Consensus statement on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: Localized scleroderma, systemic sclerosis and overlap syndromes.

The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present consensus provides an update to the 2017 European Dermatology Forum Guidelines, focusing on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, updated strategies for the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 1 of this consensus provides clinicians with an overview of the diagnosis and treatment of localized scleroderma (morphea), and systemic sclerosis including overlap syndromes.

Outcome of children with oligoarticular juvenile idiopathic arthritis compared to polyarthritis on methotrexate- data of the German BIKER registry.

BACKGROUND: Oligoarticular juvenile idiopathic arthritis (oligoJIA) is the most commonly diagnosed category of chronic arthritis in children. Nevertheless, there are no evidence- based guidelines for its treatment, in particular for the use of methotrexate (MTX). The primary objective of this analysis is to evaluate the outcomes in patients with persistent oligoJIA compared to those with extended oligoJIA and rheumatoid factor (RF) negative polyarthritis treated with methotrexate. METHODS: Patients with persistent or extended oligoJIA or RF negative PA recorded in the Biologics in Pediatric Rheumatology Registry (BiKeR), receiving methotrexate for the first time were included in the analyses. Efficacy was determined using the Juvenile Arthritis Disease Activity Score 10 (JADAS 10). Safety assessment included the documentation of adverse and serious adverse events. RESULTS: From 2005 through 2011, 1056 patients were included: 370 patients with persistent oligoJIA, 221 patients with extended oligoJIA and 467 patients with RF negative PA. Therapeutic efficacy was observed following the start of methotrexate. Over a period of 24 months JADAS-minimal disease activity (JADAS ≤2) was reached in 44% of patients with persistent oligoJIA, 38% with extended oligoJIA, 46% with RF negative PA, JADAS-remission defined as JADAS ≤1 was reached in 33% of patients with persistent oligoJIA, 29% with extended oligoJIA and 35% (RF negative PA). Patients with extended oligoJIA achieved JADAS remission significantly later and received additional biologic disease-modifying drugs significantly more often than patients with persistent oligoJIA or RF negative PA (p < 0.001). Tolerability was comparable. New onset uveitis occurred in 0.3 to 2.2 per 100 patient years. CONCLUSIONS: Patients with persistent oligoJIA taking methotrexate are at least as likely to enter remission as patients with extended oligo JIA or polyarticular JIA. Patients with extended oligoJIA achieved JADAS remission significantly later. Within 2 years, almost half of the patients with persistent oligoJIA achieved JADAS-minimal disease activity.

Temporomandibular joint involvement in patients with juvenile idiopathic arthritis: a retrospective chart review.

OBJECTIVE: To study the proportion of patients with temporomandibular joint (TMJ) involvement among patients with juvenile idiopathic arthritis (JIA), as well as associated clinical characteristics and signs/symptoms. METHOD: We performed a retrospective chart review on consecutive patients followed in the Hamburg Centre for Paediatric and Adolescent Rheumatology Eilbek between January 2010 and July 2012. TMJ involvement was diagnosed based on clinical examination; a subgroup of patients was also assessed by magnetic resonance imaging (MRI). RESULTS: The study included 2413 patients with JIA (52.1% girls, mean age at JIA onset 9.5 years). The most frequent JIA category was oligoarthritis (46.6%), followed by enthesitis-related arthritis (ERA; 38.1%). TMJ involvement was diagnosed in 843/2413 patients (34.9%) (677 MRI-confirmed, four not MRI-confirmed, no MRI examination in 162). Female gender (p = 0.017), higher number of additional joints with active arthritis (p < 0.001), anti-nuclear antibody (ANA) positivity (p = 0.005), higher age (p = 0.020), and oligoarthritis (persistent and extended; p = 0.043) were significantly associated with TMJ involvement. Human leucocyte antigen-B27-positive patients were less likely to have TMJ involvement (p = 0.023). Pain on palpation and pain while chewing were statistically significantly associated with TMJ involvement (p = 0.008 and p = 0.020, respectively). CONCLUSIONS: Based on our findings, to identify TMJ involvement special attention should be paid to JIA patients with female gender, ANA positivity, and oligoarthritis, as well as those with a higher number of additional joints with active arthritis; and regular examinations of the TMJ should be performed.

