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Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a potentially life-threatening, idiosyncratic, acute adverse drug reaction. Fever, morbilliform cutaneous eruption, and eosinophilia are essential features for the diagnosis of this syndrome, along with significant multisystem involvement, hepatitis being the most common, followed by nephritis. The pathogenesis of DRESS syndrome is not yet fully understood. Several hypotheses have been proposed which support the involvement of an intricate interplay of multiple factors. We report a clinical case of DRESS syndrome with renal injury, induced by carbamazepine, in a patient with alcohol abstinence syndrome. In order to define the case, the RegiSCAR score and the Japanese Group score, used in the diagnosis of drug-induced hypersensitivity, were applied. DRESS syndrome is a potentially fatal disease, with a mortality that can reach up to 40% of cases. This condition endangers the patient's life by affecting the internal organs, mainly the liver, kidneys, heart, and lungs. Our case attempts to increase awareness among physicians about this serious disease and the importance of early diagnosis, especially since carbamazepine is a commonly used anticonvulsant drug.

We aimed to investigate the prevalence of US findings in the hand joints and related tendons and explore clinical and laboratory associations in SLE patients of the typical lupus clinic.One hundred consecutive SLE patients were enrolled in the study. Using B-mode and Doppler US, bilateral wrist, metacarpophalangeal and proximal interphalangeal joints were examined for synovitis and erosions, as well as for signs of hand tenosynovitis.US detected synovitis (grade 1-3) in 75% and erosive changes in 25% of the cohort. We found that clinical examination underestimated grade ≥2 synovitis by 13%, while US detected SH grade ≥2 in 10% of asymptomatic patients. The overall inflammatory burden, reflected by the US score, was associated with disease activity (respectively with CPR, SELENA-2K, MS-BILAG, and hypocomplementemia), as well as the presence of bone erosions. Rhupus patients had higher inflammatory markers, significantly more synovial hypertrophy, more erosions, more grade 3 tenosynovitis, and were more likely to receive methotrexate (p0.001) than patients with SLE arthritis, while patients with Jaccoud's arthropathy were more likely to accumulate damage. The dominant hand exhibited more inflammatory changes (respectively synovial hypertrophy grade ≥2) at both the wrist and MCP joints; however, handedness was not associated with structural damage.In conclusion: 1. joint involvement in SLE is frequent and underacknowledged; 2. the overall inflammatory burden is associated with systemic disease activity and joint damage; (3) destructive arthritis is more likely to occur in the context of concomitant RA or within an "RA-like" subtype of SLE arthropathy; 4. hand dominance is associated with synovitis, but not structural changes; 5. US assessment may help tailor the management of joint involvement, thus preventing joint damage and disability in SLE patients.

Gastrointestinal tract involvement is the most common visceral affectation in systemic sclerosis (SSc), but the manifestations may vary in extension and severity. Endoscopic and histopathological gastroesophageal findings were investigated in patients with SSc. A total of 79 consecutive patients with definite SSc were enrolled in a cross sectional study. Clinical data were collected, upper gastrointestinal endoscopy and biopsies from gastric mucosa were performed in all cases. Fifty-seven (72.1%) out of 79 SSc patients had gastroesophageal symptoms. The most frequent were dysphagia, present in 33 (41.7%) and gastroesophageal reflux symptoms in 23 (29.1%) patients. Out of the 79 patients, 22 were asymptomatic, but in 16 esophageal and gastric mucosa changes were endoscopically detected. Reflux esophagitis was found in 39 (49.3%) patients. The presence of esophageal manifestations was not related to the disease duration or with its other variables. Signs of gastritis were endoscopically described in 47 (59.4%) and confirmed on histopathologic examinations in 45 patients. In 31 patients without any endoscopic changes, 18 (22.7%) showed signs of gastritis on histopathologic examination. No significant statistical differences were found between symptomatic and asymptomatic patients or between those with limited cutaneous SSc and those with diffuse cutaneous SSc in terms of clinical, endoscopic or histopathological findings, except the higher proportion of hiatal hernia in symptomatic patients. The results of this study might suggest that upper gastrointestinal endoscopy should be performed during the early stage of the disease and then periodically in patients diagnosed with SSc, even in the absence of typical symptoms.

