Composite haemangioendothelioma in the heart: a case report.

Background: Primary cardiac neoplasm is rare and generally benign. Epithelioid haemangioendothelioma, a potentially malignant tumour of vascular origin, has been occasionally described in the heart. Composite haemangioendothelioma, characterized by a heterogeneous architecture of vascular components and usually located in soft tissue of the extremities, has only been reported twice in the heart. We herein report another case of this extremely uncommon cardiac tumour. Case summary: Comprehensive cardiac examination of a 59-year-old female patient with palpitations and personal history of Hodgkin's lymphoma and chest radiation revealed a mass in the left atrium. After surgical resection, histopathological and immunohistochemical analysis identified a composite haemangioendothelioma. After two years, repeated imaging revealed neither signs of local relapse nor metastasis. Conclusions: Composite haemangioendothelioma, a very uncommon form of potentially malignant vascular tumour, can also be encountered in the heart. In this present case, the outcome was favourable two years after surgical resection without adjuvant therapy.

Tricuspid Regurgitation and Right Ventricular Function in Patients Undergoing Percutaneous Treatment of Mitral Regurgitation.

OBJECTIVES: Patients with severe mitral regurgitation (MR) frequently present with concomitant right ventricular (RV) dysfunction and tricuspid regurgitation (TR). We aimed to investigate the prognostic relevance of RV function, RV dimension, and TR in patients undergoing percutaneous intervention for MR. METHODS: Consecutive patients undergoing percutaneous mitral valve intervention were enrolled in the prospective MitraSwiss registry. Tricuspid annular plane systolic excursion (TAPSE), pulmonary artery systolic pressure (PASP), right ventricular pulmonary arterial coupling (RVC, defined as TAPSE/ PASP ratio), indexed tricuspid annulus (TA) dimension, and TR severity grade were analyzed at baseline, post procedure, and at 6-month follow-up. The endpoints of all-cause mortality, hospitalization for heart failure, and the combined endpoint of the 2 were observed during long-term follow-up (up to 4 years). RESULTS: We analyzed 218 patients (mean age, 76 ± 9 years; 36% female). Edge-to-edge mitral valve repair resulted in an increase in TAPSE and RVC ratio and a decrease in indexed TA and PASP, but concomitant TR did not change significantly. In multivariable analysis, RV dysfunction and moderate/severe TR were independently associated with increased all-cause mortality (hazard ratio, 1.61; 95% confidence interval, 1.05-2.46; P=.03 and hazard ratio, 2.10; 95% confidence interval, 1.34-3.29; P<.01, respectively) and moderate/severe TR was further an independent predictor for hospitalization for heart failure and for the combined endpoint. CONCLUSION: Treatment of MR resulted in favorable changes of RV function and dimension but did not reduce TR in the majority of patients. TR at baseline remained the strongest predictor for outcomes, outperforming parameters of RV function and dimension.

Outcome of patients with double valve surgery between 2009 and 2018 at University Hospital Basel, Switzerland.