Update of the evidence based, interdisciplinary guideline for anti-inflammatory treatment of uveitis associated with juvenile idiopathic arthritis.

BACKGROUND: Uveitis in juvenile idiopathic arthritis (JIAU) is frequently associated with the development of complications and visual loss. Topical corticosteroids are the first line therapy, and disease modifying anti-rheumatic drugs (DMARDs) are commonly used. However, treatment has not been standardized. METHODS: Interdisciplinary guideline were developed with representatives from the German Ophthalmological Society, Society for Paediatric Rheumatology, Professional Association of Ophthalmologists, German Society for Rheumatology, parents' group, moderated by the Association of the Scientific Medical Societies in Germany. A systematic literature analysis in MEDLINE was performed, evidence and recommendations were graded, an algorithm for anti-inflammatory treatment and final statements were discussed in a consensus meeting (Nominal Group Technique), a preliminary draft was fine-tuned and discussed thereafter by all participants (Delphi procedure). RESULTS: Consensus was reached on recommendations, including a standardized treatment strategy according to uveitis severity in the individual patient. Thus, methotrexate shall be introduced for uveitis not responding to low-dose (≤ 2 applications/day) topical corticosteroids, and a TNFalpha antibody (preferably adalimumab) used, if uveitis inactivity is not achieved. In very severe active uveitis with uveitis-related deterioration of vision, systemic corticosteroids should be considered for bridging until DMARDs take effect. If TNFalpha antibodies fail to take effect or lose effect, another biological should be selected (tocilizumab, abatacept or rituximab). De-escalation of DMARDs should be preceded by a period of  ≥ 2 years of uveitis inactivity. CONCLUSIONS: An interdisciplinary, evidence-based treatment guideline for JIAU is presented.

Adalimumab versus adalimumab and methotrexate for the treatment of juvenile idiopathic arthritis: long-term data from the German BIKER registry.

OBJECTIVE: Adalimumab (ADA) has become a valuable treatment option for juvenile idiopathic arthritis (JIA). The importance of combination with methotrexate (MTX) is unclear. METHOD: Data from the German Biologics in Paediatric Rheumatology (BIKER) registry are reported. Response to treatment was analysed using JIA American College of Rheumatology (ACR) scores, 10-joint Juvenile Arthritis Disease Activity Score (JADAS10), and improvement of functional status and ACR inactive disease criteria. Compa-risons between rates of adverse events (AEs) and serious adverse events (SAEs) provided data for the safety assessment. RESULTS: Overall, 584 patients with non-systemic JIA started ADA therapy, 61% of whom received concomitant MTX treatment at baseline. The latter patients were younger (p < 0.001), with shorter disease duration (p = 0.001), more frequently had antinuclear antibodies (p = 0.04), and had higher baseline JADAS10 scores (p = 0.03). In patients with ADA monotherapy, enthesitis-related arthritis (p = 0.004) and presence of human leucocyte antigen-B27 (p = 0.008) were documented more often. Mean treatment duration in both cohorts was 15 months. Comparable last follow-up rates for JIA ACR 30/50/70/90% response, JADAS minimal disease activity, JADAS remission, and ACR inactive disease were, respectively, 75/72/64/49%, 66%, 46%, and 58% for ADA monotherapy, and 77/72/61/45%, 64%, 48%, and 55%, for ADA + MTX. During 1082 patient-years (PY) of ADA exposure, 725 AEs (67/100 PY), including 57 SAEs (5.3/100 PY), were reported. Serious infections were reported in 10 patients (0.9/100 PY) and 11 (1.0/100 PY) had varicella infections/zoster reactivation. Rates of AEs, SAEs, infectious events, and serious infections did not differ between the cohorts. Elevated transaminases (p = 0.005) and gastrointestinal events (p < 0.0001) were reported more often in the combination cohort. Two pregnancies and no deaths were reported. CONCLUSION: ADA demonstrated an acceptable risk profile and high percentages of patients in both cohorts showed sufficient treatment response. No differences in treatment response or adherence to treatment were found.

There is a need for new systemic sclerosis subset criteria. A content analytic approach.

