Kompass Autoimmun最新文献

{"title":"Morbus Crohn: Vielversprechende Behandlungsoption eines neuen IL-23-Inhibitors auf dem Weg zur Zulassung","authors":"E. Schnoy","doi":"10.1159/000538956","DOIUrl":"https://doi.org/10.1159/000538956","url":null,"abstract":"Background: Many patients with moderately to severely active Crohn’s disease do not respond to available therapies or lose response over time. The GALAXI-1 study previously found that three intravenous guselkumab dosages showed superior clinical and endoscopic outcomes over placebo at week 12 in patients with moderately to severely active Crohn’s disease. We report the safety and efficacy of subcutaneous guselkumab maintenance regimens to week 48 in the GALAXI-1 study. Methods: We did a phase 2, randomised, multicentre, double-blind trial. Adult patients with moderately to severely active Crohn’s disease were randomly allocated with a computer-generated randomisation schedule to receive one of five treatment groups, with regimens consisting of an intravenous induction phase transitioning to a subcutaneous maintenance phase starting at week 12 in a treat-through design: (1) guselkumab 200→100 mg group (200 mg intravenous at weeks 0, 4, and 8, then 100 mg subcutaneous every 8 weeks; (2) guselkumab 600→200 mg group (600 mg intravenous at weeks 0, 4, and 8, then 200 mg subcutaneous every 4 weeks); (3) guselkumab 1200→200 mg group (1200 mg intravenous at weeks 0, 4, and 8, then 200 mg subcutaneous every 4 weeks); (4) ustekinumab group (approximately 6 mg/kg intravenous at week 0, then 90 mg subcutaneous every 8 weeks); or (5) placebo group (placebo induction followed by either placebo maintenance [for those with CDAI clinical response at week 12] or crossover to ustekinumab [for those without CDAI clinical response at week 12]). Endpoints assessed at week 48 included CDAI remission (CDAI score <150), endoscopic response (≥50% improvement from baseline in SES-CD or SES-CD score ≤2), and endoscopic remission (SES-CD score ≤2) in the primary efficacy analysis population of all randomised patients who received at least one dose of study drug, excluding those discontinued during a temporary study pause. Safety analyses included all randomised patients who received at least one study drug dose. This trial is registered at Clinical Trials.gov (NCT03466411) and is active but not recruiting. Findings: Among 700 patients screened, 309 (112 biologic-naive; 197 biologic-experienced) were included in the primary efficacy analysis population: 61 in the guselkumab 200→100 mg group, 63 in the guselkumab 600→200 mg group, 61 in the guselkumab 1200→200 mg group, 63 in the ustekinumab group, and 61 in the placebo group. 126 (41%) women and 183 (59%) men were included, with median age 36·0 years (IQR 28·0-49·0). At week 48, the numbers of patients with CDAI clinical remission were 39 (64%) in the guselkumab 200→100 mg group, 46 (73%) in the guselkumab 600→200 mg group, 35 (57%) in the guselkumab 1200→200 mg group, and 37 (59%) in the ustekinumab group. The corresponding numbers of patients with endoscopic response were 27 (44%), 29 (46%), 27 (44%), and 19 (30%), respectively, and endoscopic remission was seen in 11 (18%), 11 (17%), 20 (33%), and four (6%) patients, respe","PeriodicalId":17887,"journal":{"name":"Kompass Autoimmun","volume":"3 4","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140653700","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Moderne Immunologie hat unsere Behandlungsoptionen stark verändert","authors":"M. Sticherling","doi":"10.1159/000538957","DOIUrl":"https://doi.org/10.1159/000538957","url":null,"abstract":"","PeriodicalId":17887,"journal":{"name":"Kompass Autoimmun","volume":"11 17","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140658050","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Behandlung der eosinophilen Granulomatose mit Polyangiitis: Ist Benralizumab eine Alternative?","authors":"P. Xanthouli","doi":"10.1159/000538827","DOIUrl":"https://doi.org/10.1159/000538827","url":null,"abstract":"Background: Benralizumab is effective in the treatment of eosinophilic asthma and is being investigated for the treatment of other eosinophil-associated diseases. Reports on the use of benralizumab for the treatment of eosinophilic granulomatosis