CTNI-31. RESECTION AND SURGICALLY TARGETED RADIATION THERAPY FOR TREATMENT OF LOCALLY RECURRENT GBM IN 28 PROSPECTIVELY TREATED PATIENTS

INTRODUCTION Recurrent GBM is a diffuse disease and resection (R) alone does not routinely provide durable local control (LC) or prolonged overall survival (OS). We hypothesized R plus immediate radiation (RT) utilizing a novel brachytherapy device might achieve more durable LC and thereby secondarily improve OS. METHODS From 2/2013-2/2018, locally recurrent GBM were treated in a single arm trial (ClinicalTrials.gov, NCT#03088579) of R plus immediate implantation of a surgically targeted radiation therapy (STaRT) device utilizing Cs-131 in bioresorbable collagen tiles (GammaTile, GT Medical Technologies, Tempe AZ USA). RESULTS 28 patients (pts) were treated, 20 at first recurrence (range 1-3). Median age 58 (range 21-80), KPS 80 (60-100), female:male ratio 10:18. Median OS was 10.7 mo., radiographic LC 8.8 mo., and no first failure was local. MGMT, KPS, and sex were non-predictive. Post hoc analysis disclosed after R+STaRT, 17 pts (54%) received > 1 cycle of Sys (“Sys+”) and 13 (46%) did not (“Sys- “). Sys was given as adjuvant, salvage, or both, either alone or in combination. 15 pts received bevacizumab (BEV), 12 temozolomide (TMZ) and 8 lomustine (CCNU). Median OS (mo.) for Sys+ vs. Sys- was 15.1/6.5 (hazard ratio (HR) .38, p=.017); OS for BEV+ vs. BEV- was 16.7/4.5 (HR .38, p=.017), TMZ+ vs. TMZ- 17.5/6.7 (HR .40, p=.025) and for CCNU+ vs. CCNU- 17.5/7.9 (HR .61, p=.25), respectively. LC was 11.4 mo. for Sys+ vs. 2.1 mo. for Sys- (HR .44; p=.16). Three attributed AE occurred, 1 wound infection requiring surgery and 2 radiation brain effects, managed medically. CONCLUSION Post hoc analysis suggests R+STaRT+Sys may have the potential to impact OS in locally recurrent GBM, possibly by allowing sufficient time for effective but biologically slower treatments to have an impact. FDA clearance was received in 2018 for recurrent intracranial neoplasms and in 2020 for newly-diagnosed malignant brain tumors.

Clinical and serological characteristics of systemic sclerosis: Experience of a tertiary care center in Pakistan

Objectives: This study aims to evaluate the clinical and serological characteristics of systemic sclerosis (SSc) in Pakistani population. Patients and methods: This prospective, cross-sectional study included a total of 38 patients (6 males, 32 females; mean age: 34.5±1.5 years; range, 16 to 60 years) with SSc who were admitted to our rheumatology clinic between November 2019 and January 2020. We evaluated the clinical, serological, and radiological features of SSc patients. Results: Thirty-four (89.5%) patients developed Raynaud phenomenon at the time of disease onset, while sclerodactyly was found in 34 (89.5%), digital ulcers in 25 (65.8%), and tendon friction rub in 12 (31.6%) patients. Interstitial lung disease was present in 30 (78.9%) patients with a higher prevalence in diffuse scleroderma (100%) than in limited scleroderma (70%) (p=0.01). Pulmonary hypertension was present in 18 patients with a significantly higher prevalence in diffuse disease (57.1%) than limited disease (11.8%) (p<0.01). Thirty (78.9%) patients had impaired pulmonary function tests. Fibromyalgia was present in seven (18.4%) patients, and depression was present in 10 (26.3%) patients. Antinuclear antibody (ANA) was positive in 30 (78.9%) patients. Anti-Scl-70 antibodies were present in 24 (63.2%) patients with a significant association with diffuse disease (85% vs. 35.3%, respectively; p<0.01). The anti-centromere antibodies (ACA) were present in 20 (52.6%) patients with a significantly higher rate in limited disease (94.2% vs. 19.0%, respectively; p<0.01). Conclusion: Scleroderma has a female preponderance. Raynaud phenomenon is the most initial clinical feature followed by other manifestations of a variable course and disease severity.

