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Einleitung: In den letzten Jahren gewinnt die Sonographie aufgrund der besseren Verfügbarkeit beim Monitoring entzündlicher Myositiden gegenüber der MRT-Diagnostik zunehmend an Bedeutung. Insbesondere bei Kindern und Jugendlichen mit einer juvenilen Dermatomyositis (JDM) fehlt jedoch [zum vollständigen Text gelangen Sie über die oben angegebene URL]

Background: Pediatric sarcoidosis is a complex inflammatory disorder with multisystemic manifestations. Kidney involvement in children is rare, and prognostic factors are unknown. Case Report and Methods: We report the case of a 16-year-old girl with multiorgan sarcoidosis and renal involvement. The patient presented with tubulointerstitial nephritis, acute kidney injury (AKI), chest CT disseminated noduli, granulomatous iridocyclitis, giant-cell sialadenitis, and arthralgia. The kidney biopsy revealed non-granulomatous interstitial nephritis. Treatment consisted of initial high-dose methylprednisolone pulse followed by oral prednisolone and methotrexate. Full remission was achieved. In addition, we performed a literature review using PubMed and analyzed data on pediatric renal sarcoidosis cases. Results: We identified 36 cases of pediatric sarcoidosis with renal involvement on presentation and data on the end-of-follow-up glomerular filtration rate (GFR). The data from the literature review showed that renal involvement was slightly more prevalent in males (60%). AKI was present in most of the described patients (84%). Oral prednisolone was used in 35 of 36 cases; in more severe cases, other immunosuppressants were used. We newly identified renal concentration impairment and granulomatous interstitial nephritis as factors with a clear trend toward GFR loss at the end of follow-up, emphasizing the importance of kidney biopsy in symptomatic patients. In contrast, higher GFR at presentation and hypercalcemia were rather favorable factors. According to the identified predictive factors, our patient has a good prognosis and is in remission. Conclusion: The factors indicating a trend toward an unfavorable renal outcome in pediatric sarcoidosis are renal concentration impairment and granulomatous interstitial nephritis at presentation, while a higher GFR is beneficial.

Additional file 1: Age distribution at disease onset (PPTX 38 kb)

Additional file 2: NSAID and DMARD therapy in selected CNO cohorts. NPRD: National Pediatric Rheumatologic Database (PPTX 43 kb)

Additional file 1: Table S1. PRO-KIND overarching consensus statements for the management of CAPS/TRAPS/HIDS/MKD*. * The PRO-KIND statements were adapted from the SHARE recommendations for the management of autoinflammatory diseases [19]. Evidence levels were adapted from the Oxford Centre for Evidence-based Medicine levels of evidence and grades of recommendation [24]: 1A, Systematic reviews of randomized controlled trials; 1B, individual randomised controlled trial; 2A, systematic review of cohort studies; 2B, individual cohort study; 3B, individual case-control study, non-consecutive cohort study; 4, case series; 5, expert opinions. S, strength of recommendation: A, consistent level 1 studies; B, consistent level 2 or 3 studies or extrapolations from level 1 studies; C, level 4 studies or extrapolations from level 2 or 3 studies; D, level 5 evidence or troublingly inconsistent or inconclusive studies of any level. STIKO: German Permanent Vaccination Commission. 1 this recommendation was noticeably modified in comparison with the SHARE recommendations.2 regular checks should also be carried out with low or absent disease activity (see treat-to-target). 3 in patients with CAPS and HIDS/MKD, severe inflammatory responses have been reported especially to pneumococcal but also meningococcal vaccines [47, 61, 62].

