BACKGROUND:Glomerulonephritis is a common, organ-threatening manifestation in ANCA-associated vasculitis (AAV). Various clinico-histological scores have been developed assessing the risk of kidney failure, including the simplified ANCA kidney risk score (AKRiS). METHODS:This retrospective study included 220 patients with biopsy-confirmed kidney involvement of granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) recruited from four tertiary European referral centers. We collected biometric and clinical baseline data, disease activity parameters, AKRiS classes and histomorphological features. We examined the occurrence of a composite endpoint (ie all-cause mortality or kidney failure defined as persistent estimated glomerular filtration rate [eGFR] decline <15 mL/min/1.73 m² or kidney replacement therapy after ≥6 months, respectively) within individual AKRiS and precursor risk classes. RESULTS:GPA and MPA patients exhibited largely comparable baseline characteristics and treatment regimens. Histological analysis revealed that MPA biopsies showed significantly more sclerosis, interstitial fibrosis and tubular atrophy (IFTA) than their GPA counterparts. Histologically, most patients fell into focal and mixed classes, which mainly transitioned into low- and medium-risk AKRiS categories. Higher risk classifications predominantly derived from crescentic and sclerotic classes. Patients categorized as medium risk in the precursor score most frequently transitioned to lower AKRiS scores. AKRiS stratification was superior to the ANCA Renal Risk Score (ARRS) in the prediction of adverse outcomes over the entire period (AUC 0.76, C-Index 0.899). CONCLUSIONS:The AKRiS score was found to be effective in predicting kidney failure and a strong composite kidney endpoint in a large European AAV cohort, despite slight differences in histological and event patterns between GPA and MPA under comparable treatment regimens.

BACKGROUND/OBJECTIVES:Imaging is central to the diagnosis of giant cell arteritis (GCA). However, direct comparisons between magnetic resonance imaging (MRI) and ultrasound remain limited, particularly regarding vascular involvement patterns and their combined diagnostic value. This study compared MRI and ultrasound in suspected GCA and evaluated whether their combination improves diagnostic accuracy. METHODS:We retrospectively analyzed 183 patients with suspected GCA evaluated at a tertiary care center between July 2017 and October 2022. MRI was performed in all patients using a graded sum score across multiple cranial and extracranial vessel territories. Ultrasound was performed in 159 patients (87 %) using a binary sum score assessing temporal, common carotid, and subclavian/axillary arteries. Vessel inflammation patterns were characterized across both modalities, and diagnostic performance was assessed using receiver operating characteristic curve analysis. RESULTS:GCA was diagnosed in 101 patients (55 %). Bilateral vessel involvement distinguished GCA from non-GCA and extended beyond subclavian/axillary arteries to include temporal, occipital, and vertebral territories. MRI achieved strong diagnostic accuracy (AUC 0.90; sensitivity 0.88; specificity 0.85), as did ultrasound (AUC 0.89; sensitivity 0.84; specificity 0.92). Cross-modality correlations were strongest for temporal arteries (r = 0.52). When MRI and ultrasound findings were concordant (82 % of cases), diagnostic performance improved further (AUC 0.94; sensitivity 0.96; specificity 0.92). CONCLUSION:MRI and ultrasound each provide high diagnostic accuracy for GCA while capturing complementary patterns of vascular inflammation. Bilateral involvement across multiple vascular territories is a defining disease feature. Concordant multimodal imaging substantially enhances diagnostic confidence and supports a combined imaging approach in patients with suspected GCA.