The diagnosis of rheumatoid arthritis (RA) is, most often, challenging, due to the variety of clinical features and to the absence of gold standard tests for diagnosing this disease. Early diagnosis as well as the identification of poor prognostic markers will lead to an early, appropriate treatment that will prevent the evolution towards a severe disease, offering the chance for a complete remission. Ultrasonography (US), used as an extension of clinical examination, helps in the early classification of the disease. Power Doppler is important for quantification of synovial inflammation, both at diagnosis and during therapy monitoring, but has limits in detecting blood flow in small vessels. Ultrasound contrast-agents improve visualization of vascularity and allow a better characterization and quantification of synovial pannus inflammation. We aim to discuss the role of contrast-enhanced ultrasonography (CEUS) in the diagnosis and follow-up of early RA.

The case of a male patient is reported, who presented with renal carcinoma and tumor thrombus in the inferior vena cava (IVC) extending from the right atrium (RA) to the bifurcation of IVC, common and external right iliac vein thrombosis, common and deep right femoral vein thrombosis, right popliteal vein thrombosis, with pulmonary and hepatic metastasis, treated with sorafenib. Renal cell carcinoma (RCC), the most common form of kidney cancer, occurs in 90% of cases and is nearly twice as common in men as in women. The diagnosis of RCC is accompanied by intravascular tumor thrombus in 10% of cases, and further extension of the tumor reaching RA is detected in approximately 1% of all patients. Therapy for advanced renal cell cancer has evolved considerably in the past decade, with new agents greeted like “buried treasure.” Before 2005, the widely used systemic agents were cytokine interferon alfa and interleukin-2, which yielded modest efficacy and substantial toxicity. Tyrosine kinase inhibitors (TKIs) increase progression-free survival and/or overall survival as both first-line and second-line treatments for metastatic RCC. Sorafenib is an oral multikinase inhibitor with activity against Raf-1 serine/threonine kinase, B-Raf, vascular endothelial growth factor receptor-2 (VEGFR-2), platelet-derived growth factor receptor (PDGFR), FMS-like tyrosine kinase 3 (FLT-3), and c-KIT.

Objective The present study aimed to assess the role of ultrasound (US) in the rapid classification of early rheumatoid arthritis (RA) by investigating whether the US features of inflammation and bone damage in early arthritis overlap with the actual clinical concept of classifying and identifying an aggressive disease. Methods Patients with recent-onset arthritis of at least 1 peripheral joint of the hands and/or the feet were consecutively included in this study. Clinical examination, laboratory tests, the Disease Activity Score 28 (DAS28), and the 2010 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for RA were assessed for all patients. Medication with disease-modifying antirheumatic drugs was recorded. Ultrasound assessment was performed at the following anatomical sites: wrists, metacarpophalangeal joints 2 to 5, and metatarsophalangeal joints 2 to 5 for assessing the presence/absence of synovial hypertrophy, the presence/absence of power Doppler signal, and the presence/absence of bone erosions. The US features of inflammation and bone damage were analyzed in comparison with the DAS28, with the presence/absence of rheumatoid factor and anti–cyclic citrullinated peptide, with the fulfillment of the new 2010 ACR/EULAR classification criteria, and with the initiated disease-modifying antirheumatic drug. The prescription of methotrexate was considered a marker of an aggressive disease. Results The US features of inflammation and bone damage correlated with the activity scores measured by the DAS28. The presence of US bone erosions overlapped with the presence of rheumatoid factor and anti–cyclic citrullinated antibodies. Synovial hypertrophy, intra-articular power Doppler signal, and bone erosions detected in at least 1 anatomical site were seen in patients fulfilling (77.7%) and in patients not fulfilling (72.7%) the 2010 ACR/EULAR classification criteria for RA. Synovial hypertrophy was found in at least 1 site in 83.3% and 58.8% of patients in whom methotrexate was prescribed and in whom methotrexate was not prescribed, respectively (P = 0.01). The US features were not correlated with the initiation of sulfasalazine or hydroxychloroquine. The patients presenting bone erosions received in significantly higher percentages the indication for methotrexate (50%) compared with sulfasalazine (20%), P = 0.03, or hydroxychloroquine (26%), P = 0.05. Conclusions The US features of inflammation might be of help in classifying early arthritis patients despite the presence of the immune markers for RA. Together with the US features of bone damage, these might be used as an indicator of a more aggressive disease. The absence of correlation between the US findings of RA and the 2010 ACR/EULAR classification criteria indicates a possible independent contribution of US in the understanding of the future evolution of these patients.