OBJECTIVES: Systemic sclerosis (SSc) is heterogenous. The objectives of this study were to evaluate the purpose, strengths and limitations of existing SSc subset criteria, and identify ideas among experts about subsets. METHODS: We conducted semi-structured interviews with randomly sampled international SSc experts. The interview transcripts underwent an iterative process with text deconstructed to single thought units until a saturated conceptual framework with coding was achieved and respondent occurrence tabulated. Serial cross-referential analyses of clusters were developed. RESULTS: Thirty experts from 13 countries were included; 67% were male, 63% were from Europe and 37% from North America; median experience of 22.5 years, with a median of 55 new SSc patients annually. Three thematic clusters regarding subsetting were identified: research and communication; management; and prognosis (prediction of internal organ involvement, survival). The strength of the limited/diffuse system was its ease of use, however 10% stated this system had marginal value. Shortcomings of the diffuse/limited classification were the risk of misclassification, predictions/generalizations did not always hold true, and that the elbow or knee threshold was arbitrary. Eighty-seven percent use more than 2 subsets including: SSc sine scleroderma, overlap conditions, antibody-determined subsets, speed of progression, and age of onset (juvenile, elderly). CONCLUSIONS: We have synthesized an international view of the construct of SSc subsets in the modern era. We found a number of factors underlying the construct of SSc subsets. Considerations for the next phase include rate of change and hierarchal clustering (e.g. limited/diffuse, then by antibodies).

European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis.

The term 'sclerosing diseases of the skin' comprises specific dermatological entities which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, current strategies in the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 2 of this guideline provides clinicians with an overview of the diagnosis and treatment of scleromyxedema, scleredema (of Buschke) and nephrogenic systemic sclerosis (nephrogenic fibrosing dermopathy).

European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes.

The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, current strategies in the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 1 of this guideline provides clinicians with an overview of the diagnosis and treatment of localized scleroderma (morphea), and systemic sclerosis including overlap syndromes of systemic sclerosis with diseases of the rheumatological spectrum.

[Uveitis in juvenile idiopathic arthritis]. / Uveitis bei juveniler idiopathischer Arthritis.

BACKGROUND: Uveitis is the most common extra-articular manifestation of juvenile idiopathic arthritis (JIA). It occurs, according to German registry data, in around 12% of JIA patients and can lead to a loss of vision, especially in cases of delayed diagnosis and/or inadequate therapy. OBJECTIVE: A review of current aspects of diagnosis and therapy was carried out. MATERIAL AND METHODS: This is a review article of the current literature. RESULTS: The risk of uveitis is significantly elevated in patients with an oligoarticular course of JIA, ANA positivity and young age at onset of JIA. During the mostly asymptomatic course of uveitis severe complications, such as cataracts, glaucoma or macular edema can occur, limiting visual acuity. Early detection of uveitis and interdisciplinary cooperation of uveitis specialists and pediatric rheumatologists is of major importance to ensure a favorable long-term prognosis. The initial therapy consists of topical steroids; however, in cases of insufficient response or complicated course of uveitis, conventional synthetic (mainly methotrexate) or biological disease-modifying antirheumatic drugs (bDMARDs) are required. In respect to bDMARDs, the highest evidence exists for treatment with adalimumab; however, depending on the clinical course of disease, other bDMARDs, such as infliximab, golimumab, tocilizumab, abatacept or rituximab may also have a beneficial effect. Despite these treatment options, uveitis or arthritis may frequently persist into adult age. Adequate and early recognition and treatment of uveitis-related complications is of major importance to ensure a good long-term visual prognosis. CONCLUSION: Early diagnosis of JIA-associated uveitis and early implementation of effective treatment, especially steroid-sparing DMARD therapy, aims at achieving uveitis inactivity and prevention of ocular damage.

[Current news from the BIKER register]. / Aktuelles aus dem BIKER-Register.

Innovative developments in the pharmacotherapy of juvenile idiopathic arthritis and especially biologics allow the formulation of new therapeutic targets, such as the rapid induction of remission with shortening of the period of active disease and therefore preventing damage and disability. These new therapies also represent a challenge to the monitoring of drug safety, the pharmacovigilance. For this purpose the Society for Paediatric and Adolescent Rheumatology has set up an early register to record achievements in treatment improvement and in addition to independently assess information on drug safety, acute tolerance and long-term safety.

Efficacy and safety of oral and parenteral methotrexate therapy in children with juvenile idiopathic arthritis: an observational study with patients from the German Methotrexate Registry.