Zusammenfassung Die Kontrastmittel-verstärkte Sonografie (CEUS) ist in der Lage, auch kleine und langsame Blutflüsse zu detektieren. Sie kommt daher zur Beurteilung von Vaskularisation und zur semiquantitativen Beurteilung von Entzündungsprozessen auch in der Rheumatologie zum Einsatz. Wir fassen mit der vorliegenden Arbeit die derzeit verfügbare Literatur zur CEUS in der Rheumatologie zusammen und fokussieren uns dabei auf den Einsatz bei Großgefäßvaskulitiden und Arthritiden. Die bis dato publizierten Daten zeigen eine überwiegend gute Korrelation der Kontrastmittelaufnahme in Synovia bzw. Gefäßwand mit klinischen, laborchemischen und teils auch bildgebenden Befunden. Fehlende Daten über Informationszugewinn zu konventionellen Methoden der Entzündungsdarstellung und erhöhter Sachmittel-, Zeit- und Personalaufwand lassen die CEUS in der Rheumatologie derzeit noch keine Breitenanwendung erleben. Weitere Studien mit größeren Fallzahlen sind wünschenswert. Ziele sind die Validierung des Verfahrens und die Klärung der prognostischen Bedeutung positiver Befunde bei Patienten, die nach Einsatz bisheriger Mittel als in Remission befindlich betrachtet werden. Der potenzielle Nutzen der CEUS in der Rheumatologie liegt – wie in anderen Fachbereichen – in der Verbesserung und Präzisierung der Behandlung unserer Patienten, um irreversible Langzeitkomplikationen und kostspielige Großgeräte-Bildgebungen zu vermeiden.

KEY POINTS:Kidney involvement in patients with multiple myeloma has many different findings in kidney histology. Persistent dialysis requirement is a major cause of mortality. The rapid initiation of modern myeloma therapy is essential to prevent the need for dialysis and reduce mortality. BACKGROUND:Acute or CKD affects 20%-40% of patients with multiple myeloma (MM). While the negative prognostic effect of kidney involvement is established, the specific effect of different histological patterns remains poorly understood. We aimed to characterize the clinical outcomes associated with distinct kidney histologies in patients with MM. METHODS:We retrospectively analyzed 193 patients with MM who underwent kidney biopsy, comparing outcomes with 62 patients with MM without kidney disease. Patients were categorized by histological findings: cast nephropathy (CN, n =120), lightchain (AL) amyloidosis ( n =19), monoclonal Ig deposition disease (MIDD, n =8), and other renal disease ( n =46). We evaluated kidney function, dialysis requirements, treatment responses, and survival across histological subtypes and treatment modalities (conventional therapy versus modern agents including proteasome inhibitors, CD38 antibodies, and autologous stem cell transplantation). RESULTS:The CN and MIDD groups had the lowest median eGFR at biopsy (13.0 and 25.5 ml/min per 1.73 m 2 , respectively). Nephrotic-range proteinuria (>3.5 g/24 hours per Kidney Disease Improving Global Outcomes) was observed in the AL amyloidosis group (median 3910 mg/24 hours). KRT was initiated in 42.5% of patients. The proportion achieving KRT independence was 42.4% in the CN group and 50% in the other renal disease group, whereas none in the AL amyloidosis or MIDD groups recovered sufficient native kidney function. Modern combination therapy significantly improved renal outcomes, with 100% of patients receiving triple therapy showing renal response. KRT dependency was the strongest predictor of mortality: Patients remaining on KRT had significantly shorter survival compared with those never requiring KRT (12.7 versus 56.2 months, P < 0.001). The extent of improvement in kidney function did not influence survival; only dialysis status was prognostically relevant. CONCLUSIONS:Kidney histology determines distinct clinical trajectories in patients with MM, with CN showing potential for dialysis reversibility. Modern combination therapies significantly improve renal outcomes. The requirement for ongoing KRT was the primary predictor of overall survival.