Aims: The aim of the study was to assess the evolution of time-intensity curves parameters of contrast-enhanced ultra- sonography (CEUS) after 6 months of conventional treatment in early arthritis patients with wrist involvement. Material and methods: Patients diagnosed with early rheumatoid arthritis or undifferentiated arthritis on the basis of 2010 ACR/EU- LAR classification criteria, with bilateral wrist arthritis and both radiocarpal (RC) and intercarpal (IC) synovial hypertrophy identified by grey-scale ultrasonography, were enrolled. Synovial hypertrophy was semi-quantitatively scored (grade 0-3) by grey-scale and by Power Doppler at wrist level. CEUS was performed using Sonovue. The region of interest was selected as the area corresponding to the synovial hypertrophy of the RC and IC joints. Time-intensity curves parameters were cal- culated with Contrast Dynamic Software. The minimum and the maximum values of Peak, area under the curve (AUC), and slope were selected for each patient at baseline and after 6 months of conventional treatment. The difference between the visits was noted as “Δ”. Results: Eleven patients fulfilled the inclusion criteria. Maximum time-intensity curves parameters’ difference significantly decreased at 6 months: Peak (30.00±5.90% vs 23.22±5.22%, p=0.008), AUC (1206.08±216.91%s vs 949.13±280.12%s, p=0.04) and slope (1.6 (1.4;2.3) %/s vs 1(0.7;1.2) %/s, p=0.03). Moderate correlations were found between maximum ΔPeak, maximum ΔAUC and maximum ΔPower Doppler grade (r=0.44, p=0.17; r=0.46, p=0.16, respec- tively). Conclusions: Peak and AUC for joints that had high baseline values significantly decreased following treatment with conventional synthetic drugs in EA patients with wrist arthritis. This decrease in Peak and AUC was moderately correlated with a decrease in US parameters. The joint with the highest values of these parameters may be used for evaluation of EA patients at follow-up.

Background Contrast enhanced ultrasonography (CEUS) may be superior to power Doppler US in detecting synovial inflammation by visualizing and quantifying low velocity blood flow at the microvascular level, thus making possible the differentiation of active synovitis from inactive intra-articular thickening. Time-intensity curve (TIC) parameters are descriptive and offer an objective quantification of vascularization in a specific anatomical area. Currently, the role of CEUS in daily rheumatological clinical practice is not well defined. [1,2] It is still not clear which CEUS abnormalities are of special interest for rheumatologists. Objectives The main objective of our study was to compare TIC parameters of CEUS at four joint levels in early arthritis (EA) patients having wrist involvement, with the purpose of characterizing CEUS parameters uniformity. As a secondary objective, we aimed to identify which TIC parameters vary most in the examined joints. Methods Patients diagnosed with active early rheumatoid or undifferentiated arthritis on the basis of the 2010 ACR/EULAR classification criteria, having bilateral wrist arthritis and both radiocarpal (RC) and intercarpal (IC) synovial hypertrophy identified by grey scale US, were consecutively enrolled. CEUS was performed using a second-generation contrast agent and quantified using dedicated software. The examinations started with the dominant hand. After 30 minutes, the other hand was examined, using the same protocol. The region of interest (ROI) was selected as the area corresponding to the synovial hypertrophy of the RC and IC joints. TIC parameters were calculated for the first minute of contrast enhancement and were analyzed in each of the four examined joints. Results Nineteen patients were included in the study. Right hand dominance was present in 94.7% of patients. Dominant hand expressed higher peak, AUC and slope values, compared to the other hand. The percentage difference of the parameters measured at right IC and the other joints varied between 12–32% for Peak (p=0.007), 22–38% for AUC (p=0.015) and 9–30% for slope (p=0.514). No differences for any of the TIC parameters between the other assessed joints were found. Conclusions CEUS behaviour was not uniform in the wrists joints of EA patients, TIC parameters revealing the highest degree of inflammation at IC joint of the dominant hand. Peak and AUC in CEUS, measured at this joint level, demonstrated the highest variation when compared to the other examined joints, suggesting their potential value for quantification of synovial inflammation. References Klauser AS, et al. Contrast-enhanced ultrasonography for the detection of joint vascularity in arthritis-subjective grading versus computer-aided objective quantification. Ultraschall Med. 2011 Dec;32 Suppl 2:E31–7. Platzgummer H, et al. Quantification of synovitis in Rheumatoid Arthritis: Do we really need quantitative measurement of contrast-enhanced ultrasound? European Journal of Radiology 2009;71:237–41. Acknowledgement The research was performed with financial support of the European Social Fund, Human Resources Development Operational Programme 2007–2013, POSDRU grant no. 159/1.5/S/138776. Disclosure of Interest None declared