Cross Sectional Data on Profile of Coronary Artery Disease in Human Immunodeficiency Virus Infection Patients in a Tertiary Care Hospital of Central India

Background: Human immunodeficiency virus infection leads to a variety of Cardiovascular manifestations. Pericardial effusion, systemic hypertension, dilated cardiomyopathy, coronary artery disease, pulmonary hypertension, primary as well as secondary cardiac tumours etc are seen in about 30% of patients and the incidence has increased with improved longevity. Coronary artery disease ranging from subclinical atherosclerosis to coronary plaque rupture causing acute coronary syndrome have been documented. The main underlying pathophysiological process is a state of perennial inflammation which leads to initiation and acceleration of coronary atherosclerosis. Objective: To study the coronary angiographic profile in HIV patients. Methodology: HIV patients undergoing coronary angiography were assessed clinically, biochemically and finally by coronary angiography to see the coronary pathoanatomy. Results: Of the 37 HIV patients studied between Jan to Dec 2019, the mean age was 47 years.(19-66 years). Two thirds were males; most common traditional risk factor was dyslipidemia followed by hypertension. Most patients were already on ART and the CD 4 counts ranged from 67-366cells/cumm. Anterior wall MI and unstable angina were the most common clinical presentations. Coronary angiography revealed diffuse disease (both insignificant and significant) of the left anterior descending artery as the most common pattern of angiographic involvement. Conclusion: Coronary artery disease both subclinical and syndromic occurs with increased frequency in HIV patients. The dominant angiographic presentation correlates with the clinical presentation and in our study, left anterior descending artery was more frequently diseased as compared to other coronaries. The most common coronary angiographic pattern was the presence of ectasia and diffuse disease. Keywords: Human immunodeficiency virus, cardiovascular manifestations, coronary artery disease, coronary angiography.

Epidemiology and treatment of peripheral neuropathy in systemic sclerosis

Objective The epidemiology and treatment of peripheral neuropathy in systemic sclerosis (SSc) is poorly understood. The objectives of this study were to evaluate the incidence, prevalence, risk factors, and treatments of peripheral neuropathy in SSc. Methods A systematic review of Medline, Embase and CINAHL databases for literature reporting peripheral neuropathy in SSc was performed. Studies evaluating incidence, prevalence, risk factors, and treatments were synthesized. Meta-analysis using a random effects model was used to evaluate the prevalence of peripheral neuropathy. Results 113 studies reported 949 subjects with at least one type of peripheral neuropathy out of 2143 SSc patients studied. The mean age was 48.5 years. The mean time between SSc onset and detection of peripheral neuropathy was 8.85 years. The pooled prevalence of neuropathy was 27.4% (95%CI 22.4% - 32.7%). Risk factors for peripheral neuropathy in SSc included advanced diffuse disease, anticentromere antibodies, calcinosis cutis, ischemia of the vasa nervosum, iron deficiency anemia, metoclopramide, pembrolizumab, silicosis and uremia. There were 73 subjects with successful treatments (n=36 restoring sensation, n=37 restoring motor or sensorimotor function). Treatments included decompression surgery, prednisone, cyclophosphamide, carbamazepine, transcutaneous electrical nerve stimulation, tricyclic antidepressants and IVIG. Conclusion All-cause peripheral neuropathy is not uncommon in SSc. Compression neuropathies can be treated with decompression surgery. Observational data reporting immunosuppressive and anticonvulsants to treat peripheral neuropathy in SSc is limited and conflicting. This data provides the signal of effect to justify RCT to evaluate the efficacy of these interventions.

Need for analgesia after percutaneous liver biopsy: a real-life experience

Objective: To evaluate variables affecting the need for analgesia after ultrasound-guided percutaneous liver biopsy performed on an outpatient basis. Materials and Methods: This was a retrospective analysis of 1,042 liver biopsies performed between 2012 and 2018. The data collected included the age and sex of the patient, as well as self-reported pain in the recovery room, the pain treatment used, the indication for the biopsy, and the lobe punctured. As per the protocol of our institution, physicians would re-evaluate patients with mild pain (1-3 on a visual analog scale), prescribe analgesics for those with moderate pain (4-6 on the visual analog scale), and prescribe opioids for those with severe pain (7-10 on the visual analog scale). Results: The main indications for biopsy were related to diffuse disease (in 89.9%), including the follow-up of hepatitis C (in 47.0%) and suspicion of nonalcoholic steatohepatitis (in 38.0%). Pain requiring analgesia occurred in 8.0% of procedures. Of the 485 female patients, 51 (10.5%) needed analgesia, compared with 33 (5.9%) of the 557 male patients (p < 0.05). The need for analgesia did not differ in relation to patient age, the lobe punctured, or the indication for biopsy (nodular or diffuse disease). The analgesic most commonly used was dipyrone (in 75.9%), followed by paracetamol alone (16.4%) and their combination with opioids (7.6%). Conclusion: Ultrasound-guided percutaneous liver biopsy is safe and well tolerated. Postprocedural pain does not correlate with the lobe punctured, patient age, or the indication for biopsy and appears to affect more women than men.