To assess for the first time a combination of oscillometric, greyscale- and novel color-Doppler ultrasound (US) indices of carotid and aortic damage in patients with primary Sjögren's syndrome (pSS). Moreover, to examine associations of these markers with patient and disease-characteristics, as well as with a traditional cardiovascular (CV) risk score (SCORE) and its EULAR-modified version (mSCORE). Greyscale and color-Doppler indices [resistance (RI)- and pulsatility (PI)-index], as well as markers of atherosclerosis [Intima-Media-Thickness (cIMT), plaques, and cumulative calcification surface], were examined in the common- (CCA) and internal- (ICA) carotid arteries of pSS patients and healthy controls. The gold standard oscillometric marker of aortic stiffness (carotid-femoral pulse wave velocity; cfPWV) and the traditional SCORE/mSCORE, were also assessed. We recruited 119 pSS-patients and 97 controls. Patients exhibited significantly higher cfPWV (padj = 0.025), cIMT (padj < 0.001), and calcification area (p = 0.013), compared to controls. According to mSCORE, 5.7% of the patients had high CV risk. However, cfPWV and carotid-sonography revealed increased aortic stiffness in 45.4% and carotid atherosclerosis in 69.2%, respectively. Among pSS-patients, cfPWV correlated with C-reactive-protein (rho = 0.325, p < 0.001), erythrocyte-sedimentation-rate (rho = 0.271, p = 0.003), and traditional CV-risk factors (age, cholesterol, systolic blood pressure: all; p < 0.01). ICA-RI and ICA-PI were higher in patients with further (non-rheumatological) autoimmune diseases (both; p < 0.05). In the largest cfPWV/US-cohort examined to date, pSS-patients had significantly higher aortic stiffness and atherosclerosis than controls. Aortic stiffness was predicted by systemic inflammation, alongside traditional CV risk factors. cfPWV and carotid-US may help identify subclinical end-organ disease and atherosclerosis and thus assist CV/CVB-screening in pSS. DRKS00031470. .

This study compares the diagnostic performance of the 1990 American College of Rheumatology (ACR) criteria and the 2022 ACR/European League Against Rheumatism (EULAR) classification criteria for giant cell arteritis (GCA), with focus on the complementary roles of MRI and ultrasound imaging. A retrospective analysis was conducted on 192 patients presenting with suspected GCA at a tertiary care center between 2017 and 2022. Both classification criteria were applied to all cases, and their sensitivity, specificity, and diagnostic accuracy were compared. The diagnostic contribution of MRI versus ultrasound for bilateral axillary involvement was systematically analyzed. Among 192 patients, 99 were diagnosed with GCA. The 1990 ACR criteria demonstrated high specificity (0.96, 95

OBJECTIVES:To evaluate the combination of novel colour Doppler US (CDUS), greyscale US (GSUS), and oscillometric indices of macroangiopathy in patients with idiopathic inflammatory myopathies (IIM). Second, to explore the associations between these imaging markers and both patient-related and disease-related characteristics, as well as traditional cardiovascular (CV) risk factors. METHODS:We conducted CDUS to evaluate arterial compliance markers, specifically the resistance (RI) and pulsatility (PI) indices, both in the common (CCA) and internal carotid arteries (ICA) of patients with IIM and healthy controls. Additionally, we performed GSUS examinations to measure carotid intima-media thickness (cIMT), identify plaques, and quantify cumulative carotid calcification surface. Oscillometric assessments determined aortic stiffness using carotid-femoral pulse wave velocity (cfPWV). RESULTS:We recruited 82 IIM patients and 88 healthy controls. Patients showed significantly higher cIMT (Padj = 0.032), CCA-RI (Padj = 0.015), CCA-PI (Padj = 0.013), ICA-RI (Padj = 0.012), and ICA-PI (Padj = 0.039), compared with controls. RI and PI of CCA and ICA were higher in patients with lower lung function vital capacity, respectively (all Ps < 0.05). cfPWV correlated positively with traditional CV risk factors including age (ρ = 0.546, P < 0.001), mean arterial pressure (ρ = 0.331, P = 0.003), diabetes (P = 0.007), and hyperlipidaemia (P = 0.032), and associated negatively with lung carbon monoxide (CO) diffusion (ρ = -0.329, P = 0.031). CONCLUSION:In one of the largest CV surrogate marker studies in IIM, patients exhibited increased carotid pulsatility, resistance, and atherosclerosis compared with controls. Lower lung function parameters predicted aortic stiffness and Doppler indices, suggesting a possible link between lung involvement and increased CV risk. Angiopathy markers may reveal significant vascular abnormalities in IIM patients, enhancing CV screening and risk classification.