Autoimmune hepatitis (AIH) is a chronic disorder characterized by persistent hepatocellular inflammation and necrosis. AIH overlap syndromes with other autoimmune diseases have been reported, including connective tissue diseases (CTD). Reports of AIH in systemic sclerosis (SSc), however, are scarce and have been particularly described in the limited SSc subtype. We report a case of systemic sclerosis-polymyositis overlap syndrome that developed AIH and subsequently, cerebral vasculitis. To our knowledge, this is the first report of such a complex mosaic of autoimmunity. We also review the literature regarding scleroderma-related AIH.

The degree of inflammation is the keystone of therapy management in rheumatoid arthritis and other arthritides. The assessment of synovial perfusion using power Doppler ultrasound is an important point in the quantification of the joint inflammation but it is limited by the subjectivity of the vascularization grading and incapacity to detect flows in very small vessels. Contrast agent improves the ultrasound ability to depict and quantify blood flows in synovitis. Contrast-enhanced ultrasonography (CEUS) better differentiates synovitis from collection and distinguishes the active synovitis from inactive fibrotic or necrotic pannus. Quantitative assessment of inflammation is possible analyzing the time-intesity curves and by the correct measurement of the synovial thickness. The additional informations and the diagnostic value of CEUS in arthritides are still controversial but its excellent imaging of synovial vessels open the way for further clinical applications. This review aims to discuss the actual knowledges of CEUS in inflammatory arthritis.

Musculoskeletal ultrasonography (MSUS) is currently used in daily medical practice, as an extension of the clinical examination. The development of training programmes for MSUS has increased the interest of physicians from different specialties in performing this exploration. We realized a survey in order to describe the current practice of MSUS in Romania, as well as determining physicians' preferences for MSUS training implementation.In 2010, 196 questionnaires were completed and returned at two congresses in Romania.Most of the participants were rheumatologists and radiologists, followed by physical medicine and rehabilitation doctors, internal medicine doctors, general practitioners and other specialists. 41% of respondents practice MSUS themselves. Doctors use MSUS as a diagnostic tool (95%) and for monitoring diseases (75%). Lack of training and lack of MSUS competence were the main reasons for not performing MSUS. The respondents expressed their preference for future training to be via a programme of regular, intensive courses and active participation in clinics where MSUS is performed. Most of the participants consider mentoring the assessement method of choice.The majority of participants in this survey use MSUS in the management of their patients. The results indicate participants' preferences on how training should be delivered in the future.