Prospective comparison of whole body MRI and FDG PET/CT for detection of multiple myeloma and correlation with markers of disease burden: Results of the iTIMM trial.

8012 Background: Early and sensitive detection of bone marrow disease and stratified patient management according to clinical risk can confer survival advantages in multiple myeloma (MM). Whole body MRI (WB MRI) and Fluorodeoxyglucose (FDG) PET/CT are included in international guidelines for imaging in patients with a suspected diagnosis of MM. However prospective studies comparing detection of MM by contemporary WB MRI as per recent MY-RADS consensus against FDG PET/CT are lacking. We report here protocol-defined endpoints from the prospective iTIMM (NCT02403102) study, comparing WB MRI and PET/CT, their relationship with serum and bone marrow estimates of disease burden, as well as molecular tumor characteristics. Methods: Patients with newly diagnosed MM or at first relapse planned to receive chemotherapy and autologous stem cell transplantation were enrolled in iTIMM. Matched baseline WB MRI and FDG PET/CT were performed and baseline clinical data including tumor genetics collected. Scans were double reported for presence of focal and diffuse disease by expert MRI and PET/CT radiologists, blinded to each other’s assessment. Paired methods were used to compare burden and patterns of disease on WB MRI compared to FDG PET/CT at baseline. Primary and secondary trial endpoints include relationship between post-treatment WB MRI response and progression-free survival, for which follow-up is ongoing. Exploratory endpoints include comparison of baseline WB MRI and PET/CT and their correlation with laboratory parameters, for which data is complete and reported here. Results: From May 2015 to March 2018, sixty patients (35 male; mean age 60 years) underwent baseline WB MRI as per MY-RADS consensus and FDG PET/CT. At least one focal lesion was detected in 50/60 patients (83.3%) by WB MRI and in 36/60 patients (60%) by PET/CT. WB MRI was more sensitive ( P< 0.05) across anatomical regions except for ribs and cervical spine. Four patients in our study showed two or more focal lesions ≥5 mm only on WB MRI but not PET/CT. All lesions detected by WB MRI but not PET/CT resolved in follow-up scans after treatment, excluding false positives. In 49/60 (81.7%) patients, diffuse disease was detected by WB MRI, compared to 10/60 (16.7%) by PET-CT; WB MRI was more sensitive across all anatomical areas ( P< 0.05). Plasma cell infiltration and paraprotein levels were significantly higher for patients with diffuse disease on WB MRI, but not on PET/CT. All genetically high-risk tumours, defined by t(4;14), t(14;16), del(1p), gain(1q) or del(17p), showed diffuse infiltration on WB MRI. Conclusions: WB MRI increases detection of focal and diffuse disease compared with FDG PET/CT, including improved detection of focal lesions meeting criteria for active disease as per International Myeloma Working Group diagnostic criteria, proposing it as a gold standard for tumor imaging in MM. Clinical trial information: NCT02403102.

AB0093 CLINICAL AND SEROLOGICAL CHARACTERISTICS OF SYSTEMIC SCLEROSIS: EXPERIENCE FROM A TERTIARY CARE CENTER IN PAKISTAN