BACKGROUND: In isolated mitral valve regurgitation general consensus on surgery is to favor repair over replacement excluding rheumatic etiology or endocarditis. If concomitant aortic valve replacement is performed however, clinical evidence is more ambiguous and no explicit guidelines exist on the choice of mitral valve treatment. Both, double valve replacement (DVR) and aortic valve replacement in combination with concomitant mitral valve repair (AVR + MVP) have been proven to be feasible procedures. In our single-center, retrospective, observational cohort study, we compared the outcome of these two surgical techniques focusing on mortality and morbidity. METHODS: 89 patients underwent DVR (n = 41) or AVR + MVP (n = 48) in our institution between 2009 and 2018. Follow-up data was collected using electronic patient records, by contacting treating physicians and by telephone interviews. We used the Kaplan-Meier method to analyze mortality during follow-up and Cox regression to investigate potential predictors of mortality. RESULTS: During a median follow-up duration of 4.5 [IQR 2.9 to 6.1] years, there was no significant difference in mortality between both cohorts. Thirty days mortality was 6.3% in the DVR and 7% in the AVR + MVP cohort. Overall mortality amounted to 17% for DVR and 23% for AVR + MVP. DVR was the preferred procedure for valve disease of rheumatic etiology and for endocarditis, while in degenerative valves AVR + MVP was predominant. More biological valves were used in the AVR + MVP cohort (p < 0.001) and more mechanical valves were implanted in the DVR cohort. The rate of rehospitalization, deterioration of left ventricular ejection fraction and postoperative complications were equally distributed among the two cohorts. CONCLUSION: Our data analysis showed that both DVR and AVR + MVP are safe and feasible options for double valve surgery. Based on our findings we could not prove superiority of one surgical technique over the other. Choosing the appropriate procedure for the patient should be influenced by valve etiology, patients' comorbidities and the surgeons' experience. TRIAL REGISTRATION: This was a retrospectively registered trial, registered on April 1st 2018, ClinicalTrials.gov Identifier: NCT03667274.

The relevance of tricuspid regurgitation in patients undergoing percutaneous treatment of mitral regurgitation.

OBJECTIVES: Investigate the impact of concomitant tricuspid regurgitation (TR) on clinical outcomes during long-term follow-up in patients undergoing percutaneous treatment of mitral regurgitation (MR) with the MitraClip system. BACKGROUND: Patients undergoing mitral repair using the MitraClip frequently present with concomitant TR. It is uncertain how the presence of TR impacts the long-term outcomes of such patients. METHODS: We analyzed consecutive patients with MitraClip implantation from the prospective MitraSwiss registry. Endpoints were all-cause mortality, hospitalization for heart failure, and the composite endpoint of the two. RESULTS: We enrolled 177 patients (mean age 76 ± 9 years, 37% female). Acute procedural success was achieved in 149 (84%). Concomitant moderate or severe TR was present in 31% at baseline and 32% before discharge. After a median follow-up of 1103 days (IQR: 555-1766 days), 70 (40%) of patients had died, and 34 (19%) were hospitalized for heart failure. In multivariable analysis, TR at baseline was associated with an increase in all-cause mortality (HR: 2.34, 95% CI: 1.36-4.03, p < 0.01), hospitalization for heart failure (HR: 3.19, 95% CI: 1.37-7.41, p = 0.01), and the composite endpoint (HR: 2.00, 95% CI: 1.19-3.36, p = 0.01). CONCLUSION: Despite treatment of MR, TR did not improve in most patients. The presence of relevant TR at baseline was associated with reduced survival and higher rates of hospitalization for heart failure. More research is needed to understand the causal role of TR in such patients and to investigate if simultaneous treatment of concomitant TR may improve prognosis in patients undergoing percutaneous treatment of MR.

Humanoids for teaching and training coronary artery bypass surgery to the next generation of cardiac surgeons.

OBJECTIVES: Technical skills are an essential component of cardiac surgery, and the operating room is becoming an even more challenging environment for trainees who want to acquire such skills. Simulation, which partially overcomes this limitation, represents a valid adjunct to surgical education. We describe an original simulator and provide results in terms of trainees' evaluations and ratings. METHODS: We used a humanoid that is a silicone replica of the chest of an adult human that provides a complete anatomical platform for hands-on skin-to-skin practice of surgical techniques in arrested heart coronary artery bypass graft (CABG) surgery cases. Learners were residents in cardiac surgery. The teaching sessions included 2 full three-vessel CABG procedures using both mammary arteries and a hydrogel vein. Five board-certified cardiovascular surgeons scored the surgical activity of all trainees. The trainees were asked to complete an exit questionnaire to evaluate their course. RESULTS: Overall, 16 residents participated in the simulation, including 5 women and 11 men, with a mean age of 30 ± 4 years, all of whom had at least 2 years of cardiac surgery training. All participants completed the 2 CABG operations. Three mammary arteries (4.6%) were seriously damaged during harvesting. In 1 case (3.1%), an aortic tear occurred during aortic cannulation. Each trainee performed overall 6 distal and 2 proximal coronary anastomoses. All participants agreed that the 'humanoid reproduces real-life situations, the feeling is realistic, and they are now more confident in performing coronary anastomosis'. CONCLUSIONS: Trainees involved in this simulation curriculum acquired and refined technical skills that could be applied directly to human patients. In addition, we were able to foster a higher level of teamwork within the operating room team.

Endovascular repair of ascending aortic diseases with custom-made endografts.

OBJECTIVES: The aim of this article is to report the mid-term results of ascending thoracic endovascular aortic repair using a custom-made device (CMD). METHODS: This was a retrospective study performed at tertiary centres. Nine patients considered unfit for open surgery received elective total endovascular repair of the ascending aorta with a Relay® (Terumo Aortic, Sunrise; FL, USA) CMD: pseudoaneurysn (n = 5), localized dissection (n =3) and contained rupture (n = 1). RESULTS: Primary clinical success was achieved in all patients with no major complications and no early conversion to open surgery. All patients were discharged home and independent: median length of stay was 7 days (interquartile range, 6-18). No patient was lost to follow-up at a median 26 months (interquartile range, 12-36). Three patients died 2, 6 and 24 months after intervention; 1 was aorta related (late aorto-atrial fistula due to infection that required open surgery). At the last follow-up available, no endoleaks, migrations, fractures or ruptures were observed in the remaining 6 patients. CONCLUSIONS: Ascending thoracic endovascular aortic repair with Terumo Aortic CMDs was technically feasible, effective and safe in very selected lesions. CMDs showed good ascending aorta conformability with different configurations and diameters, and satisfactory mid-term durability as shown by both structural integrity and aortic lesion exclusion.

Low-Volume Cardioplegia and Myocardial Protection in Coronary Artery Bypass Graft Surgery.

We studied myocardial protection during coronary artery bypass graft surgery using low-volume cardioplegia (Cardioplexol) and minimal extracorporeal circulation (MECC) for different types of coronary artery diseases. In total, 426 consecutive patients were included and divided into four groups: those with left main stem stenosis (n = 45), those with three-vessel disease (n = 200), those with both (n = 141), and those with neither (n = 40). The peak postoperative myocardial markers and 30-day mortality were analyzed. Both myocardial markers and 30-day mortality were significantly elevated in patients with isolated main stem stenosis. We conclude that the use of low-volume cardioplegia and MECC is safe. However, patients with underlying isolated left main stem stenosis might be less protected.

X-ray Structures and Feasibility Assessment of CLK2 Inhibitors for Phelan-McDermid Syndrome.

CLK2 inhibition has been proposed as a potential mechanism to improve autism and neuronal functions in Phelan-McDermid syndrome (PMDS). Herein, the discovery of a very potent indazole CLK inhibitor series and the CLK2 X-ray structure of the most potent analogue are reported. This new indazole series was identified through a biochemical CLK2 Caliper assay screen with 30k compounds selected by an in silico approach. Novel high-resolution X-ray structures of all CLKs, including the first CLK4 X-ray structure, bound to known CLK2 inhibitor tool compounds (e.g., TG003, CX-4945), are also shown and yield insight into inhibitor selectivity in the CLK family. The efficacy of the new CLK2 inhibitors from the indazole series was demonstrated in the mouse brain slice assay, and potential safety concerns were investigated. Genotoxicity findings in the human lymphocyte micronucleus test (MNT) assay are shown by using two structurally different CLK inhibitors to reveal a major concern for pan-CLK inhibition in PMDS.

Modified frozen elephant trunk for acute type A aortic dissection: a comparative study with standard repair technique.