The assessment of patients with pulmonary focal abnormalities continues to represent an important problem in pulmonary diagnosis. A large number of lung nodules remains undetermined after bronchoscopy, radiological and CT imaging analysis. Ultrasound can contribute to the determination of the nature of peripheral lung tumors, serving as an important aid in decision-making. It helps the investigator to decide the appropriate subsequent step in order to establish the etiologic diagnosis: another imagistic investigation, ultrasonographic guided transthoracic needle biopsy or surgery. This pictorial essay summarizes the main sonographic signs of subpleural lung tumors and the differentiation of benign and malignant lesions.

The association of Crohn's disease and ankylosing spondylitis is described in up to 30% of cases. Treatment of both conditions is not an easy task. We present the case of a 53 year old woman, diagnosed with colonic Crohn's disease and ankylosing spondilitis, treated initially with increasing doses of sulphasalazine and moderate dose of corticosteroids, with the persistence of severe gastrointestinal and articular symptoms. She underwent therapy with tumor necrosis factor alpha (TNFalpha) inhibitor infliximab, with a spectacular improvement of symptoms, signs and quality of life.

Introduction Macrophage activation syndrome is a rare and potentially life threatening condition, triggered by various events like bacterial and viral illness and sometimes it can be a complication of childhood rheumatic disorders. Physical and laboratory results usually show non-remitting fever, hepatosplenomegaly, lymphadenopathy, bleeding diathesis, altered mental status, rash, pancytopenia and impaired liver and renal function tests, elevated triglyceride and serum ferritin levels. Corticosteroids and Cyclosporine are the drugs commonly used in its management. Early diagnosis and prompt treatment can be life saving. Objective To present a case of Macrofage activation syndrome triggered by a Mycoplasma pneumoniae infection. Background A 18 years old male pacient presented with high fever, tachycardia, lethargy, leucopenia and artharlgias after having a Mycoplasma pneumoniae infection treated in the Infectious disease department. Investigations Physical and laboratory results showed hepatosplenomegaly, high fever, tachycardia, low levels of Ig G and Ig M, leucopenia and the bone marrow aspiration showed cells phagocytosing hematopoietic elements. Also, on the basis of the effectuated investigations ( CBC; biochemistry and immunology tests; cultures of boold, urine, stool; abdomen and heart ultrasonography; thoracic CT) other infectious, malignant or rheumatic diseases were exclused. Diagnosis Macrophage activation syndrome triggered by a Mycoplasma pneumoniae infection. Management The patient9s disease was successfully controlled following the treatement with Methyprednisolone and Cyclosporine. Discussion and conclusion This case illustrates this rare, but potentially life-threatening condition sometimes associated with chronic rheumatic disorders with various triggering events like bacterial and viral illness. Differentiation from a disease flare of a rheumatic disorder is difficult and that is why pacients with this condition need further investigation and careful monitoring. In conclusion, MAS is a severe, potentially life-threatening complication of chronic rheumatic disease; therefore it is essential for clinicians to have a high threshold of suspicion, make an early diagnosis, and start prompt treatment to have success.