Management of ANCA-associated vasculitis (AAV) has significantly improved, yet up to 40% of patients experience relapses, leading to worse long-term outcomes and organ damage. This multicenter cohort study investigated the prognostic value of histopathological patterns in renal AAV for predicting relapse risk and treatment response. We retrospectively analyzed 264 patients with newly diagnosed granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) with renal involvement recruited at four tertiary rheumatology and/or nephrology centers. Baseline clinical data, disease activity scores, and kidney biopsy findings were assessed. Patients were followed for at least twelve months. An overall relapse was defined as renewed disease activity requiring changes in maintenance therapy, initiation of a new induction regimen, or an increase in prednisone dosage > 10 mg/day. A severe relapse required new immunosuppressive induction therapy. After a median follow-up of twelve months the renal domain of the Birmingham Vasculitis Activity Score (BVAS) and its subcategories were evaluated. AAV patients with moderate-to-high (> 25%) interstitial fibrosis and tubular atrophy (IFTA) had a lower risk for both, overall and severe relapses. Severe relapses occurred more frequently in older and male patients. Furthermore, we identified glomerular sclerosis (≥ 50%) to be the strongest predictor for ongoing activity in the renal BVAS domain. Histopathological features do not only help to predict renal recovery and need for dialysis but also to forecast relapse risk and renal BVAS activity. Incorporating these patterns into clinical decision-making could enable more personalized therapy approaches, highlighting the need for prospective validation.

Pulmonale, renale und kardiovaskuläre Erkrankungen sowie die Osteoporose sind in der Behandlung von Patientinnen und Patienten mit entzündlich rheumatischen Erkrankungen immer zu berücksichtigen. Unabhängig davon, ob es sich um eine Organmanifestation der rheumatischen Grunderkrankung, eine Medikamentennebenwirkung oder eine Komorbidität handelt, haben diese Krankheiten einen ausschlaggebenden Einfluss auf Morbidität, Mortalität, aber auch therapeutische Entscheidungen. Bei diesem Beitrag handelt es sich um Teil 1 des CME-Beitrags. Sie erhalten Informationen über aktuelle diagnostische und therapeutische Empfehlungen zu pulmonalen und kardiovaskulären Komorbiditäten. Neben den neuesten ACR(American College of Rheumatology)/EULAR(European Alliance of Associations for Rheumatology)-Leitlinien zu kardiovaskulären Erkrankungen präsentieren wir Leitlinienempfehlungen sowie Einblicke in aktuelle Therapiestudien zu Diagnostik und Therapie häufiger pulmonaler Komorbiditäten bei entzündlich rheumatischen Erkrankungen.

ObjectiveGiant cell arteritis (GCA) is one of the most common forms of vasculitis.There is an abundance of studies which are conducted in a randomised controlled trial setting but limited with respect to cohort size and follow-up time.GeVas is the first large-scale registry for vasculitides in German-speaking countries that enables to evaluate this rare disease.Herein we focus on the subgroup of GCA patients including follow-up data up to one year. MethodsGeVas is a prospective, web-based, multicentre registry for the documentation of organ manifestations, outcomes, and therapy regimens in vasculitides.Recruitment started in June 2019.By April 2023, 15 centres were initiated and have started to enrol patients. ResultsAfter 4 years, 195 GCA-patients were included in the registry, of which 64% were female and 36% were male.The average age was 76 years at the time of recruitment (IQR=69-82).Seventy-nine percent were included in the registry because of a newly diagnosed GCA and 21% because of a relapse.At the first assessment most of the patients (89%) described general symptoms.Thirty-one percent stated ocular symptoms.Cranial symptoms were documented in 78% of the cases.All patients were documented with immunosuppressive treatment at start, of whom 95% received prednisolone, 16% cyclophosphamide, 20% methotrexate, and 48% tocilizumab.After three months 62% and after one year 91% of the patients achieved remission. Conclusion Regarding demographics, clinical manifestations and diagnostics, our study showed a similar composition comparedto other studies.However, our data differed in terms of treatment regimens.

OBJECTIVES:Prospective long-term observational data on the disease course of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) were missing in Germany to date. Therefore, the Joint Vasculitis Registry in German-speaking countries (GeVas) has been established to follow the course of patients with AAV. The aim of this study is to present baseline data of patients with newly diagnosed and relapsing AAV enrolled in the GeVas registry.METHODS:GeVas is a prospective, web-based, multicentre, clinician-driven registry for the documentation of organ manifestations, damage, long-term outcomes, and therapy regimens in various types of vasculitis. Recruitment started in June 2019.RESULTS:Between June 2019 and October 2022, 266 patients with AAV were included in the GeVas registry: 173 (65%) with new-onset and 93 (35%) with relapsing AAV. One hundred and sixty-two (61%) patients were classified as granulomatosis with polyangiitis (GPA), 66 (25%) as microscopic polyangiitis (MPA), 36 (13%) as eosinophilic granulomatosis with polyangiitis (EGPA), and 2 (1%) as renal limited AAV. The median age was 59 years (51-70 years, IQR), 130 (51%) patients were female. Most patients were ANCA positive (177; 67%) and affected by general symptoms, pulmonary, ear nose throat (ENT), renal and neurological involvement. For induction of remission, the majority of patients received glucocorticoids (247, 93%) in combination with either rituximab (118, 45%) or cyclophosphamide (112, 42%).CONCLUSIONS:Demographic characteristics are comparable to those in other European countries. Differences were found regarding ANCA status, frequencies of organ manifestations, and therapeutic regimens. The GeVas registry will allow longitudinal observations and prospective outcome measures in AAV.

To explore disease characteristics, renal involvement and induction treatment strategies over the last decades and evaluate relapse rates and renal outcomes in ANCA-associated vasculitides (AAV). We retrospectively analyzed remission, relapse rates and the occurrence of the composite endpoint (comprising death and renal failure) in newly diagnosed AAV cases in four tertial referral centers in Germany and Switzerland diagnosed between 1999 and 2022. Hazard ratios were computed by Cox proportional hazard and Kaplan–Meier curves were plotted to compare therapeutic strategies after propensity-matching. In our cohort of 358 AAV patients, 203 (58.1