Objectives: We hypothesized that antegrade open stent graft implantation in the descending aorta during acute type A aortic dissection surgery is safe and improves patient outcome compared with the standard repair technique. Methods: Hundred and forty-one consecutive patients underwent surgery for acute type A aortic dissection at our institution from 2010 to 2016. Of those, 104 patients underwent ascending aorta and hemiarch repair under hypothermic circulatory arrest with antegrade cerebral perfusion (standard group). Since 2013, 37 patients have undergone the standard procedure combined with antegrade stent implantation in the descending aorta (stented group). A matched analysis using the logistic EuroSCORE (37 patients per group) was done. All data were collected prospectively. Results: The mean logistic EuroSCORE was 29 in both groups, P = 1. Cardiopulmonary bypass time was 150 ± 57 (standard) vs 157 ± 48 (stented) min, P = 0.6; aortic clamping 99 ± 47 (standard) vs 100 ± 36 (stented) min, P = 1. Stented patients had longer circulatory arrest times with antegrade cerebral perfusion, 23 ± 7 vs 15 ± 7 min, P < 0.001. Stroke occurred in 24.3% (standard) vs 8.1% (stented), P = 0.1; paraplegia developed in 2.7% (standard) vs 0% (stented), P = 1. Abdominal intervention due to suspected visceral ischaemia was needed in 18.9% (standard) vs 5.4% (stented), P = 0.2. 30-day mortality was 13.5% (standard) vs 0% (stented), P = 0.05. Survival at 6-month was 100% and 86.5% in patients with implanted stents and standard repair, respectively, P = 0.02. Conclusions: Antegrade, open stent graft implantation into the descending aorta during acute type A aortic dissection repair is safe and is associated with improved outcomes at 6 months postoperatively compared to the standard repair technique.

Indolyl-naphthyl-maleimides as potent and selective inhibitors of protein kinase C-α/ß.

The indolyl-naphthyl maleimide 7 is a potent inhibitor of the classical PKC isotypes α,ß and shows excellent selectivity over the novel PKC isotypes δ,ε,η,θ and other kinases belonging to the AGC family. The SAR around 7 as well as the physico-chemical characteristics of selected derivatives and their activity in T and B cell activation and proliferation assays are discussed.

Computed Tomography Scan Predicts Abdominal Interventions but Not Stroke after Surgery for Acute Type A Aortic Dissection.

BACKGROUND: We hypothesized that preoperative computed tomography (CT) is a predictor of abdominal intervention for visceral malperfusion and stroke after emergent surgery for acute type A aortic dissection (AAAD). METHODS: A total of 90 patients, mean (± SD [standard deviation]) age 62 (± 12) years, 76% males, undergoing emergent surgery for AAAD at our institution from May 2008 to August 2013 were included. All clinical data were collected prospectively and correlated to CT images. RESULTS: At initial presentation 9 (10%) patients showed preoperative focal neurologic deficit or coma, 10 patients (11%) complained of abdominal pain, and the logistic EuroSCORE was 44 (± 22). Hemiarch replacement was performed in 96%, total arch in 4%. The duration of hypothermic circulatory arrest (HCA) at 28°C bladder temperature was 26 (± 19) minutes. Cross-clamp time was 88 (± 39) minutes, and cardiopulmonary bypass (CPB) time 148 (± 49) minutes. Overall 30-day mortality was 13%. Moreover, 12 (13%) patients required postoperative abdominal interventions for suspected visceral malperfusion; stroke occurred in 25 (28%) patients. Logistic regression revealed that "dissection of the celiac trunk and/or the superior mesenteric artery" in preoperative CT images is a predictor of postoperative abdominal interventions for visceral malperfusion (p = 0.03), but preoperative abdominal pain is not similarly predictive. Postoperative stroke is best predicted by preoperative neurologic symptoms (p = 0.01), but not by supra-aortic vessel dissection in preoperative CT images. CONCLUSION: In patients undergoing surgery for AAAD, analysis of preoperative CT images allows identifying those with a high risk of postoperative abdominal intervention for visceral malperfusion. Postoperative stroke is best predicted by preoperative neurologic symptoms.

Reduced aspirin responsiveness as assessed by impedance aggregometry is not associated with adverse outcome after cardiac surgery in a small low-risk cohort.

Reduced aspirin responsiveness (i.e. persistent high platelet reactivity in platelet function testing) might be associated with increased risk of myocardial ischemia and cardiac mortality in patients with coronary disease. However, the impact in patients undergoing coronary artery bypass grafting (CABG) is unclear. The aim of this prospective cohort study was to evaluate the predictive value of reduced aspirin responsiveness on cardiac and thromboembolic events in patients undergoing elective isolated CABG surgery with aspirin intake until at least two days before surgery. We included 304 patients in this prospective single-center cohort study. Impedance platelet aggregometry (Multiplate®) was performed directly before and on the first day after surgery. Reduced aspirin responsiveness was defined as area under the curve in ASPItest (AUCASPI) ≥300 U. The primary outcome was a composite of all-cause mortality and/or major adverse cardiac or thromboembolic events within 1 year. Reduced aspirin responsiveness was found in 13 and 24% of patients pre and postoperatively, respectively. There was no difference in the outcomes between patients with normal and reduced aspirin responsiveness in the preoperative measurement (log-rank test, p = 0.540). Multivariate analysis including logistic EuroSCORE I and postoperative troponin T levels did not show any association of reduced aspirin responsiveness with adverse outcome (hazard ratio, 0.576; (95% CI 0.128-2.585; p = 0.471). Similarly, postoperative reduced aspirin responsiveness was not associated with adverse events. To conclude, reduced aspirin responsiveness as evaluated by Multiplate® platelet function analyzer was not associated with increased incidence of major adverse cardiac and thromboembolic events and mortality after CABG surgery.

Automated fastener versus manually tied knots in minimally invasive mitral valve repair: impact on operation time and short- term results.

BACKGROUND: This study compares the influence of two different annuloplasty attachment suture applications, namely the use of an automated fastener versus manually tied knots using a traditional knot pusher, on total operation time, on cardiopulmonary-bypass time and on cross-clamp time, and on short-term outcome. METHODS: Sixty patients underwent isolated minimally invasive mitral valve repair in Carpentier Type-II mitral disease with implantation of an annuloplasty ring in combination with correction of the prolapsing leaflet using artificial chords. The first 30 patients after implementation of a novel automated fastener were compared with the last 30 patients corrected with a traditional knot pusher. No significant differences with regard to demographic data (age, gender, NYHA class, ejection fraction, BMI, cardiovascular risk factors) between the two groups were found. All patients received isolated mitral valve repair in the first run. Bretschneider HTK was used for cardioplegic cardiac arrest in all patients. RESULTS: Transesophageal and transthoracic echocardiography at the end of operation and at discharge revealed no (n = 25), trace (n = 28) or mild (n = 7) residual regurgitation with no evidence of ring dehiscence and without any significant clinical differences between the groups. Cross-clamp, cardiopulmonary-bypass and total- operation time were significantly reduced in the automated fastener group compared to the group using a traditional knot pusher (87.1 ± 17.9 vs. 101.3 ± 17.8; p < 0.01, 138.1 ± 25.6 vs. 152.7 ± 24.9; p < 0.05, and 203.9 ± 31.02 vs. 223.8 ± 29.01; p < 0.01, respectively). CONCLUSION: Our short-term results indicate a safe, reliable and fast application of the novel automated fastener device in combination with significant time savings in cardioplegic arrest and cardiopulmonary bypass.

Elevated Membrane and Soluble CD64: A Novel Marker Reflecting Altered FcγR Function and Disease in Early Rheumatoid Arthritis That Can Be Regulated by Anti-Rheumatic Treatment.

OBJECTIVES: Fc receptors (FcR) interacting with immune complexes (ICs) is a central event in the immune pathogenesis of rheumatoid arthritis (RA). Here we asked if a specific FcR is linked to RA pathogenesis and if FcR activities relate to disease and treatment outcome in early RA. MATERIAL AND METHODS: Twenty autoantibody-positive RA patients and 33 HC were included. The patients were evaluated before and after treatment with methotrexate and prednisolone. At follow-up, the EULAR response criteria were applied to determine the individual treatment outcomes. Serum immunoglobulin levels were measured and the expression of FcR for IgG (FcγR) and IgA (FcαR) on peripheral blood monocytes were determined by flow cytometry. The monocytic FcγR function was evaluated by human IgG1 and IgG3 IC-binding and TNFα stimulated release. Plasma levels of soluble FcRs (sFcRs) were determined with ELISA. RESULTS: The IgG1 and IgG3 levels were elevated in the RA sera. The RA monocytes expressed more CD64 and cell surface-bound IgG than HC monocytes, and showed an impaired FcγR function as reflected by changes in IC-binding and decreased IC-stimulated TNFα secretion. These findings correlated significantly with different disease activity markers. Furthermore, sFcRs were elevated in the patient plasma, and sCD64 was specific for RA (compared with a reference group of patients with active psoriatic arthritis). Following treatment, immunoglobulins and sFcR levels were reduced, whereas membrane CD64 was only decreased in patients with good response to treatment. CONCLUSIONS: Early RA patients display increased membrane and soluble CD64 and an impaired FcγR function correlating with joint disease activity. Beneficial responses of anti-rheumatic treatment in patients reduce CD64. These data suggest sCD64 as an important objective biomarker in RA.

One-year patency control and risk analysis of eSVS®-mesh-supported coronary saphenous vein grafts.

BACKGROUND: The eSVS® external venous nitinol mesh (Kips Bay Medical, Minneapolis, USA) was designed to improve long-term patency of coronary saphenous vein grafts (SVG) by preventing pressure-induced wall stress and reactive neo-intimal hyperplasia. We present one-year-patency rates of meshed SVGs assessed by coronary computed tomographic angiography (cCTA). PATIENTS AND METHODS: Data from consecutive patients receiving an eSVS® meshed coronary bypass SVG from 06/2010 to 06/2011 were prospectively collected and analysed post-hoc. Patient characteristics, coronary artery disease, SVG quality, surgery (including number of anastomoses and transit time flow-measurement: TTFM), postoperative course and graft patency by cCTA were recorded. Potential risk factors for meshed graft occlusion were evaluated. RESULTS: 22 patients received an eSVS® mesh (18 isolated CABG, 4 combined with aortic valve replacement). Three patients died prior to the one-year follow-up and were excluded. All 19 surviving patients (mean age 70.4 ± 9.5 years, 3 female) completed a cCTA of all grafts at 12 ± 0.1 months after surgery including 21 meshed SVGs (33 distal anastomoses), 7 unmeshed SVGs (13 distal anastomoses) and 22 arterial grafts (30 distal anastomoses). Mesh application was safe with patent grafts (by intraoperative TTFM) and perioperative course uneventful in all patients. The average graft/anastomosis number per patient was 2.6 ± 0.5/3.7 ± 0.8. Patency was unrestricted in all arterial and unmeshed SVGs (cCTA). Meshed SVG patency was 85 % (n = 28/33) for distal anastomoses and 76 % (n = 16/21) among meshed SVGs. Four SVGs with single distal anastomosis to the right coronary were completely occluded. One sequential graft to the left coronary was occluded between proximal and first distal anastomosis (see Fig. 1). Patency was independent of target site, coronary run-off, SVG quality and sequential distal grafting. All patients were asymptomatic. CONCLUSIONS: The overall one-year patency rate of eSVS® meshed SVGs/anastomoses was 76 %/85 %. Surgical implantation is safe independently of target site, run-off, vein quality and sequential distal anastomoses. However, graft patency of meshed veins (76 %) was inferior to non-meshed (100 %) or arterial grafts (100 %). Thus our mid-term data do not sustain the concept of improving vein graft patency by external reinforcing with the eSVS® mesh. Further long-term follow-up is warranted.