with polyangiitis (EGPA) are limited to case reports and small case series. Methods: We conducted a multicentre, retrospective study including EGPA patients treated with off-label benralizumab. The primary endpoint was the rate of complete response defined as no disease activity (Birmingham Vasculitis Activity Score=0) and a prednisone dose ≤4 mg/day. Partial response was defined as no disease activity and a prednisone dose ≥4 mg/day. Results: Sixty-eight patients were included, including 31 (46%) who had previously received mepolizumab. The use of benralizumab was warranted by uncontrolled asthma in 54 (81%), persistent ear, nose and throat (ENT) manifestations in 27 (40%) and persistent glucocorticoids (GCs) use in 48 (74%) patients. Median (IQR) follow-up after starting benralizumab was 23 (9–34) months. Thirty-three patients (49%) achieved a complete response, 24 (36%) achieved a partial response and 10 (15%) did not respond. Among the 57 patients who initially responded, 10 (18%) eventually required further line treatments. GCs were discontinued in 23 patients (38%). Prior mepolizumab use was associated with a higher rate of primary failure (26.7% vs 5.4%, p=0.034) and less frequent GCs discontinuation (14.8% vs 55.9%, p=0.001). Vasculitis flares occurred in 7 patients (11%) and were associated with histological evidence of vasculitis and/or antineutrophil cytoplasmic antibodies positivity at benralizumab initiation (p=0.004).","PeriodicalId":17887,"journal":{"name":"Kompass Autoimmun","volume":" 9","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140686606","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Treat hard and early in SSc-ILD: milde ILD ist immer noch ILD","authors":"P. Xanthouli","doi":"10.1159/000538830","DOIUrl":"https://doi.org/10.1159/000538830","url":null,"abstract":"Background: Interstitial lung disease (ILD) is the leading cause of death in systemic sclerosis (SSc). According to expert statements, not all SSc-ILD patients require pharmacological therapy. Objectives: To describe disease characteristics and disease course in untreated SSc-ILD patients in two well characterised SSc-ILD cohorts. Methods: Patients were classified as treated if they had received a potential ILD-modifying drug. ILD progression in untreated patients was defined as (1) decline in forced vital capacity (FVC) from baseline of ≥10% or (2) decline in FVC of 5%–9% associated with a decline in diffusing capacity for carbon monoxide (DLCO)≥15% over 12 ± 3 months or (3) start of any ILD-modifying treatment or (4) increase in the ILD extent during follow-up. Multivariable logistic regression was performed to identify factors associated with non-prescription of ILD-modifying treatment at baseline. Prognostic factors for progression in untreated patients were tested by multivariate Cox regression. Results: Of 386 SSc-ILD included patients, 287 (74%) were untreated at baseline. Anticentromere antibodies (OR: 6.75 (2.16–21.14), p=0.001), limited extent of ILD (OR: 2.39 (1.19–4.82), p=0.015), longer disease duration (OR: 1.04 (1.00–1.08), p=0.038) and a higher DLCO (OR: 1.02 (1.01–1.04), p=0.005) were independently associated with no ILD-modifying treatment at baseline. Among 234 untreated patients, the 3 year cumulative incidence of progression was 39.9% (32.9–46.2). Diffuse cutaneous SSc and extensive lung fibrosis independently predicted ILD progression in untreated patients. Conclusion: As about 40% of untreated patients show ILD progression after 3 years and effective and safe therapies for SSc-ILD are available, our results support a change in clinical practice in selecting patients for treatment.","PeriodicalId":17887,"journal":{"name":"Kompass Autoimmun","volume":"8 7","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140710766","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Morbus Crohn: Top-down-Behandlungsschema erreicht substanziell höhere Remissionsraten, jedoch unabhängig von einem vorgeschlagenen Biomarker","authors":"B. Bengsch","doi":"10.1159/000538712","DOIUrl":"https://doi.org/10.1159/000538712","url":null,"abstract":"Background: Management strategies and clinical outcomes vary substantially in patients newly diagnosed with Crohn’s disease. We evaluated the use of a putative prognostic biomarker to guide therapy by assessing outcomes in patients randomised to either top-down (ie, early combined immunosuppression with infliximab and immunomodulator) or accelerated step-up (conventional) treatment strategies. Methods: PROFILE (PRedicting Outcomes For Crohn’s disease using a moLecular biomarker) was a multicentre, open-label, biomarker-stratified, randomised controlled trial that enrolled adults with newly diagnosed active Crohn’s disease (Harvey-Bradshaw Index ≥7, either elevated C-reactive protein or faecal calprotectin or both, and endoscopic evidence of active inflammation). Potential participants had blood drawn to be tested for a prognostic biomarker derived from T-cell transcriptional signatures (PredictSURE-IBD assay). Following testing, patients were randomly assigned, via a secure online platform, to top-down or accelerated step-up treatment stratified by biomarker subgroup (IBDhi or IBDlo), endoscopic inflammation (mild, moderate, or severe), and extent (colonic or other). Blinding to biomarker status was maintained throughout the trial. The primary endpoint was sustained steroid-free and surgery-free remission to week 48. Remission was defined by a composite of symptoms and inflammatory markers at all visits. Flare required active symptoms (HBI ≥5) plus raised inflammatory markers (CRP > upper limit of normal or faecal calprotectin ≥200 μg/g, or both), while remission was the converse – ie, quiescent symptoms (HBI <5) or resolved inflammatory markers (both CRP ≤ the upper limit of normal and calprotectin <200 μg/g) or both. Analyses were done in the full analysis (intention-to-treat) population. The trial has completed and is registered (ISRCTN11808228). Findings: Between Dec 29, 2017, and Jan 5, 2022, 386 patients (mean age 33.6 years [SD 13.2]; 179 [46%] female, 207 [54%] male) were randomised: 193 to the top-down group and 193 to the accelerated step-up group. Median time from diagnosis to trial enrolment was 12 days (range 0–191). Primary outcome data were available for 379 participants (189 in the top-down group; 190 in the accelerated step-up group). There was no biomarker-treatment interaction effect (absolute difference 1 percentage points, 95% CI –15 to 15; p = 0.944). Sustained steroid-free and surgery-free remission was significantly more frequent in the top-down group than in the accelerated step-up group (149 [79%] of 189 patients vs 29 [15%] of 190 patients, absolute difference 64 percentage points, 95% CI 57 to 72; p < 0.0001). There were fewer adverse events (including disease flares) and serious adverse events in the top-down group than in the accelerated step-up group (adverse events: 168 vs 315; serious adverse events: 15 vs 42), with fewer complications requiring abdominal surgery (one vs ten) and no difference in serious infection","PeriodicalId":17887,"journal":{"name":"Kompass Autoimmun","volume":"23 s1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140715244","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Symptomatische Erhöhung der Kreatinphosphokinase bei einer Patientin mit Morbus Crohn, verursacht durch Upadacitinib","authors":"Anna Schuitema, Suzanne I. Anjie, Agnies M. van Eeghen, Sander W. Tas, Mark Löwenberg","doi":"10.1159/000538605","DOIUrl":"https://doi.org/10.1159/000538605","url":null,"abstract":"Wir stellen eine Patientin mit Morbus Crohn vor, die während der Behandlung mit hochdosiertem Upadacitinib erhöhte Kreatinphosphokinase-Werte und eine Myopathie zeigte, und bieten dem Leser praktische Tipps zur Unterbrechung und erneuten Verarbeichung von Upadacitinib, wobei die Notwendigkeit einer angemessenen Überwachung zu betonen ist.","PeriodicalId":17887,"journal":{"name":"Kompass Autoimmun","volume":"28 6","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140714562","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Rheumatoide Arthritis: Langzeitverläufe haben sich seit Einführung von Biologika und gezielter Therapie verbessert","authors":"S. Adler","doi":"10.1159/000538592","DOIUrl":"https://doi.org/10.1159/000538592","url":null,"abstract":"Background: Advances in rheumatoid arthritis (RA) treatment, highlighted by biological disease-modifying antirheumatic drugs (bDMARDs) and targeted synthetic DMARDs (tsDMARDs), have altered the paradigm of RA treatment in the last decade. Therefore, real-world clinical evidence is needed to understand how treatment strategies and outcomes have changed. Methods: Using an observational cohort of RA from 2012 to 2021, we collected cross-sectional data of RA patients annually to analyze a trend in RA management. For patients who initiated b/tsDMRDs, we evaluated treatment outcomes between b/tsDMARDs. Mixed-effect models were applied to examine the statistical implications of changes over time in treatment outcomes with a background adjustment. Results: We analyzed annual cross-sectional data from 5070 patients and longitudinal data from 1816 patients in whom b/tsDMARDs were initiated between 2012 and 2021. b/tsDMARD use increased, whereas glucocorticoid use decreased from 2012 to 2021. Disease activity and functional disability measures improved over time. The percentage of tsDMARD prescriptions considerably increased. All b/tsDMARDs showed clinical improvements in disease activity and functional disability. Statistically, TNFi showed better short-term improvements in b/tsDMARD-naïve patients, while IL6Ri demonstrated significant long-term benefits. IL6Ri had better retention rates in switched patients. After adjustment for patient characteristics, the annual change of RA disease activity and functional disability fared significantly better from 2012 to 2021. Conclusions: With the development of new RA therapeutics, overall treatment outcomes advanced in the past decade.","PeriodicalId":17887,"journal":{"name":"Kompass Autoimmun","volume":"3 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140715488","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Birmekizumab: Erweitert die Therapieoptionen besonders bei axialer Spondylarthritis","authors":"P. Sewerin","doi":"10.1159/000538713","DOIUrl":"https://doi.org/10.1159/000538713","url":null,"abstract":"Objectives: Bimekizumab (BKZ), a monoclonal IgG1 antibody that selectively inhibits interleukin (IL)-17F in addition to IL-17A, has demonstrated superior efficacy versus placebo in patients with non-radiographic (nr-) and radiographic (r-) axial spondyloarthritis (axSpA) at Week 16. Here, the objective is to report the efficacy and safety of BKZ at Week 52. Methods: BE MOBILE 1 (nr-axSpA; NCT03928704) and BE MOBILE 2 (r-axSpA; NCT03928743) comprised a 16-week, double-blind, placebo-controlled period, then a 36-week maintenance period. From Week 16, all patients received subcutaneous BKZ 160 mg every 4 weeks. Results: Improvements versus placebo in Assessment of SpondyloArthritis International Society ≥40% response (primary endpoint), Ankylosing Spondylitis Disease Activity Score, high-sensitivity C-reactive protein levels and MRI inflammation of the sacroiliac joints/spine at Week 16 were sustained to Week 52 in BKZ-randomised patients. At Week 52, responses of patients switching from placebo to BKZ at Week 16 were comparable to BKZ-randomised patients. At Week 52, ≥1 treatment-emergent adverse events (TEAEs) were reported in 183 (75.0%) and 249 (75.5%) patients with nr-axSpA and r-axSpA, respectively. Serious TEAEs occurred in 9 (3.7%) patients with nr-axSpA and 20 (6.1%) patients with r-axSpA. Oral candidiasis was the most frequent fungal infection (nr-axSpA: 18 (7.4%); r-axSpA: 20 (6.1%)). Uveitis occurred in three (1.2%) and seven (2.1%) patients with nr-axSpA and r-axSpA, and inflammatory bowel disease in two (0.8%) and three (0.9%).","PeriodicalId":17887,"journal":{"name":"Kompass Autoimmun","volume":"65 4","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140713572","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Biologische Therapie bei systemischem Lupus erythematodes, Antiphospholipid-Syndrom und Sjögren-Syndrom: evidenz- und praxisbasierte Leitlinien","authors":"António Marinho, José Delgado Alves, Jorge Fortuna, Raquel Faria, Isabel Almeida, Glória Alves, João Araújo Correia, Ana Campar, Mariana Brandão, Jorge Crespo, Daniela Marado, João Matos-Costa, S. Oliveira, Fernando Salvador, Lelita Santos, Fátima Silva, Milene Fernandes, Carlos Vasconcelos","doi":"10.1159/000538603","DOIUrl":"https://doi.org/10.1159/000538603","url":null,"abstract":"Systemischer Lupus erythematodes (SLE), das Antiphospholipid-Syndrom (APS) und das