Additional file 2: Table S2. PRO-KIND disease-specific consensus statements for diagnosis and management of CAPS/TRAPS/HIDS/MKD *. * The PRO-KIND statements were adapted from the SHARE recommendations for the management of autoinflammatory diseases [19]. Evidence levels were adapted from the Oxford Centre for Evidence-based Medicine levels of evidence and grades of recommendation [24]: 1A, Systematic reviews of randomized controlled trials; 1B, individual randomised controlled trial; 2A, systematic review of cohort studies; 2B, individual cohort study; 3B, individual case-control study, non-consecutive cohort study; 4, case series; 5, expert opinions. S, strength of recommendation: A, consistent level 1 studies; B, consistent level 2 or 3 studies or extrapolations from level 1 studies; C, level 4 studies or extrapolations from level 2 or 3 studies; D, level 5 evidence or troublingly inconsistent or inconclusive studies of any level. 1 this recommendation was noticeably modified in comparison with the SHARE recommendations.

Background Systemic juvenile idiopathic arthritis (SJIA) is an autoinflammatory disease associated with chronic arthritis. Early diagnosis and effective therapy of SJIA is desirable, so that complications are avoided. The PRO-KIND initiative of the German Society for Pediatric Rheumatology (GKJR) aims to define consensus-based strategies to harmonize diagnostic and therapeutic approaches in Germany. Methods We analyzed data on patients diagnosed with SJIA from 3 national registries in Germany. Subsequently, via online surveys and teleconferences among pediatric rheumatologists with a special expertise in the treatment of SJIA, we identified current diagnostic and treatment approaches in Germany. Those were harmonized via the formulation of statements and, supported by findings from a literature search. Finally, an in-person consensus conference using nominal group technique was held to further modify and consent the statements. Results Up to 50% of patients diagnosed with SJIA in Germany do not fulfill the International League of Associations for Rheumatology (ILAR) classification criteria, mostly due to the absence of chronic arthritis. Our findings suggest that chronic arthritis is not obligatory for the diagnosis and treatment of SJIA, allowing a diagnosis of probable SJIA. Malignant, infectious and hereditary autoinflammatory diseases should be considered before rendering a diagnosis of probable SJIA. There is substantial variability in the initial treatment of SJIA. Based on registry data, most patients initially receive systemic glucocorticoids, however, increasingly substituted or accompanied by biological agents, i.e. interleukin (IL)-1 and IL-6 blockade (up to 27.2% of patients). We identified preferred initial therapies for probable and definitive SJIA, including step-up patterns and treatment targets for the short-term (resolution of fever, decrease in C-reactive protein by 50% within 7 days), the mid-term (improvement in physician global and active joint count by at least 50% or a JADAS-10 score of maximally 5.4 within 4 weeks) and the long-term (glucocorticoid-free clinically inactive disease within 6 to 12 months), and an explicit treat-to-target strategy. Conclusions We developed consensus-based strategies regarding the diagnosis and treatment of probable or definitive SJIA in Germany. Electronic supplementary material The online version of this article (10.1186/s12969-018-0224-2) contains supplementary material, which is available to authorized users.

Expert opinion on individual management steps in various case scenarios reflecting the spectrum of systemic juvenile idiopathic arthritis. (DOCX 28 kb)

Characteristics of several widely available biomarkers of inflammation in active systemic juvenile idiopathic arthritis. (DOCX 53 kb)

Application of the various classification criteria to patients with systemic juvenile idiopathic arthritis in the German AID registry and the ICON-JIA cohort. (DOCX 22 kb)

Results from the online survey on diagnostic considerations and terminology in cases of possible systemic juvenile idiopathic arthritis. (DOCX 22Â kb)

Consensus process for the development of statements on the management of systemic juvenile idiopathic arthritis. AID-Net; autoinflammatory disease registry; ICON-JIA, inception cohort for patients with new-onset juvenile idiopathic arthritis; SJIA, systemic juvenile idiopathic arthritis. (DOCX 21 kb)

Key components of clinical case scenarios used for the online survey. (DOCX 29 kb)

Background: Colchicine is the standard therapy for prophylaxis of attacks and amyloid deposition in patients with FMF. The aim of this study was to identify clinical parameters and biomarkers, which predict a colchicine dose increase in patients with FMF in the near future; therefore we analyzed, whether[for full text, please go to the a.m. URL], 45. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 31. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh), 27. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR)