tbuckley/cabot-search huggingface dataset:
Multiphoton microscopy of human prostatic and periprostatic tissue architecture for improving identification of tissue structures: Implication in nerve-sparing radical prostatectomy.
Authors: S. Grover, S. Mukherjee, P. Sooriakumaran, A. Srivastava, J. Sterling, M. Shevchuk, A. Tewari
Journal: Journal of Clinical Oncology (2011)

61 Background: Introperative differentiation of various tissue types would aid the surgeon during dissection in radical prostatectomy. Specifically, it will enhance decision-making with regards wide excision or nerve-sparing. We investigated the value of a novel imaging technology (multiphoton microscopy-MPM) for providing “virtual” histology. Methods: We imaged intraoperative surgical margins and biopsies, as well as radical prostatectomy sections. The imaging was carried out using intrinsic fluorescence and scattering properties of the tissues without any exogenous dye or contrast agent. The corresponding tissues were subjected to hematoxylin and eosin staining for histological confirmation of the structures. Results: High-resolution images of the periprostatic tissue, nerves, prostate capsule, underlying acini, and individual acinar cells were obtained at varying magnifications. There was good correlation between MPM and histology in identification of all anatomic structures. Conclusions: MPM provides microscopy level resolution in fresh human prostatic tissue, without the need for any extrinsic labeling agents or phototoxicity. It shows promise as a real-time intraoperative aid during radical prostatectomy. No significant financial relationships to disclose.
Abstract 3827: Histone methytransferase SMYD3 promotes invasion in ovarian carcinoma spheroids
Authors: Tianjiao Lyu, Nan Jia, Jieyu Wang, Robert C. Bast, Jan Pa Baak, Keqin Hua, Weiwei Feng
Journal: Cancer Research (2015)

Abstract Epithelial ovarian cancer (EOC) metastasis is a direct contributor to high recurrence and low survival for patients with this disease. The ovarian carcinoma spheroids are cells shed from primary tumors and aggregate in ascites, bearing the main responsibility for metastasis. “Epigenetic plasticity” refers to the capability of mammalian cells to alter their differentiation status via chromatin remodeling-associated alterations in gene expression. In the current study, we extend this attribute of epigenetic plasticity to aggressive ovarian cancer cells. Upon a simple alteration in the microenvironment, transition from two- to three-dimensional culture, we mimicked the in-vivo spheroids formation and found that a batch of histone markers and their enzymes experienced a marvelous elevation, among which, H3K4Me3 and its methyltransferase SMYD3 emerged as focal points for their concordant change. Using siRNA-SMYD3-depletion, we proved SMYD3 was attributed to the enhanced invasion and adhesion abilities in spheroids compared to monolayer cells. Gene array results further discovered that integrin-related molecules were suppressed after SMYD3 depletion. By ChIP assay, we confirmed SMYD3 and H3K4me3 binding increased at ITGA8, ITGB6 and ITGAM's promoter, indicating that SMYD3 regulated those downstream genes through methylating H3K4. At last, we reconfirmed the SMYD3 and integrin-mediated adhesion molecules were both activated in in vitro model, in vivo intraperitoneal xenograft model and EOC patients’ spheroids. Overall, our results indicated that ovarian cancer cell epigenetic plasticity reflects an inherent transcriptional flexibility for context-responsive alterations in phenotype and SMYD3 could be a promising epigenetic marker for anti-tumor treatment. Citation Format: Tianjiao Lyu, Nan Jia, Jieyu Wang, Robert C. Bast, Jan Pa Baak, Keqin Hua, Weiwei Feng. Histone methytransferase SMYD3 promotes invasion in ovarian carcinoma spheroids. [abstract]. In: Proceedings of the 106th Annual Meeting of the American Association for Cancer Research; 2015 Apr 18-22; Philadelphia, PA. Philadelphia (PA): AACR; Cancer Res 2015;75(15 Suppl):Abstract nr 3827. doi:10.1158/1538-7445.AM2015-3827
Next-Generation Sequencing of Immunoglobulin Heavy Chain Variable Region in Diagnostic Samples of Pediatric Acute Lymphoblastic Leukemia Identifies Hundreds of Clonal Subpopulations with Multiple Immunophenotypes
Authors: Charles Gawad, Malek Faham, Tom Willis, Martin Moorhead, Victoria Carlton, Jianbiao Zheng, Mark Klinger, Gary V Dahl, Norman J. Lacayo
Journal: Blood (2011)
Rapamycin in patients with advanced hepatocellular carcinoma progressing on prior antiangiogenic therapy.