Background: Until now, prospective long-term observational data on the disease course of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) were missing in Germany. Therefore, the Joint Vasculitis Registry in German-speaking countries (GeVas) has been established to follow the course of patients with AAV. Objectives: To present baseline data of patients with newly diagnosed and relapsing AAV enrolled in the GeVas registry. Methods: GeVas is a prospective, web-based, multicenter, clinician-driven registry for the documentation of organ manifestations, damage, long-term outcomes, and therapy regimens in various types of vasculitis [1]. Recruitment started in June 2019 and is currently being conducted by 15 centers in Germany to date. Results: Between June 2019 and October 2022, 266 patients with AAV were included in the GeVas registry, hereof 173 (65%) with new-onset and 93 (35%) with relapsing AAV. One hundred and sixty-two (61%) patients were classified as granulomatosis with polyangiitis (GPA), 68 (26%) as microscopic polyangiitis (MPA), and 36 (14%) as eosinophilic granulomatosis with polyangiitis (EGPA). The median age was 59 years (51-70 years, IQR), 130 (51%) patients were female, 136 (49%) males. Most patients were ANCA positive (177; 67%) and affected by general symptoms, pulmonary, ear nose throat (ENT), renal and neurological involvement. For induction of remission, the majority of patients received glucocorticoids (247, 93%) in combination with either rituximab (118, 45%) or cyclophosphamide (112, 42%) (Table 1). Conclusion: Herein, we present baseline data from an inception cohort of AAV patients in the GeVas registry. Demographic characteristics are largely comparable to those in other European countries. Hoewever, differences were found regarding ANCA-status, frequencies of organ manifestations, and therapeutic regimen [2-4]. The GeVas registry will allow continued longitudinal observation and prospective outcome measures in AAV. REFERENCES: [1] Iking-Konert C, et al. The Joint Vasculitis Registry in German-speaking countries (GeVas) – a prospective, multicenter registry for the follow-up of long-term outcomes in vasculitis. BMC Rheumatol 2021;5:40. [2] Wójcik K, Wawrzycka-Adamczyk K, Włudarczyk A, et al. Clinical characteristics of Polish patients with ANCA-associated vasculitides-retrospective analysis of POLVAS registry. Clin Rheumatol 2019;38(9):2553-2563. (In eng). DOI: 10.1007/s10067-019-04538 w. [3] Solans-Laqué R, Fraile G, Rodriguez-Carballeira M, et al. Clinical characteristics and outcome of Spanish patients with ANCA-associated vasculitides: Impact of the vasculitis type, ANCA specificity, and treatment on mortality and morbidity. Medicine (Baltimore) 2017;96(8):e6083. DOI: 10.1097/md.0000000000006083. [4] Ponte C, Khmelinskii N, Teixeira V, et al. Reuma.pt/vasculitis - the Portuguese vasculitis registry. Orphanet J Rare Dis 2020;15(1):110. (In eng). DOI: 10.1186/s13023-020-01381-0. Table 1. Demographics and clinical characteristics of AAV patients enrolled in GeVas registry (06/2019 – 10/2022). Acknowledgements: GeVas was supported by unrestricted grants by DGRh, John Grube Foundation, Vifor Pharma, and Roche Pharma. Disclosure of Interests: Sabrina Arnold: None declared, Pia Wallmeier: None declared, Arlette Tais: None declared, Gabriele Ihorst: None declared, Marco Janosche: None declared, Fabian Schubach: None declared, Peer Malte Aries: None declared, Raoul Bergner personal fees for lectures from Abbvie, BMS, Chugai, GSK, Galapagos, MSD, Novartis, Roche,, consultant of: VIFOR, GSK, Phillip Bremer personal fees for lectures from: UCB Pharma, Chugai, Celgene, Abbvie, Janssen, Galapagos, Lilly, Sanovi-Aventis, Novartis, AstraZeneca, GSK, Roche, Consultant of: Galapagos, Norman Görl: None declared, Eva Gutdeutsch: None declared, Bernhard Hellmich personal fees for lectures or advisory services from Amgen, AstraZeneca, BMS, Boehringer Ingelheim, Chugai, InflaRx, GSK, Pfizer, Phadia, MSD, Roche, Novartis and Vifor outside the submitted work, personal fees for lectures or advisory services from Amgen, AstraZeneca, BMS, Boehringer Ingelheim, Chugai, InflaRx, GSK, Pfizer, Phadia, MSD, Roche, Novartis and Vifor outside the submitted work, Jörg Henes: None declared, Bimba Hoyer: None declared, Antje Kangowski: None declared, Ina Kötter: None declared, Martin Krusche: None declared, Tim Magnus: None declared, Claudia Metzler: None declared, Ulf Müller-Ladner: None declared, Jana Petersen: None declared, Anke Reichelt de Tenorio: None declared, Matthias Schaier: None declared, Jan Schirmer Grant/research support from: John-Grube Foundation, Deutsche Gesellschaft für Rheumatologie, Ulf Schönermark Lecture fees from Janssen-Cilag and Alexion/AstraZeneca, Study fees and consultancy fees from Alexion/AstraZeneca, Ablynx/Sanofi and Chemocentryx/Vifor., Jens Thiel personal fees for lectures or advisory services from Abbvie, AstraZeneca, BMS, GSK, Pfizer, Novartis and Vifor, personal fees for lectures or advisory services from Abbvie, AstraZeneca, BMS, GSK, Pfizer, Novartis and Vifor, Leonore Unger: None declared, Nils Venhoff Speakers bureau: Roche and Vifor: speaker honoraries, Consultant of: Roche and Vifor: advisory boards, Grant/research support from: John- Grube Research Award 2021, Julia Weinmann-Menke: None declared, Christoph Iking-Konert Speakers bureau: Lecture fees from: Chugai, GSK, Roche, and Vifor, SConsultant of: Consulting fees from: Chugai, GSK, Roche, and Vifor, Grant/research support from: Research grants for GeVas: Roche, Vifor, DGRh, John Grube Foundation,, Peter Lamprecht Speakers bureau: BMS, FOFM, GSK, Janssen, UCB, and Vifor Pharma, Consultant of: GSK, and Vifor Pharma, Grant/research support from: BMBF, DFG, DGRh, John Grube Foundation, and Vifor Pharma.