OBJECTIVE: The German Methotrexate Registry has been collecting data concerning the efficacy and safety of methotrexate (MTX) treatment since 2005. The aim of this retrospective analysis is to compare oral and parenteral MTX treatment regarding efficacy and safety. METHODS: Inclusion criteria were diagnosis of juvenile idiopathic arthritis, MTX treatment for at least 6 months, a consistent route of administration of MTX, and no previous or concomitant treatment with biologic agents. Efficacy was measured by the American College of Rheumatology (ACR) pediatric (Pedi) criteria. Primary outcome was efficacy defined as the number of patients reaching ACR Pedi 30 improvement criteria after 6 months of treatment. Secondary outcome criteria were the ACR Pedi 50 and Pedi 70 criteria at 6 and 12 months, respectively. Analyses were performed with the intent-to-treat population. RESULTS: Of the 411 eligible patients, 259 (63%) received oral MTX and 152 (37%) received subcutaneous MTX. In both patient groups, a comparable weekly dose of MTX (0.4 mg/kg versus 0.42 mg/kg) was used, and a comparable number of patients received concomitant steroids. The primary outcome in both treatment groups was that a comparably high number of patients showed a clinical response according to the ACR Pedi 30 score after 6 months of treatment (73% versus 72%; P = 0.87). Twenty-two percent of patients with oral therapy and 27% with subcutaneous therapy had at least 1 documented adverse event. Discontinuation of treatment was observed in both groups with equal frequency, while significantly more patients with subcutaneous application discontinued MTX because of adverse events (11% versus 5%; P = 0.02). CONCLUSION: In this retrospective analysis, parenteral MTX was not superior to oral administration regarding efficacy and tolerability.

Evidence-based, interdisciplinary guidelines for anti-inflammatory treatment of uveitis associated with juvenile idiopathic arthritis.

Uveitis in juvenile idiopathic arthritis (JIA) is frequently associated with the development of complications and visual loss. Topical corticosteroids are the first-choice therapy, and immunosuppression is commonly used. However, treatment has not been standardized. Representatives from the German Ophthalmological Society, Society for Childhood and Adolescent Rheumatology, and the German Society for Rheumatology reached consensus on a standardized treatment strategy according to disease severity in the individual patient. The recommendations were based on a systematic literature analysis in MEDLINE and consensus expert meetings. Evidence and recommendations were graded, and an algorithm for anti-inflammatory treatment and final statements confirmed in a Delphi method. An interdisciplinary, evidence-based treatment guideline for JIA uveitis is presented.

[Evidence and consensus based treatment guidelines 2010 for juvenile idiopathic arthritis by the German Society of Paediatric Rheumatology]. / Interdisziplinäre S2-Therapieleitlinie der Juvenilen Idiopathischen Arthritis (2. Auflage).

BACKGROUND: Treatment of Juvenile Idiopathic Arthritis (JIA) has improved quality of life in children and adolescents with JIA. Standardisation of care offers the chance to improve the quality of care of those patients. New studies have been published after completion of our last treatment guideline (2007). An updated consensus process is mandatory. METHODS: A systematic literature analysis in PUBMED (key words: juvenile idiopathic (rheumatoid) arthritis, therapy; limits: humans, published in the last 3 years, all child 0-18 years, clinical trial) revealed 17 relevant studies. Studies relating to diagnosis of JIA, Uveitis, vaccination, transition were excluded. Representatives nominated by scientific societies and organisations were invited to consensus conferences which were hosted by a professional moderator. The following societies were invited: Berufsverband der Kinder- und Jugendärzte (BVKJ), Deutsche Gesellschaft für Kinder- und Jugendmedizin (DGKJ), Deutsche Gesellschaft für Rheumatologie (DGRh), Deutsche Ophthalmologische Gesellschaft (DOG), Deutsche Rheuma-Liga Bundesverband, Verein zur Förderung und Unterstützung rheumatologisch erkrankter Kinder und deren Eltern, Vereinigung für Kinderorthopädie, Zentraler Verband der Physiotherapeuten und Krankengymnasten (ZVK). Consensus conferences were each attended by more than 95% of the nominated representatives. Consensus statements were confirmed by nominal group technique and Delphi method. RESULTS AND CONCLUSION: Updated consensus statements regarding drug therapy, symptomatic and surgical management of JIA were compiled and judged strictly by the criteria of Evidence-Based Medicine (EBM).