Sjögren-Syndrom (SS) sind heterogene Autoimmunerkrankungen. Schwere Ausprägungen sowie Therapieresistenz bzw. -unverträglichkeit gegenüber herkömmlichen Immunsuppressiva erfordern andere Behandlungsoptionen, d.h. biologische Arzneimittel und kleine Moleküle. Unser Ziel war es, evidenz- und praxisbasierte Leitlinien für die zulassungsüberschreitende Anwendung von Biologika bei SLE, APS und SS zu definieren. Die Empfehlungen wurden nach einem umfassenden Literaturreview und 2 Konsensrunden durch ein unabhängiges Expertengremium abgegeben. Das Gremium umfasste 17 Experten für Innere Medizin mit anerkannter Praxis im Bereich der Behandlung von Autoimmunerkrankungen. Die Literaturrecherche erfolgte systematisch für die Jahre von 2014 bis 2019 und wurde später durch Querverweisprüfungen und Experteninformationen bis 2021 aktualisiert. Es wurden vorläufige Empfehlungen von Arbeitsgruppen für jede Krankheit erarbeitet. Ein Revisionsmeeting mit allen Experten fand vor dem Konsensmeeting im Juni 2021 statt. Alle Experten stimmten in 2 Runden ab (stimme zu, stimme nicht zu, stimme weder zu noch widerspreche ich), und Empfehlungen mit mindestens 75% Zustimmung wurden anerkannt. Insgesamt 32 abschließende Empfehlungen (20 für die SLE-, 5 für die APS- und 7 für die SS-Behandlung) wurden von den Experten anerkannt. Diese Empfehlungen berücksichtigen die Organbeteiligung, die Ausprägung, den Schweregrad und das Ansprechen auf frühere Behandlungen. Bei diesen 3 Autoimmunkrankheiten beziehen sich die meisten Empfehlungen auf Rituximab, was auf die höhere Anzahl von Studien und der klinischen Erfahrung mit diesem biologischen Wirkstoff zurückzuführen ist. Eine sequenzielle Behandlung mit Belimumab nach Rituximab kann auch bei schweren Fällen von SLE und SS indiziert sein. Eine Zweitlinientherapie mit Baricitinib, Bortezomib, Eculizumab, Secukinumab oder Tocilizumab kann bei SLE-spezifischen Ausprägungen erwogen werden. Diese evidenz- und praxisbasierten Empfehlungen können die Behandlungsentscheidung unterstützen und letztendlich das Behandlungsergebnis bei Patienten mit SLE, APS oder SS verbessern.","PeriodicalId":17887,"journal":{"name":"Kompass Autoimmun","volume":"9 7","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140713034","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Kutanes mikrovaskuläres Okklusionssyndrom als erste Manifestation eines katastrophalen Lupus-assoziierten Antiphospholipid-Antikörper-Syndroms: ein Fallbericht","authors":"Nastaran-Sadat Hosseini, S. Babaei, Hamid Rahimi, Alaleh Gheissari, Banafsheh Sedaghat, Mahsa Pourmahdi-Boroujeni, Bahareh Abtahi-Naeini","doi":"10.1159/000535813","DOIUrl":"https://doi.org/10.1159/000535813","url":null,"abstract":"Hintergrund: Das Antiphospholipid-Syndrom (APS), das durch thrombotische Ereignisse oder geburtshilfliche Komplikationen bei anhaltend hohen Antiphospholipid-Antikörpern definiert ist, zeichnet sich durch eine Vielzahl von klinischen Erscheinungsformen aus, und die Auswirkungen des Gefäßverschlusses können nahezu jedes Organsystem oder Gewebe betreffen. Da die Klassifizierungskriterien für das APS bei Erwachsenen in der Pädiatrie (wo schwangerschaftsbedingte Probleme selten sind) nicht gut verifiziert sind, ist die Schätzung der Prävalenz im Kindesalter schwierig. Schlaganfall und Lungenembolie sind thromboembolische Ereignisse, die bei Kindern auftreten und eine erhebliche Langzeitmorbidität verursachen können. Kinder mit APS sind anfälliger für wiederkehrende Thromboembolien als Erwachsene. Die kutanen Symptome stehen im Vordergrund und sind in der Regel der erste Hinweis auf ein APS. Obwohl dermatologische Befunde äußerst heterogen sind, ist es wichtig zu überlegen, welche dermatologischen Symptome die Untersuchung auf ein APS und die erforderliche weitere Behandlung rechtfertigen. Falldarstellung: Wir beschreiben einen 7-jährigen iranischen Jungen mit retiformer Purpura und akralen kutanen ischämischen Läsionen als erste klinische Präsentation des APS im Rahmen eines systemischen Lupus erythematodes.","PeriodicalId":17887,"journal":{"name":"Kompass Autoimmun","volume":"19 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-01-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139525323","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}