s / Pancreatology examination with bile from biliary tract or duodenum has been useful for the diagnosis of microlithiasis. Aims: We evaluated the reliability of bile samples collected directly from the biliary tract during ERCP for polarized microscopic examination. Patients m to identify factors favoring necrosis liquefaction. Patients & methods: Fifty-eight patients managed by a step-up approach or open necrosectomy in a tertiary referral center (2008 – 2012) were assigned to 3 groups, according to necrosis status at the intervention time: G1 – 26patients with solid necrosis; G2 – 28patients semisolid walled-off necrosis (WOPN); G3 – 4patients liquefied WOPN. Groups outcome were compared. Logistic regression was used to identify factors favoring necrosis liquefaction. Results: Median timing of intervention was 27days (G11⁄421, G21⁄431, G31⁄437days). Twenty patients died (34.48%): G11⁄453.8%, G21⁄421.4% and G31⁄40%. Open necrosectomy was performed in all G1 patients. Percutaneous catheter drainage (PCD) was primarily used in G2 and G3, with 41% and 100% success. When PCD failed, VARD or open necrosectomy followed. Mean hospital stay was G11⁄489days, G21⁄477days and G31⁄451days. Factors favoring necrosis liquefactions were: time from pancreatitis onset, age > 55 years, hypertriglyceridemic etiology, high amylase content, antibiotic prophylaxis > 2 weeks. Conclusion: Interventional debridement should be postponed in solid necrosis, where enzymatic debridement may be tried as suggested by one of the found factors (high amylase content). Antibiotic prophylaxis > 2 weeks favors liquefaction only by postponing necrosectomy. Time is the strongest factor favoring necrosis evolution to semisolid and liquefied WOPN status, when a step-up approach offers better survival and shorter hospital stay. PII-48 Abstract id: 161. Predictive markers for severe acute pancreatitis: A comparative prospective study within a representative cohort Hanna Sternby, Hannes Hartman, Dorthe Johansen, Stefan Appelros, Henrik Thorlacius, Sara Regn er. Department of Surgery, Sk ane University Hospital Malm€ o{, Lund University, Sweden Introduction: In order to predict severity of Acute Pancreatitis (AP) a large number of biomarkers have been studied retrospectively and in different cohorts. Aims: In this study promising biomarkers will be studied prospectively in a representative cohort of patients with AP aiming to enable comparisons of their predictive capacity in a clinical setting. Patients & methods: Patients with AP admitted to the Malm€ o University Hospital were consecutively included in the study. Blood samples were obtained on admission and daily up to 72 hours. Socioeconomic factors and information of importance for etiology and severity of AP were recorded in a database. Cut off values were set by reviewing the literature for the predefined biomarkers. Results: 202 patients with AP, 111 men and 91 women, with a median age of 66 years (19-97) were included in the study. 13,4% of the patients had severe AP according to the Atlanta -92 criteria while the corresponding figures for the Revised Atlanta-12 were 5,4%. 15, 3% were classified as Moderately Severe AP. Etiology was biliary in 51,5%, alcohol in 15,8%, unknown in 23,3% and of other origin in 9,4%. CRP on Day 3 had a median value of 377 mg/l in patients with SAP and 111 mg/l in patients with mild AP. Conclusion: The clinical data shows that the cohort has a representative composition in order to obtain reliable results concerning patients with AP. Preliminary data shows a significant difference in IL-6 and IL-8 between mild AP and severe AP. Further blood samples will be analysed during the first half of 2013. PII-49 Abstract id: 68. New classifications of severity of acute pancreatitis: Validation of determinant-based and revision of the Atlanta classification Enrique de-Madaria, Nelly G. Acevedo-Piedra, Neftali Moya-Hoyo, M onica Rey-Riveiro, Inmaculada Lopez-Font, Juan Martinez, Martinez Lluis. Unidad de Patologia Pancreatica, Hospital General Universitario de Alicante, Spain Introduction: The Atlanta classification was published in 1993; definitions were given regarding local and systemic complications, as well as severity of acute pancreatitis (AP). After 2 decades we have learned new concepts about the natural history of AP that have prompted a revision of the Atlanta classification. Two new classifications have been proposed. Aims: Our aim was to validate both new classifications. Patients & methods: We analyzed a prospective database which included every adult patient with AP admitted to a third level hospital between December 2007 and January 2013. Every CT scan was retrospectively reviewed according to new definitions. The classifications were validated in terms of outcomes: length of hospital stay, need for ICU admission, nutritional support, invasive treatment and mortality. Results: We analyzed 543 episodes of AP. The most frequent etiology was gallstones (59.5%), followed by alcohol (13.6%). Pancreatic necrosis was present in 66 (12.2%) of the patients, peripancreatic fat necrosis in 109 (20.1%), acute necrotic collections in 106 (19.5%), walled-off necrosis in 61 (11.2%), peripancreatic fluid collections in 98 (18%) and pseudocysts in 19 (3.5%). Transient organ failure was present in 31 patients (5.7%) and persistent organ failure in 21 (3.9%). Sixteen (2.9%) patients died. Determinant-based and Revised Atlanta classifications were associated to statistically and clinically relevant differences in length of hospital stay, need 13 (2013) S2–S98 S67