[Uveitis in juvenile idiopathic arthritis]. / Uveitis bei juveniler idiopathischer Arthritis.

Anterior uveitis usually occurs in the context of juvenile idiopathic arthritis in about 10% of patients. Frequency is dependent on JIA subtype. Uveitis is most commonly found in patients with extended oligoarthritis (up to 25%) and early-age onset of arthritis. As the uveitis is usually without externally recognisable signs and often leads to ocular complications, all JIA patients should undergo regular ophthalmological examinations to ensure the promptest possible diagnosis and therapy. About 25% of uveitis patients have a complicated clinical course and require systemic immunosuppression. Immunosuppressive therapy should be started as early as possible if the dosage of topical glucocorticoids is not less than 3 drops per day and systemic glucocorticoid therapy is not less than 0.1 mg / kg body weight after 12 weeks. Methotrexate is commonly started as a first line immunosuppressive therapy. In the case of treatment failure, additional therapy can consist of combination therapy with cyclosporine A and biologicals. The main therapeutic goal is to achieve remission. Management of the typical vision-threatening complications such as cataract, glaucoma, ocular hypotension, and macular edema is particularly challenging.

[Autoinflammatory diseases in childhood]. / Klinik der autoinflammatorischen Erkrankungen im Kindesalter.

Autoinflammatory diseases are a group of monogenic inflammatory diseases with an early onset in childhood. Previously these diseases were summarized as"periodic fever syndromes." Included in this spectrum are familial Mediterranean fever, mevalonate kinase deficiency, and tumor necrosis factor receptor-associated disease. They are characterized by periodic or recurrent episodes of systemic inflammation causing fever, accompanied by rash, serositis, lymphadenopathy, arthritis, and other clinical manifestations. The other large group of autoinflammatory diseases consists of the cryopyrin-associated periodic syndromes, which include the cryopyrinopathies. The mildest form is familial cold-associated syndrome, a more severe form is Muckle-Wells syndrome, and the most severe is neonatal-onset multisystem inflammatory disease/chronic infantile neurological cutaneous and articular syndrome. These are characterized by chronic or recurrent systemic inflammation associated with various clinical presentations, including urticaria-like rash, arthritis, sensorineural deafness, and central nervous system and bone involvement. In our review we focus on the clinical presentation of these diseases.

Safety and efficacy of combination of etanercept and methotrexate compared to treatment with etanercept only in patients with juvenile idiopathic arthritis (JIA): preliminary data from the German JIA Registry.

OBJECTIVE: Etanercept monotherapy has been studied and approved for treatment of polyarticular juvenile idiopathic arthritis (JIA). The following study evaluates the safety and efficacy of combination therapy of etanercept and methotrexate compared to etanercept monotherapy in JIA. METHODS: We perfomed an open, non-randomised study on patients who had previously failed to respond to at least one disease-modifying antirheumatic drug (DMARD). A total of 722 patients with JIA in whom at least 1 item of follow-up data was recorded were identified; of these, 118 patients treated with further slow acting drugs were excluded. In all, 504 patients were treated with a combination of etanercept and methotrexate. A total of 100 patients treated with etanercept only were in the control group. Efficacy was calculated using the American College of Rheumatology paediatric scores for 30, 50 and 70% improvement (PedACR30/50/70). Adverse events (AEs) and serious adverse events (SAEs) were reported. RESULTS: After 12 months 55 patients in the monotherapy group and 376 patients in the etanercept and methotrexate group were available for comparison. For the intention to treat analysis, 65 patients discontinuing treatment prematurely were included. All activity parameters decreased significantly in both treatment groups. After 12 months 81%/74%/62% of patients of the etanercept and methotrexate group and 70%/63%/45% of patients of the etanercept monotherapy group achieved PedACR30/50/70 scores, respectively (p<0.05 for PedACR30, p<0.01 for PedACR70). The likelihood of achieving a PedACR70 increased with combination therapy with an odds ratio of 2.1 (95% CI 1.2 to 3.5). In total, 25 infectious and 23 non-infectious SAEs including 3 malignancies occurred in the etanercept and methotrexate group, and 1 infectious and 3 non-infectious SAEs occurred in the single etanercept group. CONCLUSIONS: The patients' disease activity improved during etanercept monotherapy and etanercept and methotrexate combination therapy. Tolerability in both treatment groups was comparable.

