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Background: Rheumatoid arthritis (RA) is a chronic autoimmune inflammatory disease characterized by progressive swelling and stiffness in the joints. Mavrilimumab is a human monoclonal antibody that may block the autoimmune mechanism of the antibodies causing RA. Objective: We aim to assess the safety and efficacy of Mavrilimumab in treating rheumatoid arthritis. Methods: We conducted an online search using PubMed, Scopus, Web of Science, and Cochrane CENTRAL till June 2019, and updated the search in May 2020, using relevant keywords. We screened studies for eligibility. Data were extracted from eligible studies and pooled as Risk ratio (RR) with a 95% confidence interval (CI), using Review Manager software (ver.3.5). Results: Five studies (with 1145 patients) were eligible to our criteria. Pooled result from three trials showed a significant reduction in Disease Activity Score 28 based on C-reactive protein (DAS28-CRP) remission < 2.6 after 12 weeks (RR = 3.31, 95% CI [1.53, 7.18], P = 0.002), American College of Rheumatology (ACR) 20, after 12 weeks (RR = 2.38, 95% CI [1.80, 3.16], P < 0.00001), ACR 50, after 12 weeks (RR = 2.93, 95% CI [1.67, 5.15], P = 0.0002), ACR 70, after 12 weeks (RR = 4.90, 95% CI [1.60, 15.00], P = 0.005). Mavrilimumab not associated with a significant adverse event (RR = 1.22, 95% CI [0.89, 1.68], P = 0.22). Conclusion: We found that subcutaneous Mavrilimumab was effective and well-tolerating in treating RA patients, with no significant adverse events.

B-mode ultrasound (B-US) is the standard imaging modality to evaluate pleural effusion. The value of B-US for assessing the malignancy of a pleural effusion (PE) is limited. For this purpose, computed tomography (CT) and thoracoscopy are the methods of choice to evaluate an effusion. The aim of this Pictorial Essay is to demonstrate contrast-enhanced ultrasound (CEUS) as a method in addition to B-US and CT for the evaluation of PE taking into account the clinical background. The characteristic patterns of pleural pathologies, effusion morphology, and associated lung parenchymal consolidations are presented.

Purpose To describe the clinical awareness and acceptance of ultrasound-diagnosed acute epiploic appendagitis (EA) and their importance to avoid unnecessary therapeutic and imaging measures. Patients and Methods The data were obtained of n=54 patients with acute, localized, peritonitic pain and EA diagnosed by B-mode ultrasound and contrast-enhanced ultrasound examination from November 2003 to September 2020. All examinations were performed by a German Society for Ultrasound in Medicine (DEGUM) Level III qualified examiner. Based on documentation by the treating physicians, the clinical awareness and acceptance of EA diagnosis was determined in all patients and compared between subgroups diagnosed before 2013 and from 2013 onwards. In 2013, a local educational training program regarding the diagnosis of and therapy for EA was initiated for physicians. Results In all patients, EA was sonographically diagnosed by a DEGUM level III qualified examiner. At enrollment, EA was mentioned as a suspected clinical diagnosis in n=1/54 (1.9%) patient. Furthermore, in n=39/54 (72.2%) cases, the EA was documented and accepted by the treating physicians at the time of patient discharge as the final clinical diagnosis. The clinical acceptance was significantly higher from 2013 onwards compared with before 2013 (p0.05). Conclusion In our retrospective study, we showed that awareness and acceptance of the disease EA are low. Low diagnostic acceptance of EA by the clinician leads to unnecessary therapeutic and imaging measures and is a general problem related to rare diseases in the healthcare system.

