[Arthritis and osteomyelitis in childhood and adolescence-Bacterial and nonbacterial]. / Arthritis und Osteomyelitis im Kindes- und Jugendalter ­ bakteriell und nichtbakteriell.

Bacterial arthritis and osteomyelitis are usually acute diseases, which in this way differ from the often insidious course of nonbacterial osteomyelitis; however, there is often an overlap both in less acute courses of bacterial illnesses and also in nonbacterial osteitis. The overlapping clinical phenomena can be explained by similar pathophysiological processes. In bacteria-related illnesses the identification of the pathogen and empirical or targeted anti-infectious treatment are prioritized, whereas no triggering agent is known for nonbacterial diseases. The diagnostics are based on the exclusion of differential diagnoses, clinical scores and magnetic resonance imaging (MRI). An activity-adapted anti-inflammatory treatment is indicated.

TNF-inhibitors or bisphosphonates in chronic nonbacterial osteomyelitis? - Results of an international retrospective multicenter study.

Chronic nonbacterial osteomyelitis (CNO) can cause significant morbidity, including bone pain and damage. In the absence of clinical trials, treatments include non-steroidal anti-inflammatory drugs, corticosteroids, TNF-inhibitors (TNFi) and/or bisphosphonates. In a retrospective chart review in the United Kingdom and Germany, we investigated response to TNFi and/or pamidronate. Ninety-one patients were included, receiving pamidronate (n = 47), TNFi (n = 22) or both sequentially (n = 22). Patients with fatigue [p = 0.003] and/or arthritis [p = 0.002] were more frequently treated with TNFi than pamidronate. Both therapies were associated with clinical remission at 6 months, and reduction of bone lesions on MRI at 12 months. While not reaching statistical significance, pamidronate resulted in faster resolution of MRI lesions. Fewer flares were observed with TNFi. Failure to respond to pamidronate was associated with female sex [p = 0.027], more lesions on MRI [p = 0.01] and higher CRP levels [p = 0.03]. Randomized clinical trials are needed to confirm observations and generate evidence.

Typical patterns of bone involvement in whole-body MRI of patients with chronic recurrent multifocal osteomyelitis (CRMO).

PURPOSE: The diagnosis of CRMO often involves a long patient history. We evaluated the spectrum of bone involvement in whole-body magnetic resonance imaging (WB-MRI) and assessed its potential contribution to a more rapid diagnosis. MATERIALS AND METHODS: WB-MRI (1.5 T, coronal STIR sequences) in 53 children and adolescents (mean age 11 years, 4.8 - 15.1) with histologically (n = 37) or clinically (n = 16) confirmed CRMO were retrospectively reviewed by two experienced pediatric radiologists. RESULTS: WB-MRI revealed multifocal lesions in all but one patients. Only 26 of them had presented with multifocal complaints. We detected 1 - 27 geographic lesions/patient (mean 9.7). 510 of 513 lesions were significantly hyperintense compared to normal bone marrow. The pelvis, lower extremities, shoulders and spine were most frequently involved. 40 patients (75 %) had bilateral symmetrical involvement of bones. Most of the lesions were located in tubular bones, in 87 % adjacent to one or both sides of a growth plate. 32 % of lesions showed periosteal involvement. Of 456 affected bones, 33 (7.2 %) were deformed, 6 (18 %) were vertebra plana. CONCLUSION: In the absence of more specific diagnostic criteria, WB-MRI can, in synopsis with clinical findings, substantially contribute to a rapid diagnosis of CRMO. It discovers the typical pattern of multifocal and bilateral bone involvement more often than has been reported for targeted MRI. It readily reveals the characteristic proximity of lesions to growth plates, the sacroiliac joint and triradiate cartilage and helps to uncover asymptomatic spinal complications.

[Definition, diagnosis and therapy of chronic widespread pain and so-called fibromyalgia syndrome in children and adolescents. Systematic literature review and guideline]. / Definition, Diagnostik und Therapie von chronischen Schmerzen in mehreren Körperregionen und des sogenannten Fibromyalgiesyndroms bei Kindern und Jugendlichen. Systematische Literaturübersicht und Leitlinie.