Skin involvement in systemic sclerosis.

Skin thickening is a characteristic feature of SSc. More extensive skin involvement coincides with more severe internal organ manifestation(s), poor prognosis and increased disability, at least in the early phase of the diffuse cutaneous scleroderma subset. The fully validated, feasible method ('gold standard') for measuring the dermal skin thickness is the modified Rodnan skin score (mRSS). The responsiveness of mRSS was somewhat modest in clinical trials, and a careful teaching process is necessary. Parallel method(s) for measuring skin thickness need to be used in the future. Ultrasound (US) measurement of the dermis with a 20-30 MHz probe is a valid, reproducible and responsive method in patients with dcSSc. However, US is time-consuming and requires a training process. Of the mechanical instruments available, only the durometer, which measures the hardness of skin, has been validated. The inter- and intra-observer reproducibility and sensitivity to change of durometry were good, and correlated with mRSS and US-measured skin thickness. Several further mechanical instruments exist including the elastometer, twistometer, cutometer and plicometer. They seem to distinguish between involved and non-involved skin, and therefore merit further evaluation. The measurement of late-stage, irreversible skin damage/atrophy should be resolved in the future through the development and validation of new instruments.

The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement.

OBJECTIVE: Systemic sclerosis (SSc) is a rare, heterogeneous disease, which affects different organs and therefore requires interdisciplinary diagnostic and therapeutic management. To improve the detection and follow-up of patients presenting with different disease manifestations, an interdisciplinary registry was founded with contributions from different subspecialties involved in the care of patients with SSc. METHODS: A questionnaire was developed to collect a core set of clinical data to determine the current disease status. Patients were grouped into five descriptive disease subsets, i.e. lcSSc, dcSSc, SSc sine scleroderma, overlap-syndrome and UCTD with scleroderma features. RESULTS: Of the 1483 patients, 45.5% of patients had lcSSc and 32.7% dcSSc. Overlap syndrome was diagnosed in 10.9% of patients, while 8.8% had an undifferentiated form. SSc sine scleroderma was present in 1.5% of patients. Organ involvement was markedly different between subsets; pulmonary fibrosis for instance was significantly more frequent in dcSSc (56.1%) than in overlap syndrome (30.6%) or lcSSc (20.8%). Pulmonary hypertension was more common in dcSSc (18.5%) compared with lcSSc (14.9%), overlap syndrome (8.2%) and undifferentiated disease (4.1%). Musculoskeletal involvement was typical for overlap syndromes (67.6%). A family history of rheumatic disease was reported in 17.2% of patients and was associated with early disease onset (P < 0.005). CONCLUSION: In this nationwide register, a descriptive classification of patients with disease manifestations characteristic of SSc in five groups allows to include a broader spectrum of patients with features of SSc.

[Health-related quality of life in children and adolescents with juvenile idiopathic arthritis: which role do age, sex and medical parameters play?]. / Gesundheitsbezogene Lebensqualität von Kindern und Jugendlichen mit juveniler idiopathischer Arthritis: Welche Rolle spielen Alter, Geschlecht und medizinische Parameter?

BACKGROUND: The assessment of health-related quality of life gains increasing importance in pediatric practice. In the field of rheumatic health conditions in young people only a few studies about this topic are available. Within a European study health-related quality of life of children and adolescents with idiopathic arthritis was assessed. The results of the German sample will be presented. PATIENTS: A total of N=88 children and adolescents with juvenile idiopathic arthritis (57% female) between 8 und 16 years were included in the study. METHOD: Children and adolescents filled in a questionnaire. Health-related quality of life was assessed with the DISABKIDS instrument. RESULTS: No age or gender effects on health-related quality of life were detected. The variable "limitation of mobility" as well as the item "pain" showed the strongest relationship with the health-related quality of life dimensions. CONCLUSIONS: Health-related quality of life is decisively influenced by the consequences of the health condition. For clinical practice the study showed that patients with mobility limitations and/or pain need heightened attention which should especially concentrate on the social and mental dimensions of health-related quality of life.