Update of EULAR recommendations for the treatment of systemic sclerosis

The aim was to update the 2009 European League against Rheumatism (EULAR) recommendations for the treatment of systemic sclerosis (SSc), with attention to new therapeutic questions. Update of the previous treatment recommendations was performed according to EULAR standard operating procedures. The task force consisted of 32 SSc clinical experts from Europe and the USA, 2 patients nominated by the pan-European patient association for SSc (Federation of European Scleroderma Associations (FESCA)), a clinical epidemiologist and 2 research fellows. All centres from the EULAR Scleroderma Trials and Research group were invited to submit and select clinical questions concerning SSc treatment using a Delphi approach. Accordingly, 46 clinical questions addressing 26 different interventions were selected for systematic literature review. The new recommendations were based on the available evidence and developed in a consensus meeting with clinical experts and patients. The procedure resulted in 16 recommendations being developed (instead of 14 in 2009) that address treatment of several SSc-related organ complications: Raynaud's phenomenon (RP), digital ulcers (DUs), pulmonary arterial hypertension (PAH), skin and lung disease, scleroderma renal crisis and gastrointestinal involvement. Compared with the 2009 recommendations, the 2016 recommendations include phosphodiesterase type 5 (PDE-5) inhibitors for the treatment of SSc-related RP and DUs, riociguat, new aspects for endothelin receptor antagonists, prostacyclin analogues and PDE-5 inhibitors for SSc-related PAH. New recommendations regarding the use of fluoxetine for SSc-related RP and haematopoietic stem cell transplantation for selected patients with rapidly progressive SSc were also added. In addition, several comments regarding other treatments addressed in clinical questions and suggestions for the SSc research agenda were formulated. These updated data-derived and consensus-derived recommendations will help rheumatologists to manage patients with SSc in an evidence-based way. These recommendations also give directions for future clinical research in SSc

ICAR: endoscopic skull‐base surgery

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Racial differences in systemic sclerosis disease presentation: a European Scleroderma Trials and Research group study

Objectives. Racial factors play a significant role in SSc. We evaluated differences in SSc presentations between white patients (WP), Asian patients (AP) and black patients (BP) and analysed the effects of geographical locations.Methods. SSc characteristics of patients from the EUSTAR cohort were cross-sectionally compared across racial groups using survival and multiple logistic regression analyses.Results. The study included 9162 WP, 341 AP and 181 BP. AP developed the first non-RP feature faster than WP but slower than BP. AP were less frequently anti-centromere (ACA; odds ratio (OR) = 0.4, P < 0.001) and more frequently anti-topoisomerase-I autoantibodies (ATA) positive (OR = 1.2, P = 0.068), while BP were less likely to be ACA and ATA positive than were WP [OR(ACA) = 0.3, P < 0.001; OR(ATA) = 0.5, P = 0.020]. AP had less often (OR = 0.7, P = 0.06) and BP more often (OR = 2.7, P < 0.001) diffuse skin involvement than had WP.AP and BP were more likely to have pulmonary hypertension [OR(AP) = 2.6, P < 0.001; OR(BP) = 2.7, P = 0.03 vs WP] and a reduced forced vital capacity [OR(AP) = 2.5, P < 0.001; OR(BP) = 2.4, P < 0.004] than were WP. AP more often had an impaired diffusing capacity of the lung than had BP and WP [OR(AP vs BP) = 1.9, P = 0.038; OR(AP vs WP) = 2.4, P < 0.001]. After RP onset, AP and BP had a higher hazard to die than had WP [hazard ratio (HR) (AP) = 1.6, P = 0.011; HR(BP) = 2.1, P < 0.001].Conclusion. Compared with WP, and mostly independent of geographical location, AP have a faster and earlier disease onset with high prevalences of ATA, pulmonary hypertension and forced vital capacity impairment and higher mortality. BP had the fastest disease onset, a high prevalence of diffuse skin involvement and nominally the highest mortality