BACKGROUND: The scheduled update to the German S3 guidelines on fibromyalgia syndrome (FMS) by the Association of the Scientific Medical Societies ("Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften", AWMF; registration number 041/004) was planned starting in March 2011. MATERIALS AND METHODS: The development of the guidelines was coordinated by the German Interdisciplinary Association for Pain Therapy ("Deutsche Interdisziplinären Vereinigung für Schmerztherapie", DIVS), 9 scientific medical societies and 2 patient self-help organizations. Eight working groups with a total of 50 members were evenly balanced in terms of gender, medical field, potential conflicts of interest and hierarchical position in the medical and scientific fields. Literature searches were performed using the Medline, PsycInfo, Scopus and Cochrane Library databases (until December 2010). The grading of the strength of the evidence followed the scheme of the Oxford Centre for Evidence-Based Medicine. The formulation and grading of recommendations was accomplished using a multi-step, formal consensus process. The guidelines were reviewed by the boards of the participating scientific medical societies. RESULTS AND CONCLUSION: The diagnosis FMS in children and adolescents is not established. In so-called juvenile FMS (JFMS) multidimensional diagnostics with validated measures should be performed. Multimodal therapy is warranted. In the case of severe pain-related disability, therapy should be primarily performed on an inpatient basis. The English full-text version of this article is available at SpringerLink (under "Supplemental").

[Henoch-Schönlein purpura : most frequent form of vasculitis in childhood and adolescence]. / Purpura Schönlein-Henoch : Häufigste Vaskulitis des Kindes- und Jugendalters.

Henoch-Schönlein purpura is the most common form of vasculitis in childhood and adolescence. It is manifested as palpable purpuras in the region of position-dependent extremities with otherwise good clinical general condition. The expression on the skin can, however, be variable so that sometimes other differential diagnoses must be included. In typical cases an extensive diagnostic procedure is unnecessary but the short-term complications of abdominal symptoms as well as the long-term complications of nephritis should be pursued. If musculoskeletal symptoms occur paracetamol or non-steroidal anti-inflammatory drugs are effective as are steroids for reducing the duration of abdominal pain. The effectiveness of steroid prophylaxis of renal damage is still controversial.

[Updated statement by the German Society for Pediatric and Adolescent Rheumatology (GKJR) on the FDA's report regarding malignancies in anti-TNF-treated patients from Aug. 4, 2009]. / Aktualisierte Stellungnahme der GKJR zur Meldung der FDA über Fälle von Malignomen bei Anti-TNF-behandelten Patienten vom 04.08.2009.

TNF inhibitors and other biologicals have greatly expanded the therapeutic options for juvenile idiopathic arthritis (JIA). While the efficacy of etanercept and adalimumab has been proven in randomized controlled clinical trials, their long-term safety remains the subject of ongoing investigations. Reports of leukaemia and tumours in children and adolescents treated with etanercept, infliximab and adalimumab have raised questions about an increased risk for malignancies, with lymphoma accounting for the largest group at 50% of all 48 malignancies reported by the FDA.Consequently, TNF inhibitors should be indicated under careful consideration of individual risk factors, such as increased family occurrence of malignancies, or pre-treatment with carcinogenic substances such as cyclophosphamide. This is particularly true for non-approved substances, and non-approved indications, and for combination therapy of TNF inhibitors with immunosuppressive drugs. On the other hand, however, treatment should not be stopped or started in any patient in whom treatment is necessary due to the current knowledge. Adequate patient information, surveillance and documentation of treatment in the registry of the GKJR is strongly recommended.

Acute febrile neutrophilic dermatosis (Sweet's syndrome) in childhood and adolescence: two new patients and review of the literature on associated diseases.

OBJECTIVES: The objectives of this study were to analyse the literature on Sweet's syndrome in childhood focussing on associated diseases and to suggest possible screening procedures for this group of patients. Furthermore, two new patients with Sweet's syndrome are reported. METHODS: A literature search was performed on Pub med using search terms "sweet* syndrome*" and neutrophil* dermatos*. Patients were subdivided into the following groups: classic/idiopathic, paraneoplastic, and parainflammatory Sweet's syndrome. RESULTS: The literature search revealed 64 patients (including our two patients) who were diagnosed with Sweet's syndrome in childhood and adolescence; 27 (42%) patients were categorized as "classic/idiopathic Sweet's syndrome". In 37 patients (58%) chronic associated diseases were reported. Out of these, 21 (33%) patients were categorized as "parainflammatory Sweet's syndrome" including chronic recurrent multifocal osteomyelitis, vasculitis with aortitis, recurrent infections due to immunodeficiencies, arthritis, and systemic lupus erythematosus. Sixteen (25%) patients were categorized as "paraneoplastic Sweet's syndrome" comprising both malignant and premalignant diseases like leukemia, aplastic anaemia, and Fanconi anaemia. As all five (8%) patients treated with drugs (granulocyte-colony stimulating factor, retinoid acid) suffered from malignant, premalignant, or parainflammatory diseases, these patients were categorized according to the underlying disease. Two new children with Sweet's syndrome and associated diseases are presented here, one of them suffering from recurrent infections and trisomy 21, while the other was diagnosed with CNS vasculitis 5(1/2) years after the primary diagnosis. CONCLUSIONS: Sweet's syndrome should be considered in differential diagnosis of prolonged fever with cutaneous involvement. As most cases of pediatric Sweet's syndrome are associated with other diseases we suggest careful screening and monitoring of these patients especially concerning malignant/premalignant diseases, immunodeficiencies, cardiovascular involvement, autoimmune diseases, and drug associations.