This study described the frequency of malignant synchronous focal liver lesions (FLLs) in patients with newly diagnosed non-hematologic malignant disease.From June 2013 to January 2017, 434 patients with synchronous FLLs and newly diagnosed underlying malignant disease were included in the study. The diagnosis of the FLLs was made by histology and/or contrast-enhanced imaging. The final medical report was used for analysis in the study.Of the 434 liver lesions, 258 (59.4%) were malignant and 176 (40.6%) benign. All malignant lesions were metastases. The benign liver lesions were comprised of 93 cysts (21.4%), 52 hemangiomas (12.0%), 20 focal fatty sparing (4.6%), 4 focal nodular hyperplasia (0.9%), 3 unspecified benign lesions (0.7%), 2 regenerative nodules (0.5%), 1 calcification (0.2%), and 1 adenoma (0.2%). Diseases with the highest percentage frequency of synchronous malignant FLLs were cholangiocarcinoma with 86.7%, neuroendocrine tumor with 72.7%, and pancreatic carcinoma with 72.0%. Gastric carcinoma (33.3%), breast carcinoma (44.4%), and urothelial cell carcinoma (45.5%) were the diseases with the lowest percentage frequency of synchronous malignant FLLs.In total, the frequency of malignant synchronous FLLs in newly diagnosed non-hematologic malignant disease was 59.4%. In particular, cholangiocarcinoma, neuroendocrine tumor, and pancreatic carcinoma were the diseases with the highest rate of synchronous malignant FLLs.ZIEL: Beschreibung der Häufigkeit maligner synchroner fokaler Leberläsionen (FLLs) bei Patienten mit neu diagnostizierter nichthämatologischer maligner Erkrankung.Von Juni 2013 bis Januar 2017 wurden 434 konsekutive Patienten mit synchronen FLLs und neu diagnostizierter maligner Grunderkrankung in die Studie eingeschlossen. Die Diagnose der FLLs wurde durch Histologie und/oder Kontrastmittelbildgebung gestellt. Der finale Arztbrief wurde für die Analyse in der Studie verwendet.Von den 434 Leberläsionen waren 258 (59,4%) maligne und 176 (40,6%) benigne. Alle bösartigen Läsionen waren Metastasen. Bei den gutartigen Leberläsionen handelte es sich um 93 Zysten (21,4 %), 52 Hämangiome (12,0 %), 20 fokale Fettverteilungsstörung (4,6 %), 4 fokale noduläre Hyperplasien (0,9 %), 3 nicht näher spezifizierte gutartige Läsionen (0,7 %), 2 regenerative Knötchen (0,5 %), 1 Verkalkung (0,2 %) und 1 Adenom (0,2 %). Erkrankungen mit der höchsten prozentualen Häufigkeit von synchronen malignen FLLs waren das cholangiozelluläre Karzinom mit 86,7%, der neuroendokrine Tumor mit 72,7% und das Pankreaskarzinom mit 72,0%. Magenkarzinom (33,3%), Mammakarzinom (44,4%) und Urothelzellkarzinom (45,5%) waren die Erkrankungen mit der geringsten prozentualen Häufigkeit synchroner maligner FLLs.Insgesamt betrug die absolute Häufigkeit von malignen synchronen FLLs bei neu diagnostizierten nichthämatologischen malignen Erkrankungen 59,4 %. Insbesondere cholangiozelluläre Karzinome, NETs und das Pankreaskarzinom zeigten die höchste Rate an malignen synchronen fokalen Leberläsionen.

PURPOSE This retrospective study aimed to describe the B-mode lung ultrasound (B-LUS) and contrast-enhanced ultrasound (CEUS) follow-up patterns of peripheral pulmonary lesions (PPLs) in patients with confirmed pulmonary embolism (PE). PATIENTS AND METHODS Data from 27 patients with a confirmed diagnosis of PE and PPLs over 5 mm from October 2009 to November 2018 were included retrospectively in the study. The inclusion criteria were performance of a baseline CEUS examination, a short-term B-LUS and CEUS follow-up, and a long-term B-LUS follow-up of PPLs. The homogeneity of enhancement of PPLs (homogeneous/inhomogeneous/absent) on CEUS and the presence and size of PPLs on B-LUS were evaluated. RESULTS A total of n = 25/27 (92.6%) lesions showed absent or inhomogeneous enhancement during baseline examination or short-term follow-up, indicating impaired perfusion. On short-term CEUS follow-up, 9/27 cases (33.3%) showed a pattern shift. On B-LUS long-term follow-up, 26/27 lesions (96.3%) were detectable for an average of 10 weeks (range 3-32 weeks). The size of reference lesions was significantly reduced at the time of the final follow-up examination (P

Classic Hodgkin lymphoma (CHL) and nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) are clinically distinct entities, with different prognostic and treatment implications. In addition, several large B-cell lymphomas and some T-cell lymphomas can mimic CHL. Differentiating these entities from CHL is crucial for ensuring appropriate therapy. GATA3 is a T-cell transcription factor involved in T-cell maturation and has been previously shown to be overexpressed in CHL cells via gene expression profiling. We investigated the utility of GATA3 immunostain in differentiating CHL from NLPHL and other mimicking entities.We accrued 17 NLPHLs, 49 CHLs [23 nodular sclerosis (NS), 3 syncytial variants, 3 lymphocyte rich and 13 mixed cellularity types], 4 primary mediastinal large B-cell lymphomas (PMBLs), 2 Epstein-Barr virus (EBV) positive diffuse large B-cell lymphomas (DLBCLs) (EBV+LBCLs), 2 T-cell/histiocyte-rich large B-cell lymphomas (TCHRBCLs), 1 gray zone lymphoma, and 2 tissue microarrays consisting of 72 DLBCLs. One slide from each was stained with GATA3 and percent positive tumor cells and intensity of nuclear expression was semiquantitatively graded independently by 2 board certified hematopathologists.GATA3 was positive in 80% of CHLs. Both percent positivity and intensity of staining varied greatly. Syncytial variant of NS subtype showed the highest positivity rate (3/3; 100%), followed by NS (20/23; 87%), mixed cellularity (9/13; 70%), and lymphocyte rich (2/3; 67%). GATA3 was negative in all NLPHLs, EBV+LBCLs, TCRBCLs, and DLBCLs stained. The single gray zone lymphoma and 3/4 PMBLs were positive.Nuclear expression of GATA3 can be used to delineate CHL from NLPHL. GATA3 positivity effectively excludes NLPHL with 100% negative predictive value. However, as 20% of CHL can be negative for GATA3, CHL cannot be ruled out with negative GATA3. Additional findings include GATA3 positivity among PMBLs, whereas all 72 DLBCLs were negative for GATA3. This finding further highlights similarities between CHL and PMBL.

Background and aims: The prevalence of prediabetes is increasing in Jordan and the Middle East region, which is leading to increased risk of type 2 diabetes and micro- and macro-complications. The aims of the expert panel were to understand and evaluate the current prediabetes situation and practice in Jordan and make recommendations to standardize the management of patients with prediabetes in Jordan. Methods: A panel of diabetes experts met to discuss and review all the current literature related to the region and international guidelines on prediabetes. The panel evaluated and developed a comprehensive understanding, addressing the definition, screening, diagnosis, pharmacological treatment, and management of prediabetes with consideration of local factors. Results: The experts relied on international data to establish criteria with cut-off values for screening and diagnosis to identify asymptomatic patients with prediabetes and diabetes. The panel made protocol recommendations, including lifestyle modifications, and developed potential treatment algorithms to provide guidance on the overall management for Jordanian prediabetic patients. Conclusion: The panel of experts reached consensus recommendations with a tailored detection, screening, and treatment algorithm in anticipation for a country wide adoption and implementation. These recommendations would help increase awareness of prediabetes in Jordan and create local guidelines to better serve Jordanian physicians in the management of patients with prediabetes.

Background Cardiac implantable electronic devices - PM, ICD, and CRTs- are well-proven life-sustaining and the ultimate destination for many heart conditions. Based on scientific evidence, there is a worldwide incremental increase in CIED implantations numbers. Objective Early infection of cardiac implantable electronic devices (CIED)- pacemaker (PM), implantable cardioverter-defibrillator (ICD), and cardiac resynchronization therapy (CRT)- is a growing health challenge. We examined the effectiveness of antibiotic prophylaxis and treatment of early infection of CIED in a single center. Methods This is a retrospective, single-center observational study. Data were collected from patients' records from July 2017-July, 2019. All Patients received intravenous ceftriaxone 2gm before incision, Gentamicin 120mg pocket irrigation, and oral Amoxicillin/Clavulanate for 5 days post-implantation. Results A 639 consecutive CIED implantations - PM (n=474, mean age, 64yr, female=49%), ICD (n=106, mean age 56yr, female=17%) and CRT (n=59, mean age, 54yr, female=20%)- were performed over 3years. The incidence of early infection was 1.9% (12 cases), female=41%. PM=5/474, ICD=5/106, and CRT=2/59. Three out of the 12 patients had total device explant due to pocket abscess; one PM had a generator changed; one ICD who had a pneumothorax, and the third one had reimplantation after ICD lead perforation. Nine cases were managed conservatively using saline dressing and oral Amoxicillin/Clavulanate, 3/9 patients developed a hematoma, 4/9 patients developed purulent suture line infection. None of them had infection recurrence on three months follow up. Conclusion Early infection of CIED is a rare complication with multiple predisposing factors. Our protocol is reassurance and prompt initiation of management protocol to prevent and treat this issue's sequences.

The aim of our study was to assess our experience of coronary angiography and intervention via the radial artery in comparison to the standard approach from the femoral artery at Queen Alia Heart Institute. Method; This was a prospective study conducted from September 2010 till August 2013. This was a single operator experience. All adult patients undergoing coronary angiography or intervention were enrolled into this study. It was left to the operator’s discretion to decide which patient is for trans-radial access. Demographic data, risk factors for coronary artery disease (CAD), the amount of contrast used, fluoroscopy time, and the diagnosis were collected. We also looked at the time taken for mobilization of patients following procedure, the incidence of pain from the site, any local complications and hospital stay. The data was compared to a similar number of patients who had coronary angiography via the femoral artery. Results; We looked at 456 patients. There were 346 males (76%) and 110 females (24%). Their age ranged from 29 – 76 years with a mean of 53.5 (± 10.5) years. The right radial artery was used in almost all cases (454 patients, and the left radial in 2 patients). The prevalence of diabetes was 48.5%, hypertension 59.2%, family history of premature CAD 28%, hyperlipidaemia in 30% and 44% were smokers. These figures were the same for patients who had the procedure via the trans-femoral artery done during the study period. Successful angiography was feasible in 449 patients (98.46%). Diagnostic angiography was done in 319 patients (71%), while coronary intervention was done in 130 patients (29%). Total procedural time averaged 41 (± SD = 22) minutes for trans-radial compared to 40 (± 23) for trans-femoral approach (P non significant). Fluoroscopy time was 15 (± 10) minutes for the former compared to 18 (± 13) minutes for the later approach (P non significant). The amount of contrast used was 180 (± 64) ml for trans-radial compared to 192 (± 73 ml) for the trans-femoral approach (P non-significant). On average patients in the trans-radial group had less access site complications with early mobility and earlier discharge. Conclusion; Coronary angiography and intervention via the radial artery has a high success rate with no difference in terms of procedure time, amount of contrast or fluoroscopy time. It has demonstrable advantages in terms of local site complications, early mobility and discharge leading to better patient’s satisfaction.

Aim: We set our study to assess how good are we at achieving targets of lipid profile in patients with coronary artery disease at Queen Alia Heart Institute – King Hussein Medical Centre Amman – Jordan. Method: This was a retrospective analysis of the lipid profile of patients who are being followed up for coronary artery disease at Queen Alia Heart Institute. Lipid profile (total cholesterol, LDL cholesterol, HDL cholesterol and triglycerides) of patients who are legible for secondary prevention was collected and compared to the targets set by the Adult Treatment Panel (ATP) III guidelines. Demographic data and risk factors for coronary artery disease (CAD) were collected. The type and dose of lipid lowering drug was recorded. Results; Lipid profiles of 1250 patients were looked at. Their age ranged from 29 – 76 years with a mean age of 59.6 years (±12.8). 27% of our study were females. The mean age for females was 64.2 years (±11.4) and for males was 57.4 years (±13.1). The prevalence of diabetes was 48.5%, hypertension 59.2%, hyperlipidaemia in 30%, family history of premature CAD 28% and 44% were smokers. Target total cholesterol was achieved in 67% of patients, and target LDL in 54% and HDL in 49% of patients. Almost all patients were on statins or fibrates (94%). 52% were on Simvastatin (84% of them on 20mg) and 38% on Atorvastatin (88% of them on 20mg). Conclusion; Target lipid profile in our patients has only been moderately achieved in patients with coronary artery disease. Only modest doses of statins are used. More efforts with better follow up is needed.

Monoclonal gammopathy of undetermined significance (MGUS) is the most common form of plasma cell dyscrasia, with a prevalence of 3% in the general population above age of fifty. MGUS has a malignant evolution rate of 1% per year. Large longitudinal studies have suggested that virtually all patients diagnosed with multiple myeloma (MM) had a preceding MGUS, with 75 % having detectible Monoclonal (M) protein ≥8 years prior to diagnosis. It is important to identify the features at diagnosis that can predict neoplastic transformation to MM. Purpose We identified 239 patients at our institute in whom MGUS was diagnosed between 2000 and 2010. The presenting clinico-hematologic features were correlated with the frequency of evolution into MM to identify early predictors of evolution. The primary end point was progression to MM. Results The patients' mean age was 70.7 years. The Male/Female ratio was 0.7. The mean concentration of the M component (MC) was 0.7 g/dL. IgG was the most frequent MC (77%), followed by IgA (13%). The median ratio of MC protein to total protein was 0.5. Single or multiple background polyclonal (PC) suppression was noted in 36% of patients. PC suppression of 50% or more was noted in 20.1% of patients, 49.8% had < 50% and 30.1% had no suppression. Mean bone marrow plasma cell percentage was 4.5 percent and mean hemoglobin was 12.4 g/dL. Eighteen of the 239 patients with MGUS progressed into MM over ten years of follow up. Univariate comparisons of all variables between those who progressed and those who did not, showed that the initial concentration of the serum M protein, ratio of M protein to the total protein, number of PC gamma globulins suppressed, degree of PC suppression and IgM gamma globulin suppression were statistically significant risk factors that correlated with progression into MM. Fourteen out of eighteen patients with progressive disease had either PC suppression or background IgM suppression. Conclusions Monoclonal protein concentration, ratio of M protein to the total protein and abnormal serum free light chain ratio are simple variables that have been shown in multiple previous studies to predict the progression of MGUS into MM. In our study, we additionally found that number of PC suppressed, degree of suppression and IgM suppression are also key risk factors that can predict progression. We believe that these variables can be potentially applied into an approach that uses a detailed risk stratification system to predict which cases of MGUS will progress into MM and to provide more intensive monitoring for patients more likely to progress. Disclosures: No relevant conflicts of interest to declare.

Abstract 4642 Thrombotic thrombocytopenic purpura (TTP) is a rare but serious disease in which early diagnosis and management has a major impact on outcome. TTP has been historically linked to a pentad of clinical features (mental status changes, fever, acute renal failure, anemia and thrombocytopenia). The frequency of the classical pentand in the pre plasma exchange era has been reported to be as high as 40 percent. With the introduction of plasma exchange and increased awareness of this disease a different pattern of clinical presentation of TTP might be forming based on early diagnosis. We reviewed the clinical presentation of 91 patients with a diagnosis of TTP in terms of pattern and duration of symptoms. Only 9% of patients (n=8) presented with the pentad. Duration of symptoms in all patients ranged between 1 and 60 days, with a mean of 9.3 days. Statistical analysis showed no correlation between the duration of symptoms prior to diagnosis and the presence of the pentand (mean of symptom duration in the pentad and non-pentad groups were 9.4 and 9.3 days with range of (3–30) and (1–60), respectively). The most common patterns of clinical presentation were in the form of constitutional symptoms (fatigue, malaise and generalized weakness), which were seen in 67% of patients (n=60), followed by neurological symptoms (mainly headache and confusion) in 51% of pateints (n=46). Gastrointestinal (GI) symptoms (nausea, vomiting or abdominal pain) were seen in 47% of patients (n=42). The most common triad of symptoms was neurological (headache and confusion), constitutional (fatigue, weakness and anorexia) and GI(nausea, vomiting and abdominal pain), which was present in 14% of patients (n=13). In terms of clinical outcome, there was also no correlation between the duration of symptoms and the clinical outcome (mean of symptom duration in the alive and dead groups were 9.2 and 10 days, with range of 1–60 and 1–21 days, respectively). There was no association between the presence or absence of the pentad and mortality, (25% (n=2/8) as compared to 11% (n=9/83), P-value of 0.248). On the other hand patients with poor outcome (defined as death) were less likely to present with constitutional and nonspecfic symptoms (27.3% (n=3/11) as compared to 72.5% (n=58/80), P-value of 0.005), As a conclusion, the pentad is a very rare presentation in TTP and the duration of symptoms prior to diagnosis does not predict its presence. While nonspecific and/or constitutional symptoms are the most common presentation, patients who do not present with such symptoms tend to do worse. Our study highlights the importance of defining the clinical presentation of this disease in a new and pertinent form that could help clinicians in the diagnosis and timely management. It also raises the question of prognsotic value of the clinical presentation in this potentially lethal disease. Disclosures: No relevant conflicts of interest to declare.

Abstract 5245 Introduction: The myelodysplastic syndromes (MDS) comprise a heterogeneous group of malignant stem cell disorders characterized by dysplastic and ineffective blood cell production and a variable risk of transformation to acute leukemia (AML). Treatment of MDS with immunomodulatory drugs and eryrthropoitin is a well known risk factor for venous thromboembolic disease (VTE) and has been cited in several reports. However, the frequency of VTE in MDS patients as an independent risk factor regardless of treatment modalities has not been characterized before. Methods: We reviewed all cases of MDS diagnosed between 2000 and 2010 in our institution and did a retrospective analysis on the incidence of VTE in these patients prior or during different modalities and also according to different MDS subtypes and prognostic scores. The study also sub-classified the VTE according to being provoked or not. Results: Between 2000 and 2010, 291 patients were diagnosed with MDS in our institution. Seventeen (5.8%) patients developed VTE. Of these patients, 4(23.5%) had unprovoked VTE compared to 13(76.5%) with provoked VTE. All patients with unprovoked VTE (100%) had their venous event prior to the MDS diagnosis with a median time of 154 days (range 5–150 days). 75% (3 patients) of these patients had an intermediate-1 IPSS group and 25% (1 patient) belonged to the low risk group. In the group of patients with provoked VTE, 6 (46.2%) patients had the venous event prior to MDS diagnosis with a median time of 434 days (range 90–943 days). 83.3% (5 patients) had an intermediate-1 IPSS group and 16.7% (1 patient) belonged to the high risk group. On the other hand, 7 (53.8%) patients had provoked VTE after the MDS diagnosis with a median time of 294 days (range 14–718 days). Treatment modalities during which these VTE occurred were as follows, 3 (42.8%) patients were on erythropoietin stimulating agents (ESA), 1 (14.2%) patient was on revlimid, 2 (28.5%) patients were on active chemotherapy, and 1 (14.2%) patient was on no treatment. The IPSS group distribution for post MDS diagnosis patients with provoked VTE was as follows: 0% in the low risk group, 28.5% belonged to intermediate-1 risk group, 28.5% belonged to intermediate-2 risk group and 43% belonged to the high risk group. In patients with unprovoked VTE, the median age was 77.7 years (range 76–81 years) with equal distribution between males and females (50% each). In patients with provoked VTE, the median age was 69.6 years (range 56–85 years) with 46.1% males and 53.9% females. Discussion: The results of this study shows an increased risk of VTE in patients with MDS (5.8%) compared to that in the general population which is reported to occur in about 1 per 1000 persons per year. It also shows that all the unprovoked VTE events occurred prior to the MDS diagnosis with a median time of around 5 months. Due to this finding, we recommend that part of the workup for unprovoked VTE in the elderly population (as the mean age for unprovoked VTE was 77.7 years) include at least a CBCD and a peripheral smear to rule out cytopenias or morphologic changes suggestive of MDS. Since 46.2% of the provoked VTE happened before MDS diagnosis, we also recommend checking a CBCD in the elderly population (as the mean age for provoked VTE was 69.6 years) due to its low cost and especially if the provoking factor for VTE was not strong enough. Since 42.8% of patients with provoked VTE were on ESA during the event, we encourage aggressive VTE prophylaxis in moderate/high risk situations for these patients. The study did not show a higher prevalence of the intermediate-2 or high IPSS risk groups among patients with unprovoked (0%) or provoked VTE (46%). However, we encourage further research to study the prognostic significance of VTE in MDS patients and its relationship to progression to AML and to overall survival. Conclusion: Our study showed that a higher risk of VTE is present in patients with MDS compared to the general population and we recommend that aggressive VTE prophylaxis be given in moderate/high risk situations especially for patients who are taking ESA. We also recommend further research to be done on the prognostic significance of VTE in patients with MDS regarding overall survival and progression to AML. Disclosures: No relevant conflicts of interest to declare.