Prasugrel increases the need for platelet transfusions and surgical reexploration rates compared with clopidogrel in coronary artery bypass surgery.

BACKGROUND: We hypothesized that patients treated with prasugrel compared with those treated with clopidogrel before coronary artery bypass graft (CABG) surgery are at increased risk of hemorrhagic complications needing transfusions and are more likely to need surgical reexploration. PATIENTS AND METHODS: Preoperative and postoperative clinical data were collected prospectively on 143 consecutive patients treated preoperatively with prasugrel (n = 59) or clopidogrel (n = 84) before undergoing isolated CABG at our institution from January 2011 to October 2012. RESULTS: Patients receiving prasugrel showed a slightly higher additive EuroSCORE I compared with those treated with clopidogrel (7 vs. 5, p = 0.001), and more often underwent urgent/emergent CABG (47 vs. 27%, p = 0.01). Patients treated with prasugrel were more likely than those treated with clopidogrel to need perioperative platelet transfusions (25 vs. 12%, p = 0.04). The numbers of transfusions of red blood cells, fresh-frozen plasma, or fibrinogen were high in both groups but showed no statistical differences. Patients treated with prasugrel, however, more often needed surgical reexploration for bleeding complications (8 vs. 1%, p = 0.03). Logistic regression revealed that preoperative therapy with prasugrel (RR 2.9, p = 0.01) and urgent/emergent surgery (RR 2.2, p = 0.04) were predictors for the composite endpoint "need for perioperative platelet transfusion and/or surgical reexploration." CONCLUSION: Pretreatment with prasugrel, compared with clopidogrel, in patients undergoing isolated CABG is associated with an increased need for platelet transfusions and a higher risk of surgical reexploration for bleeding complications.

Dysregulated Fc receptor function in active rheumatoid arthritis.

Given the critical role of Fc gamma receptors (FcγR) as primary targets for autoantibody-mediated effects an important issue is how the FcγR pathway is affected in autoimmune disorders. Here we investigated the FcγR function in monocytes from rheumatoid arthritis (RA) patients in relation to immunoglobulin levels and disease activity. Peripheral blood was obtained from 30 RA patients with clinical acute joint synovitis (active RA), 28 RA patients with no clinical signs of acute joint synovitis (non-active RA) and 34 healthy controls. Prior the functional studies the monocytes were characterized of their FcγRI (CD64), II (CD32), IIb (CD32b) and III (CD16) expression as well as their cell surface bound IgG. The monocytic FcγR function was assessed by binding of human IgG1 and IgG3 immune complexes (IC) and TNF secretion in vitro. IgG anti-citrullinated peptide antibodies (ACPA) were analyzed in the plasma. We found that monocytes from active RA patients had increased levels of FcγRI, II and cell surface IgG concurrently with impaired FcγR function. This was evident by reduced IgG1-IC binding and decreased TNF secretion in response to IgG3-IC. In contrast, monocytes from non-active RA patients displayed a normal FcγR function and had increased FcγRIIb expression together with elevated FcγRI, II and cell surface IgG. The ACPA levels did not differ in active and non-active RA patients but correlated with the monocytic FcγRIII expression in the patients. In conclusion, active RA patients display a dysregulated FcγR function that may represent a novel phenotypic and likely pathogenetic marker for active RA. A disease and FcγR function controlling effect is suggested by the increased inhibitory FcγRIIb in non-active RA.