[Purpura Schoenlein-Henoch: results of the Wörlitz 2005 Consensus Conference focusing on diagnosis and therapy]. / Purpura Schoenlein-Henoch - Ergebnisse der Wörlitzer Konsensusgespräche 2005 zum diagnostischen und therapeutischen Vorgehen.

Henoch-Schoenlein Purpura (PSH) is the most frequent vasculitis in childhood. Skin, bowel and joints are characteristically involved. Although renal manifestations are common PSH-nephritis is infrequent but characterized by a poor prognosis. In autumn 2005 diagnosis and treatment were discussed at the 8th consensus meeting in Wörlitz. These results are summarized.

The German etanercept registry for treatment of juvenile idiopathic arthritis.

OBJECTIVE: To describe a registry set up to monitor children treated with etanercept in Germany and Austria. METHODS: Giannini's criteria, duration of morning stiffness, number of swollen, tender and contracted joints, adverse events, and reasons for discontinuation were assessed. RESULTS: 322 patients with juvenile idiopathic arthritis (JIA) and 12 additional patients with non-JIA rheumatic diagnoses were included. Therapeutic efficacy was observed from one month after treatment was started. The number of patients with significant improvement and the degree of improvement increased during the first year. The mean (SD) number of tender and swollen joints decreased from 9 (9) and 8.4 (9) to 3.0 (6.5) and 4.5 (7) after one month, and to 2.2 (5.5) and 3.3 (5.5) after three months; morning stiffness decreased from 45 (65) minutes to 12 (30) and 7 (19) after one and three months (p<0.001 for all). Using Gianinni's criteria of 30%, 50%, and 70% improvement, a therapeutic response in JIA patients was achieved in, respectively, 66%, 54%, and 30% after one month, 78%, 61%, and 38% after three months, and 83%, 72%, and 52% after six months. Therapeutic efficacy was lower in patients with systemic onset arthritis. Overall tolerability was good: in 592 patient treatment-years there were 69 reports of adverse events in 56 patients, including one CNS demyelination. There were no opportunistic infections or lupus-like reactions. Treatment was discontinued in 53 JIA patients, in 25 because of lack of efficacy. CONCLUSION: Etanercept treatment was safe and led to a significant improvement in most JIA patients resistant to conventional treatment.

[Pelvic type of chronic recurrent multifocal osteomyelitis]. / Die Chronisch rekurrierende multifokale Osteomyelitis (CRMO)*: - Teil 2 Klinisch-radiologisch-histopathologische Studie zum kindlich-jugendlichen "Beckentyp" bei 11 Patienten -

We report about the juvenile and adolescent pelvic type of CRMO in 7 girls and 4 boys. The results show from solitary up to 7 pelvic lesions (average 3 lesions) predominantly in the acetabular and paraacetabular region (sometimes with hip joint effusion as a sign for concomitant coxitis).Tc99m-bone-scan is helpful to evaluate exactly the pattern of bone affection. We find 3 stages of an primarily chronic, non-purulent osteomyelitis going along with a "plasma-cell-sclerotic process", leading to a Garrè-type sclerosing end-stage, which probably heals after some years spontaneously, projecting on clinical symptoms and radiological appearance. The concomitant coxitis ("sympathetic coxitis") is clinically often in the foreground, but reversible. Pain in case of CRMO responds surprisingly well on medication with acithromycine. Knowing about CRMO in its different clinical appearances - especially concerning what we call "sympathetic coxitis" - can be a useful for pediatric rheumatologists and orthopedic surgeons, as well as MRI-focused radiologists and pathologists. Therapy might find a useful drug in acithromycine. In conclusion we d like to point out, that CRMO is one entity under the "roof" of the so called SAPHO-syndrome, which again shows us, that SAPHO-syndrome ist not a diagnosis itself but more a sign-post on the way to a correct diagnosis.