BACKGROUND:Immunoglobulin A (IgA) nephritis (IgAN) and renal IgA vasculitis (IgAV) show renal IgA deposits, but whether these two diseases are distinct entities or a spectrum of the same condition is under debate. In this study, we add perspective by contrasting the clinical course and histological presentation using the Oxford classification and the National Institutes of Health lupus nephritis activity index (LN-AI) and chronicity index (LN-CI) in IgAN and IgAV. METHODS:In this single-centre, retrospective study, kidney biopsies of 163 adult patients with IgAN and 60 adult patients with IgAV were compared according to the Oxford MEST-C score, LN-AI and LN-CI. At the time of biopsy, clinical presentation was compared in terms of age, arterial hypertension, diabetes mellitus, extrarenal manifestations, estimated glomerular filtration rate, proteinuria and urine sediment. IgAV patients and all IgAN patients with crescents received immunosuppressive treatment. After biopsy, kidney function was followed until patients reached end-stage renal disease (ESRD) or they died. RESULTS:The clinical course and kidney histology differ in IgAN and IgAV. IgAV patients showed more microhaematuria and nephritic sediment, while IgAN patients had a greater history of arterial hypertension, more proteinuria and a higher risk for ESRD. These clinical differences were associated with histological differences, as kidney biopsies of IgAN patients were characterized by glomerulosclerosis and tubular atrophy while kidney biopsies of IgAV patients were characterized by endocapillary hypercellularity and crescents. Overall, tubular atrophy and an LN-CI ≥4 were associated with a higher risk for ESRD in IgAN and IgAV. CONCLUSION:Our study supports the notion that IgAN and IgAV follow distinct courses, suggesting that they require different treatment strategies. Moreover, we make a point that the Oxford classification and LN-CI can be useful in categorizing and predicting long-term prognosis not only in IgAN, but also in IgAV.

ZUSAMMENFASSUNGDie Sarkoidose ist in Nordeuropa die häufigste granulomatöse Erkrankung. Man unterscheidet zwischen akuten Formen der Sarkoidose (Löfgren-Syndrom, Heerfordt-Syndrom) und der chronischen Sarkoidose. Die chronische Sarkoidose kann nahezu alle Organe betreffen. Die Lunge ist das am häufigsten betroffene Organ. Die Niere ist mit ca. 30 % der Fälle bei gründlicher Diagnostik nach der Lunge eines der am häufigsten mitbeteiligten Organe. Die in der Diagnostik gefundenen Befunde sind jedoch meistens wenig spektakulär und reichen von einer geringen Proteinurie über eine sterile Leukozyturie bis zu einer eingeschränkten Nierenfunktion. Meistens kann erst eine Nierenbiopsie den Befund einer renalen Sarkoidose oder sekundären Glomerulonephritis klären. Bedingt durch Störungen im Kalziumstoffwechsel können zudem eine Nephrokalzinose oder Nephrolithiasis auftreten. Auch Kombinationen der verschiedenen renalen Befunde sind möglich. Die Therapie der renalen Sarkoidose besteht in erster Linie aus Kortikosteroiden. Die Evidenz dafür beruht jedoch nur auf Fallserien. Andere Immunsuppressiva werden in der Literatur kasuistisch beschrieben.

Kidney involvement with resulting kidney failure leads to increased mortality in patients with multiple myeloma (MM). Cast nephropathy (CN), in particular, if left untreated, quickly leads to kidney failure requiring dialysis and has a very poor prognosis for the affected patient. The gold standard for diagnosing kidney involvement is a kidney biopsy. However, due to bleeding risk, this cannot be done in every patient. We recently reported that a quotient of urine light chain (LCurine) to glomerular filtration rate (eGFR) is a non-invasive diagnostic tool for patients with kidney involvement in MM. But this quotient has not yet been tested in everyday clinical practice. In this study, our LCurine/eGFR ratio was tested on 67 patients in two centers. Enrollment took place between January 2019 and September 2023. A total of 18 of the 67 patients had CN. With the threshold defined in our initial paper, we were able to show a sensitivity of 100% with a specificity of 85.7% for CN in patients with MM. As a result, the LCurine/eGFR quotient recognizes 100% of all CN and can therefore detect this group, which has a very poor prognosis, without the need for a kidney biopsy.