Background:Systemic sclerosis (SS) is less studied Connective tissue disease in our population. It is characterized by different manifestations which if left undiagnosed and untreated lead to serious complications. The hallmark of this disease is fibrosis of various organs including skin and also involving pulmonary, gastrointestinal and cardiovascular system. We aim to evaluate the clinical and serological characteristics of SS in our population. In addition, we evaluated the prevalence of Fibromyalgia and Depression in patients with SS.Objectives:We aimed to determine Clinical and Serological Characteristics of Systemic Sclerosis visiting our department of Rheumatology, Fatima Memorial hospital, Lahore.Methods:It is a cross-sectional study of 38 patients visiting outdoor and indoor in Rheumatology department of Fatima Memorial Hospital from September 2019 to January 2020. All systemic sclerosis patients with age 16 years or above were included in this study. After taking informed consent, we filled the performas to record all the required information. We evaluated the clinical, serological and radiological features of Systemic Sclerosis.Results:A total of 38 patients were included in the study with mean age was 34.47 ± 1.53 years. Female gender comprised of 32(84.2%), with male to female ratio of 1:5.3. Thirty-four (89.5%) had developed Raynaud phenomenon and sclerodactyly was found in 34(89.5%), digital ulcers in 25 (65.8%), and tendon friction rub in 12 (31.6%) patients. Skin tightness proximal to elbow was present in 9 (23.7%). Microstomia (decreased mouth opening) was present in 32(84.2%) patients. Twenty-eight (73.7%) patients had arthritis.Respiratory symptoms comprised of shortness of breath in 36(94.7%), palpitation in 24 (63.2%) and chest pain in 7(18.4%). Gastrointestinal (GI) symptoms comprised of regurgitation in 31 (81.5%) and dysphagia in 14 (36.8%). Interstitial lung disease was present in 30 (78.9%) patients, with higher prevalence in diffuse scleroderma (100%) than in limited scleroderma (70%) (p=0.01). Pulmonary hypertension was present in 18 patients, with significantly higher prevalence in diffuse disease (57.1%), that was secondary to interstitial lung disease and in limited disease it was found in (11.8%) (p<0.01). Thirty (78.9%) patients were found to have restricted disease on pulmonary function tests.Obstetric history showed a higher prevalence of primary infertility in at least 6 (15.8%) patients, with significantly higher prevalence in limited systemic sclerosis disease as compared to diffuse disease (23.5% vs 9.5%, and p=0.05). Fibromyalgia diagnosed as per ACR criteria was present in 7 (18.4%) patients, and depression assessed by Hospital Anxiety and depression (HADS) score was present in 10 (26.3%) patients.Anti nucleic acid antibody (ANA) was found positive in 30 (78.9%) patients. Anti Scl-70 antibodies were in 24 (63.2%) patients, with significant association with diffuse disease (85% vs 35.3% and p<0.01), while anti centromere antibodies were present in 20 (52.6%) patients; significantly higher in limited disease (94.2% vs 19.0%, and p<0.01).Conclusion:Scleroderma is a very important, autoimmune multisystem disease. It has female preponderence. Raynaud phenomenon is the most initial clinical feature followed by other manifestations of variable course and disease severity. Interstitial lung disease and pulmonary hypertension were the most important complication found in our patients which has poor prognosis. So, It is imperative to early diagnose and treat the disease manifesations to prevent future complications.Keywords:Scleroderma, Systemic sclerosis.Disclosure of interest:Sadia Asif: None declared, Muhammad Haroon: None declared, Dr Asadullah Khan: None declared, Dr. Muhammad Faiq: None declareDOI: 10.1136/annrheumdis-2021-eular.1635

Angio-Based Fractional Flow Reserve, Functional Pattern of Coronary Artery Disease, and Prediction of Percutaneous Coronary Intervention Result: a Proof-of-Concept Study

Purpose Wire-based coronary physiology pullback performed before percutaneous coronary intervention (PCI) discriminates coronary artery disease (CAD) distribution and extent, and is able to predict functional PCI result. No research investigated if quantitative flow ratio (QFR)–based physiology assessment is able to provide similar information. Methods In 111 patients (120 vessels) treated with PCI, QFR was measured both before and after PCI. Pre-PCI QFR trace was used to discriminate functional patterns of CAD (focal, serial lesions, diffuse disease, combination). Functional CAD patterns were identified analyzing changes in the QFR virtual pullback trace (qualitative method) or after computation of the QFR virtual pullback index (QVPindex) (quantitative method). QVPindex calculation was based on the maximal QFR drop over 20 mm and the length of epicardial coronary segment with QFR most relevant drop. Then, the ability of the different functional patterns of CAD to predict post-PCI QFR value was tested. Results By qualitative method, 51 (43%), 20 (17%), 15 (12%), and 34 (28%) vessels were classified as focal, serial focal lesions, diffuse disease, and combination, respectively. QVPindex values >0.71 and ≤0.51 predicted focal and diffuse patterns, respectively. Suboptimal PCI result (post-PCI QFR value ≤0.89) was present in 22 (18%) vessels. Its occurrence differed across functional patterns of CAD (focal 8% vs. serial lesions 15% vs. diffuse disease 33% vs. combination 29%, p=0.03). Similarly, QVPindex was correlated with post-PCI QFR value (r=0.62, 95% CI 0.50–0.72). Conclusion Our results suggest that functional patterns of CAD based on pre-PCI QFR trace can predict the functional outcome after PCI. Clinical Trial Registration ClinicalTrials.gov, number NCT02811796. Date of registration: June 23, 2016.