Authors: Mohamed Bouattour, Johanna Wassermann, Chantal Dreyer, Valérie Vilgrain, Valerie Paradis, Eric Raymond, Sandrine J. Faivre
Journal: Journal of Clinical Oncology (2012)

356 Background: PI3K/Akt/mTOR is a critical survival pathway in hepatocellular carcinoma (HCC) often correlated with poor prognosis. Rapamycin (sirolimus) and its analogue everolimus, are specific mTOR inhibitors that showed promising antitumor activity in preclinical models and clinical cases of HCC Aims: To evaluate the safety and efficacy of rapamycin in patients (pts) with advanced HCC after failure or intolerance to prior antiangiogenic therapy Methods: In this retrospective cohort, we analyzed consecutive patients with progressive HCC after 1 to 3 lines of treatment including at least sorafenib. All pts received oral rapamycin at 20 to 30 mg once a week. Adverse events (AEs) were assessed using NCI-CTCAE v3.0, and tumor response was evaluated according to RECIST criteria. Results: Nine patients (F/M: 1/8) with compensated liver cirrhosis (Child A, n = 6; Child B7, n = 2) or no cirrhosis (n=1) and histologically proven HCC were included in this study. Overall, therapy with rapamycin was well tolerated. Most common toxicities were asthenia (grade 1-2: 5 pts) anaemia (all grade: 5 pts; grade 3: 2 pts ) and thrombocytopenia (grade 1-2: 2 pts). Liver function deterioration was observed in 2 pts with advanced cirrhosis (Child B7). Radiological evaluation was available in 6 pts. No objective tumor response was observed however stable disease ≥ 3 months was observed in 4 cases. Moreover, 2 pts showed stable disease at 6 months. Prolonged stabilization under rapamycin was observed in pts who were previously controlled at least for 6 months with sorafenib. Rapamycin was discontinued due to disease progression in 7/8 pts, toxicity in 1/8 pts. One pt shows ongoing long-lasting disease stabilization (8 + months). Conclusions: Rapamycin displayed an acceptable safety profile and may achieved disease stabilization in patients with heavily pretreated advanced HCC.
Temsirolimus in platinum-resistant metastatic urothelial cancer: A phase II trial.
Authors: Holger Gerullis, Christoph Eimer, Evangelos Georgas, Jens Willem Bangner, Thomas Otto
Journal: Journal of Clinical Oncology (2012)

302 Background: This investigator initiated phase II trial assessed temsirolimus, an inhibitor of mamalian target of rapamycin (mTOR), as second-line therapy in patients with locally advanced or metastatic TCCU after failure of platinum-containing therapy. Methods: 15 patients were enrolledin this phase II trial from June/2009 to June/2011. Primary endpoint was overall survival, as secondary endpoints we defined time to disease progression, safety and QoL along treatment. Patients with progressive TCCU after prior platinum-based chemotherapy received 25 mg of temsirolimus for 8 weeks. Evaluation for response was acomlished every 8 weeks according to the RECIST criteria, QoL assessment was done every 4 weeks using the QLC-C30 questionnaire, adverse events (AEs) were recorded and graded using NCI-CTC criteria. Results: We enrolled 15 patients in this study, of whom 14 (93%) were available for activity and safety assessment. We treated 10 (71%) male and 4 female (29%) patients median age was 64.7 years (45-76). Patients received on average 13 (3-15) infusions of temsirolimus. Median overall survival was 3.5 months (107 days). Median time to disease prgression was 2.5 months. Four patients with transient stable disease were observed. QoL assessment along treatment revealed a reduction of EORTC-QLQ-C30 (Global Health Status-Subscale) from initial 7.86 to 5.00. Temsirolimus was well tolerated, mostly observed were fatigue (Grade 3-4) in 2 patients, pancytopenia (Grade 3-4) in 2 patients, thrombopenia (Grade 3-4) in 2 patients. All other adverse events were grade 1-2 in nature. Conclusions: The primary endpoint was not met with an overall survival of 3.5 montsh only. Therefore the trial was closed after 14 patients. Temsirolimus seems to have poor activity in patients with progressive metastasized TCCU after platinum-containing first-line therapy.
Role of the Histone Deacetylase Inhibitor Givinostat (ITF2357) in Treatment of CRLF2 Rearranged Acute Lymphoblastic Leukemia
Authors: Angela Maria Savino, Jolanda Sarno, Luca Trentin, Margherita Vieri, Grazia Fazio, Michela Bardini, Cristina Bugarin, Gianluca Fossati, Kara L. Davis, Giuseppe Gaipa, Lueder Hinrich Meyer, Garry P. Nolan, Andrea Biondi, Geertruy Te Kronnie, Chiara Palmi, Giovanni Cazzaniga
Journal: Blood (2015)
A Phase 1 Trial of Imatinib Mesylate with Daunorubicin and Cytarabine for Patients with C-Kit Positive Relapsed AML.
Authors: Anjali Advani, Edward A. Copelan, Ronald Sobecks, Mikkael A Sekeres, Jennifer Bates, Mary Lynn Rush, Barbara Tripp, Elysa Noon, Matthew Howard, Tao Jin, Eric Hsi, Matt Kalaycio
Journal: Blood (2008)
Effective and Safe Deferasirox Treatment of Patients with Various anemia's and Transfusional Iron-Overload in the Medical Practice: Results From Two German Observational Studies
Authors: Christian Junghanss, Rudolf Schlag, Bernd Gaede, Matthias Moelle, Steffen Doerfel, Frank Heits, Ulrike Haus, Alexia Junkes, Oliver Leismann
Journal: Blood (2011)
High Relapse Rate Following Alemtuzamab Use in Allogeneic Transplants for Myeloid Hematologic Malignancies.
Authors: Thomas C. Shea, Jonathan Serody, Don Gabriel, Terrance Comeau, Donald Harvey, Robert Irons, David Morris, Andrew Sharf, Carol Krasnov, Cherie Dunphy, Nicholas Banderenko, Mark Brecher
Journal: Blood (2004)
Clinical risk factors as predictors of potential cardiotoxicity related to nonpegylated liposomal doxorubicin (NPLD) in metastatic breast cancer (MBC) patients (pts) previously treated with conventional anthracyclines (A).
Authors: E. Muñoz-Couselo, J. M. Perez-Garcia, C. Saura, M. Vidal, M. Bellet Ezquerra, J. Balmaña, B. Graña, P. Gomez Pardo, L. De Mattos-Arruda, S. Di Cosimo, S. Muñoz, O. Vidal, B. Garcia Castro, R. Espallargas, V. Esteban, J. Tabernero, J. Baselga, J. Cortes
Journal: Journal of Clinical Oncology (2011)

1074 Background: Few data are available on the cardiotoxic effect of NPLD in pts previously treated with A. Several studies have reported predictive factors associated with cardiotoxicity and A but there are no data identifying clinical risk factors related to the cardiotoxicity of NPLD in MBC pts previously treated with A. Methods: Inclusion criteria: Performance status (PS) 0-2,basal left ventricular ejection fraction (LVEF)>50%,previous(neo)adjuvant A and treatment with NPLD as metastatic therapy. Cardiac events: TYPEA signs/symptoms of cardiac heart failure plus grade 3/4 LVEFdecline;TYPEB asymptomatic LVEFdecline (absolute drop>10% plus final LVEF<50%,absolute drop>20% or LVEF<50%). Baseline clinical risk factors[age,gender, PS, body mass index (BMI), basal LVEF,dose of previous A, previous treatment with radiotherapy (RT) and trastuzumab (T), free interval of A] were examined for association with cardiotoxicity using univariate and multivariate analyses. Results: 95 pts were included. Median age was 48 years (96% females), median PS 2, median BMI 25, median basal LVEF 60’8% (IC95% 59’36-62’26) and median dose of A 300mg/m2(range 120–360).89’4% and 32.6%pts received previous RT and T treatment respectively,and 92% pts had free interval of A>12 months.No TYPEA cardiac events were observed and 13’7%pts experimented TYPEB cardiac events.In the univariate analysis only gender had statistically significant association with cardiotoxicity due to NPLD (Table). In the multivariate analysis, gender, previous T and PS were statistically associated with cardiac toxicity. Conclusions: This is the first report to show clinical risk factors related to cardiotoxicity of NPLD in pts previously treated with A. This treatment seems to be feasible with no relevant cardiac toxicity in the majority of pts, but gender, PS and previous T treatment should be considered as cardiotoxicity-related factors. Univariate analysis. P value Age 0.365 Gender 0.034 PS 0.059 BMI 0.761 Previous RT 0.603 Previous T 0.088 Previous dose of D 0.205 Basal LVEF 0.288 Interval free of D 0.862
Activity of Bruton's Tyrosine Kinase (BTK) Inhibitor Ibrutinib (PCI-32765) in B-Cell Acute Lymphoblastic Leukemia (B-ALL).
Authors: Ekaterina Kim, Stefan Koehrer, Nathalie Y Rosin, Deborah A. Thomas, Farhad Ravandi, Steven M. Kornblau, Hagop M. Kantarjian, Susan O'Brien, Zeev Estrov, Joseph J. Buggy, Jan A. Burger
Journal: Blood (2012)
Once Daily YM150, an Oral Direct Factor Xa Inhibitor, for Prevention of Venous Thromboembolism in Patients Undergoing Elective Primary Hip Replacement.
Authors: Bengt I. Eriksson, Alexander G.G. Turpie, Michael R. Lassen, Martin H. Prins, Giancarlo Agnelli, Peter Kälebo, Graham Wetherill, Lambertus Meems
Journal: Blood (2007)
Anti-Tumor Activity of Novel Small Molecule Wnt Signaling Inhibitor, CWP232291, In Multiple Myeloma
Authors: Joo Young Cha, Ji-Eun Jung, Kwan-Hoo Lee, Isabelle Briaud, Fnu Tenzin, Hee Kyung Jung, Younghee Pyon, Donghee Lee, Jae Uk Chung, Jong Heun Lee, Se-Woong Oh, Kyung Yun Jung, Jin Keon Pai, Kathy Emami
Journal: Blood (2010)
Azacitidine and Lenalidomide (Combined vs Sequential Treatment) in Higher-Risk Myelodysplastic Syndromes. Long-Term Results of a Randomized Phase II Multicenter Study
Authors: Carlo Finelli, Cristina Clissa, Matilde Follo, Marta Stanzani, Sarah Parisi, Sara Mongiorgi, Marilena Barraco, Paolo Avanzini, Costanza Bosi, Barbara Castagnari, Anna Candoni, Monica Crugnola, Maria Benedetta Giannini, Marco Gobbi, Giovanna Leonardi, Gian Matteo Rigolin, Domenico Russo, Patrizia Tosi, Giuseppe Visani, Lucio Cocco, Michele Cavo
Journal: Blood (2016)
Use of C-reactive protein to predict perinephric or renal sinus fat invasion in patients with clinical T1N0M0 renal cell carcinoma.
Authors: Y. Iimura, K. Saito, Y. Fujii, J. Ishioka, A. Iwai, N. Numao, Y. Okada, F. Koga, H. Masuda, K. Kihara
Journal: Journal of Clinical Oncology (2011)

385 Background: Indication of nephron sparing surgery (NSS) for renal cell carcinoma (RCC) has been extending, as NSS can be considered for not only cT1a but also cT1b tumors. However, some cT1 diseases have pathological perinephric or renal sinus fat invasion. It is useful to know the probability of the presence of fat invasion before surgery, but diagnostic ability of imaging studies such as CT for predicting pT3a disease is limited. Therefore, estimation of the risk for pT3a disease in cT1 RCC patients could be helpful. C-reactive protein (CRP) has been shown to be a strong prognostic factor in RCC patients, reflecting tumor aggressiveness. We evaluated the predictive ability of preoperative CRP for pT3a disease in cT1N0M0 RCC patients. Methods: Of consecutive 386 patients treated with radical nephrectomy for cT1a-bN0M0 RCC, 82 were excluded due to hemodialysis, inflammatory disease, bilateral synchronous renal tumors, synchronous other malignant disease or loss of each data. The remaining 304 patients constituted the current study cohort. Cut off point of CRP was 3 mg/L. Associations were analyzed between pT3a and preoperative factors including age, sex, BMI, laterality, tumor size, CRP, hemoglobin, platelet, LDH, ALP, calcium and albumin. We developed a multivariate model for predicting pT3a disease in cT1 RCC. Results: Of the 304 patients, 28 (9%) had pT3a disease. Of 43 patients with elevated CRP level, 10 (23%) had pT3a disease. In contrast, of the remaining 261 patients with non-elevated CRP level, 18 (7%) had (p=0.002). Univariate and then multivariate analyses identified age (p=0.021), tumor size (p=0.007), and CRP (p=0.024) as independent and significant predictors for pT3a disease. Incorporating these three factors, we developed a predictive model for pT3a disease with predictive accuracy of area under the ROC curve of 0.772. Conclusions: Preoperative CRP is an independent predictor for pT3a disease in cT1 RCC along with age and tumor size. The model could be useful to estimate the risk of the probability of fat invasion before surgery, especially NSS. No significant financial relationships to disclose.
Meta-Analysis of Defibrotide (DF) In the Treatment of Severe Hepatic Veno-Occlusive Disease (VOD) with Multi-Organ Failure (MOF) with Comparison to a Historical Control (HC).
Authors: Paul G Richardson, Gideon Steinbach, Nancy Kernan, Eva C. Guinan, Allen R. Chen, Paul L Martin, Amrita Krishnan, Sally Arai, Joel A. Brochstein, Stephan A. Grupp, Shin Mineishi, Amanda Termuhlen, Marcie Tomblyn, Joseph H. Antin, Carolyn Revta, Robin Hume, Joseph Massaro, Alison L. Hannah, Massimo Iacobelli, Robert J Soiffer, Defibrotide Study Group
Journal: Blood (2010)
The structure of polymer carriers controls the efficacy of the experimental combination treatment of tumors with HPMA copolymer conjugates carrying doxorubicin and docetaxel
Authors: Milada Šírová, Jiří Strohalm, Petr Chytil, Ondřej Lidický, Jakub Tomala, Blanka Říhová, Tomáš Etrych
Journal: Journal of Controlled Release (2016)
Contrast enhanced CT (CE-CT) changes and nephrometry down-scoring of unresectable primary renal cell carcinoma (RCC) tumors in patients (Pts) treated with neoadjuvant sunitinib.
Authors: M. Salem, S. N. Shah, L. S. Wood, P. Elson, A. Medsinge, S. Campbell, B. I. Rini
Journal: Journal of Clinical Oncology (2011)

299 Background: The impact of neoadjuvant sunitinib on CE-CT parameters and nephrometry score of primary RCC tumors remains unknown. Methods: Retrospective review of baseline and prenephrectomy CE-CT from a prospective phase II trial of neoadjuvant sunitinib (50 mg sunitinib continuous dosing) in unresectable primary RCC tumors with or without metastatic disease. CE-CT parameters and R.E.N.A.L. nephrometry score for each lesion were determined in pts who underwent subsequent surgery. RECIST and MASS criteria were used to assess primary tumor radiographic response. CT changes were analyzed using the sign test and Wilcoxon signed rank test. Results: Twenty nine pts were enrolled, of which 13 pts (85%M; median age 63y) underwent post-sunitinib resection of 16 primary tumors (3 pts had multifocal RCC). Post-therapy, 88% of tumors had decreased long diameter (median 32% decrease, p&lt;0.001 vs. baseline), 88% decreased attenuation (median 30 HU reduction, p=0.006) and 69% increased necrosis (p=0.001). 56% of tumors had a decrease in nephrometry score (median 1 point decrease; 10 to 9, p=004). At baseline, 81% of tumors were highly complex by nephrometry score; following therapy 46% of the highly complex tumors became moderately complex. At baseline 13 tumors abutted renal hilar vital structures, whereas following treatment 4 tumors demonstrated abutment. Adenopathy decreased (range, 23%-83%) in 4/4 patients with enlarged baseline lymph nodes, with complete resolution in 1 patient. RECIST objective response was seen in 38% and SD in 56% of primary tumors; 1 tumor had PD based on size despite &gt; 95% necrosis. MASS criteria response was favorable 38%, intermediate 62%. Two of four pts had reduction in extent of venous thrombus (1 pt from level 0 to resolved and 1 pt from level IV to II). Conclusions: Neoadjuvant sunitinib resulted in decreased size/attenuation, increased necrosis of the primary tumor and reduction in lymphadenopathy and venous thrombus in pts who underwent subsequent surgery. Sunitinib reduced the RENAL nephrometry score and facilitated nephrectomy, notably due to impact on tumor proximity to vital structures in the renal hilum. [Table: see text]
Comparison of prazosin with hydralazine in patients receiving hydrochlorothiazide. A randomized, double-blind clinical trial.