Background BDMARD treatments in patients with psoriatic arthritis (PsA) are initiated after insufficient response to csDMARD either in monotherapy or by in parallel continuing csDMARDs. Here, the value of MTX in combination with bDMARDs in PsA is still unclear. We designed an investigator-initiated, randomized, placebo-controlled trial (IIT) in active PsA to examine the potential impact of MTX-continuation or -initiation parallel to newly started UST on different outcomes beyond clinical examination (MSUS; PsASon22 score [1]). Objectives To compare sensitive imaging efficacy outcomes measured by MSUS changes [PsASon22] from week 4 and 24 to baseline in UST+PBO vs UST+MTX (stratified to ongoing or new onset of MTX treatment). Methods A total of 186 patients with active PsA (defined as TJC≥4, SJC≥4 [68/66 joint count] and DAS28≥3.2) were screened for eligibility. 173 patients were randomized to UST+MTX (new or ongoing) or UST+PBO. 84 patients were included in the subgroup analysis with MSUS scoring of PsASon22 at BL, weeks 4 and 24. Results were compared between the groups with prior MTX treatment (UST+ ongoing MTX or UST+PBO) and with new initiation of MTX to UST (UST+ new MTX or UST+PBO) according to the study design. Results BL data were well-balanced between treatment groups (UST+MTX, n=44; UST+PBO, n=40) and subgroups with exception of slightly higher age (mean age 53.3 years) and more (45%) female patients in the UST with ongoing MTX group. All baseline data are shown in Table 1. After UST initiation, improvement of inflammatory activity measured by MSUS using PsASon22 score was seen early at week 4 in all treatment groups. The improvement was delayed in patients with previous MTX failure (“prior MTX”) and, in overall less pronounced compared to the patients without previous MTX treatment (Figure 1). At week 24, clinical meaningful changes in PsASon22 score were seen in all treatment groups but with most prominent differences in both treatment groups with UST+PBO. Conclusion IL12/23 inhibition with UST is an effective treatment for active PsA independent of MTX use. Data from this IIT indicate that additional MTX has no positive impact on UST efficacy for musculoskeletal manifestations such as arthritis and enthesitis measured sensitively by PsASon22 ultrasound score validated for PsA monitoring. Moreover, by monitoring in MSUS, patients on UST monotherapy seem to benefit best from the treatment. Reference [1]Ficjan, A., et al., Ultrasound composite scores for the assessment of inflammatory and structural pathologies in Psoriatic Arthritis (PsASon-Score). Arthritis Res Ther, 2014. 16(5): p. 476. Acknowledgements: NIL. Disclosure of Interests Michaela Köhm Speakers bureau: Janssen, UCB, Pfizer, Novartis, Consultant of: Janssen, UCB, Pfizer, Novartis, Grant/research support from: Janssen, Pfizer, GSK, BMS, Ann Christina Foldenauer Grant/research support from: Janssen, GSK, BMS, Pfizer, Tanja Rossmanith Grant/research support from: Janssen, GSK, Leo, Pfizer, BMS, Siegfried Wassenberg: None declared, Stephanie Finzel: None declared, Arnd Kleyer: None declared, Raoul Bergner: None declared, Rieke Alten: None declared, Herbert Kellner: None declared, Jochen Walter: None declared, Peter Kästner: None declared, Frank Behrens Speakers bureau: Janssen, Pfizer, UCB, Novartis, Consultant of: Janssen, Pfizer, UCB, Novartis, Grant/research support from: Janssen, Pfizer, UCB, Novartis, GSK, BMS.