International Consensus Statement on Rhinology and Allergy: Rhinosinusitis

Background: The 5 years since the publication of the first International Consensus Statement on Allergy and Rhinology: Rhinosinusitis (ICAR‐RS) has witnessed foundational progress in our understanding and treatment of rhinologic disease. These advances are reflected within the more than 40 new topics covered within the ICAR‐RS‐2021 as well as updates to the original 140 topics. This executive summary consolidates the evidence‐based findings of the document. Methods: ICAR‐RS presents over 180 topics in the forms of evidence‐based reviews with recommendations (EBRRs), evidence‐based reviews, and literature reviews. The highest grade structured recommendations of the EBRR sections are summarized in this executive summary. Results: ICAR‐RS‐2021 covers 22 topics regarding the medical management of RS, which are grade A/B and are presented in the executive summary. Additionally, 4 topics regarding the surgical management of RS are grade A/B and are presented in the executive summary. Finally, a comprehensive evidence‐based management algorithm is provided. Conclusion: This ICAR‐RS‐2021 executive summary provides a compilation of the evidence‐based recommendations for medical and surgical treatment of the most common forms of RS

Endoscopic, transnasal approach to the orbital tumors using image-guided neuronavigation system

Histopathological diagnosis of the intraorbital tumors is of crucial value for planning further conservative or surgical treatment. Small intraorbital lesions located posteriorly to the bulb, intraconally or in the orbital apex are extremely difficult to reach via traditional external approach. Precise fine needle aspiration in these cases is not easy and a small amount of examined material may lead to misdiagnosis. The aim of the study was to evaluate the usefulness of nasal endoscopy and image guided neuronavigation system in the diagnosis of orbital tumors. Seven patients with intraorbital tumors were diagnosed. All the lesions were precisely localized and the biopsy was succesfully taken using transnasal endoscopy supported by neuronavigation system. None of the patients developped ocular symptoms aggravation after the procedure. Neuronavigation system facilitated precise localization of the lesion, helped to protect vulnerable orbital structures and decreased the risk of complications

Einfluss des Eingriffes an der Nase auf die Ergebnisse der UPPP bei OSA

Einleitung: Die Rolle der Nasendurchgängigkeit in der Pathogenese der obstruktiven Schlafapnoe (OSA) ist nicht vollständig geklärt. Das Ziel der Arbeit war, die Einwirkung des chirurgischen Verkleinerung des nasalen Widerstands auf die Ergebnisse der Uvulopalatopharyngoplastik (UPPP) bei OSA.Material und Methode: Für die Untersuchungen wurden 40 Patienten mit Atemstörungen beim Schlaf gewählt, bei denen die Anzahl der Störungen in der Untersuchung vor der Operation nicht 30 überschritten hat (RDI<30) sowie deren BMI<30 ist. Bei 20 Patienten (Gruppe I) hat man eine erhebliche Behinderung der Durchgängigkeit von mindestens einem Nasengang. Bei allen an den Untersuchungen teilnehmenden Patienten hat man UPPP gemacht, bei den Patienten der I. Gruppe hat man zusätzlich Eingriffe durchgeführt, die die behinderte Nasenluftpassage korrigiert haben.Ergebnisse: Der Vergleich der Ergebnisse der chirurgischen Behandlung beider Gruppen hat ergeben, dass man bei Patienten, bei denen gleichzeitig die Nasendurchgängigkeit verbessert wurde, eine höhere Reduktion von RDI erreicht wurde. Diese Patienten haben auch weniger geschnarcht, als die Patienten, bei denen man nur UPPP (Gruppe II) durchgeführt hat.Schlussfolgerungen: Unsere Untersuchungsergebnisse bestätigen die Meinung, dass die Störung von Nasendurchgängigkeit nicht der Hauptfaktor für OSA ist, sondern nur eine Begleiterscheinung