Journal: Circulation (1981)

The antihypertensive efficacy and the incidence of side effects of prazosin and hydralazine were compared in a randomized, double-blind trial in 232 adult male hypertensives who could not be controlled with hydrochlorothiazide alone. There were no significant differences between regimens in the percentage of patients who attained goal blood pressure (reduction of diastolic blood pressure to below 90 mm Hg and at least 5 mm less than the baseline randomization pressure), effect on pulse rate or the incidence or reasons for terminations. Absolute reduction of blood pressure was similar for both drugs except for sitting systolic pressure at 3 and 6 months, when prazosin effected a 3.7- and 3.6-mm Hg greater response (p less than 0.05). Orthostatic dizziness (p less than 0.005), sexual dysfunction (p less than 0.02), and nightmares (p less than 0.02) were more frequent with prazosin than with hydralazine; nevertheless, patient compliance was similar for both drugs. An unexpected finding was the lack of pulse rate increase associated with hydralazine, particularly in older patients.
Blocking Adhesion of Sickle Erythrocytes to Endothelial P-Selectin Using an RNA Aptamer.
Authors: Jun-ichi Nishimura, Angela D. Burnette, Sabah Oney, Milena Batchvarova, Martha Delahunty, Rahima Zennadi, Bruce A. Sullenger, Marilyn J. Telen
Journal: Blood (2007)
Genetic architecture distinguishes systemic juvenile idiopathic arthritis from other forms of juvenile idiopathic arthritis: clinical and therapeutic implications
Authors: Michael J Ombrello, Victoria L Arthur, Elaine F Remmers, Anne Hinks, Ioanna Tachmazidou, Alexei A Grom, Dirk Foell, Alberto Martini, Marco Gattorno, Seza Özen, Sampath Prahalad, Andrew S Zeft, John F Bohnsack, Norman T Ilowite, Elizabeth D Mellins, Ricardo Russo, Claudio Len, Maria Odete E Hilario, Sheila Oliveira, Rae S M Yeung, Alan M Rosenberg, Lucy R Wedderburn, Jordi Anton, Johannes-Peter Haas, Angela Rosen-Wolff, Kirsten Minden, Klaus Tenbrock, Erkan Demirkaya, Joanna Cobb, Elizabeth Baskin, Sara Signa, Emily Shuldiner, Richard H Duerr, Jean-Paul Achkar, M Ilyas Kamboh, Kenneth M Kaufman, Leah C Kottyan, Dalila Pinto, Stephen W Scherer, Marta E Alarcón-Riquelme, Elisa Docampo, Xavier Estivill, Ahmet Gül, Carl D Langefeld, Susan Thompson, Eleftheria Zeggini, Daniel L Kastner, Patricia Woo, Wendy Thomson
Journal: Annals of the Rheumatic Diseases (2016)

Objectives Juvenile idiopathic arthritis (JIA) is a heterogeneous group of conditions unified by the presence of chronic childhood arthritis without an identifiable cause. Systemic JIA (sJIA) is a rare form of JIA characterised by systemic inflammation. sJIA is distinguished from other forms of JIA by unique clinical features and treatment responses that are similar to autoinflammatory diseases. However, approximately half of children with sJIA develop destructive, long-standing arthritis that appears similar to other forms of JIA. Using genomic approaches, we sought to gain novel insights into the pathophysiology of sJIA and its relationship with other forms of JIA. Methods We performed a genome-wide association study of 770 children with sJIA collected in nine countries by the International Childhood Arthritis Genetics Consortium. Single nucleotide polymorphisms were tested for association with sJIA. Weighted genetic risk scores were used to compare the genetic architecture of sJIA with other JIA subtypes. Results The major histocompatibility complex locus and a locus on chromosome 1 each showed association with sJIA exceeding the threshold for genome-wide significance, while 23 other novel loci were suggestive of association with sJIA. Using a combination of genetic and statistical approaches, we found no evidence of shared genetic architecture between sJIA and other common JIA subtypes. Conclusions The lack of shared genetic risk factors between sJIA and other JIA subtypes supports the hypothesis that sJIA is a unique disease process and argues for a different classification framework. Research to improve sJIA therapy should target its unique genetics and specific pathophysiological pathways.