
• Geografické názvy
• Evropa MeSH
• Názvy látek
• cyklosporin MeSH
• glukokortikoidy MeSH
• imunosupresiva MeSH
• kyselina mykofenolová MeSH
• methotrexát MeSH
• prednisolon MeSH
• přípravky chránící proti slunci MeSH
• rituximab MeSH