scholarly journals FRI0248 PROGNOSTIC FACTORS FOR STEROID-FREE REMISSION IN PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOPATHIES: IMPORTANCE OF ANTHROPOMETRIC MEASUREMENTS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 708.1-708
Author(s):  
J. S. Lee ◽  
S. H. Nam ◽  
S. J. Choi ◽  
W. J. Seo ◽  
S. Hong ◽  
...  

Background:Several studies have been conducted on factors associated with mortality in idiopathic inflammatory myopathies (IIM), but few studies have assessed prognostic factors for steroid-free remission in IIM.Objectives:We investigated the various clinical factors, including body measurements, that affect IIM treatment outcomes.Methods:Patients who were newly diagnosed with IIM between 2000 and 2018 were included. Steroid-free remission was defined as at least three months of normalisation of muscle enzymes and no detectable clinical disease activity. The factors associated with steroid-free remission were evaluated by a Cox regression analysis.Results:Of the 106 IIM patients, 35 displayed steroid-free remission during follow-up periods. In the multivariable Cox regression analyses, immunosuppressants’ early use within one month after diagnosis [hazard ratio (HR) 6.21, 95% confidence interval (CI) 2.61–14.74, p < 0.001] and sex-specific height quartiles (second and third quartiles versus first quartile, HR 3.65, 95% CI 1.40–9.51, p = 0.008 and HR 2.88, 95% CI 1.13–7.32, p = 0.027, respectively) were positively associated with steroid-free remission. Polymyositis versus dermatomyositis (HR 0.21, 95% CI 0.09–0.53, p = 0.001), presence of dysphagia (HR 0.15, CI 0.05–0.50, p = 0.002) and highest versus lowest quartile of waist circumference (WC) (HR 0.24, 95% CI 0.07–0.85, p = 0.027) were negatively associated with steroid-free remission.Conclusion:The early initiation of immunosuppressant therapy, type of myositis and presence of dysphagia are strong predictors of steroid-free remission in IIM; moreover, height and WC measurements at baseline may provide additional important prognostic value.Disclosure of Interests:None declared

2020 ◽  
Vol 12 ◽  
pp. 1759720X2093682
Author(s):  
Jung Sun Lee ◽  
Jung Eun Lee ◽  
Seokchan Hong ◽  
Chang-Keun Lee ◽  
Bin Yoo ◽  
...  

Background: Several studies have been conducted on factors associated with mortality in idiopathic inflammatory myopathies (IIMs), but few studies have assessed prognostic factors for steroid-free remission in IIM. We investigated the various clinical factors, including body measurements, that affect IIM treatment outcomes. Methods: Patients who were newly diagnosed with IIM between 2000 and 2018 were included. Steroid-free remission was defined as at least 3 months of normalisation of muscle enzymes and no detectable clinical disease activity. The factors associated with steroid-free remission were evaluated by a Cox regression analysis. Results: Of the 106 IIM patients, 35 displayed steroid-free remission during follow-up periods. In the multivariable Cox regression analyses, immunosuppressants’ early use within 1 month after diagnosis [hazard ratio (HR) 6.21, 95% confidence interval (CI) 2.61–14.74, p < 0.001] and sex-specific height quartiles (second and third quartiles versus first quartile, HR 3.65, 95% CI 1.40–9.51, p = 0.008 and HR 2.88, 95% CI 1.13–7.32, p = 0.027, respectively) were positively associated with steroid-free remission. Polymyositis versus dermatomyositis (HR 0.21, 95% CI 0.09–0.53, p = 0.001), presence of dysphagia (HR 0.15, CI 0.05–0.50, p = 0.002) and highest versus lowest quartile of waist circumference (WC; HR 0.24, 95% CI 0.07–0.85, p = 0.027) were negatively associated with steroid-free remission. Conclusion: The early initiation of immunosuppressant therapy, type of myositis and presence of dysphagia are strong predictors of steroid-free remission in IIM; moreover, height and WC measurements at baseline may provide additional important prognostic value.


2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e15585-e15585
Author(s):  
Anatoly Bulanov ◽  
Mikhail Fedyanin ◽  
Alexey Tryakin ◽  
Ilya Pokataev ◽  
Tatiana Zakharova ◽  
...  

e15585 Background: According to IGCCCG, pts with MNGCT belong to poor prognostic group. But, there are no independent prognostic factors which could determine prognosis in this group of pts. We retrospectively studied prognostic factors in pts with MNGCT. Methods: We analyzed data on 61 pts with MNGCT, who were treated in our department during 1986-2011. Median age was 23 years (range: 18-44). Median follow-up time was 52 months (range 4-180). Biopsy was performed in 35/61 (57.4%) pts before treatment. At the beginning of therapy median AFP was 3,360 IU/ml (range: 1-300,000), HCG – 4.5 mIU/ml (range: 0.1 to 326210), LDH – 791 U/l (range: 249-4,475). Conventional induction chemotherapy (CT): classical BEP regimen – 23 (37.8%) pts, T-BEP – 17 (27.8%) pts, CPOB – 17 (27.8%) pts, accelerated (two-weekly) BEP – 4 (6.6%) pts. After CT, residual tumor was resected in 28 (45.9%) pts. Multivariate Cox regression analysis was performed to determine independent factors, which influenced on overall survival (OS). Results: Marker-negative objective response was revealed in 40/61 (65.6%). Progression disease during induction CT was detected in 21 (34.4%) pts. 5-years OS was 44% for all pts. Multivariate analysis revealed the following independent negative prognostic factors: age ≥ 24 (р=0.08, HR 1.9, 95%CI 0.92-4.1), size of the primary mediastinal tumor ≥ 19 cm (р= 0.03, HR 5.8, 96%CI 1.85-18.67). Median OS hasn’t been reached and 3-year OS was 62% in pts with good prognosis (age < 24 years and/or size of mediastinal tumor < 19 cm) vs. 15 months and 30% in pts with poor prognosis (р=0.02, HR 0.42, 95%CI 0.19-0.87) respectively. Conclusions: Age ≥ 24 and size of the primary mediastinal tumor ≥ 19 cm are independent negative prognostic factors in pts with MNGCT. These factors could be used as strata in clinical trials. However, this tendency has to be confirmed in large series of pts.


2019 ◽  
Vol 6 (2) ◽  
pp. 236
Author(s):  
Srujith C. H. ◽  
Kavitha Mohanasundaram ◽  
Jagadeesan M. ◽  
Halleys Kumar E. ◽  
Kannan R. ◽  
...  

Background: Idiopathic inflammatory myopathies (IIMs) are a group of chronic systemic autoimmune diseases characterized by proximal muscle weakness and elevated muscle enzymes. Aim and Objective was to analyze the demographic profile of patients with idiopathic inflammatory myopathies (IIM).Methods: This was a cross sectional observational study conducted over a period of two years (2016-2018). After obtaining institutional ethical committee clearance, informed consent from patients. 16 patients who fulfilled the criteria were included in the study. The demographic and the clinical data were analysed.Results: The mean age was 47.3±11.2 years. The study showed female predominance. ANA was positive in 11(68.7%) patients. Among the 16 patients, 5 (31.25%) had polymyositis and 11 (68.7%) had dermatomyositis. The median enzymes levels were creatinine kinase 1134 U/L, lactic dehydrogenase 477U/L, ALT (alanine aminotransferase) 154 IU/L, AST (aspartate aminotransferase) 236IU/L. Raynaud's phenomenon was seen in 37.5%. In our study, 31.25% had hypothyroidism and 6.25% had diabetic mellitus. On follow up 37.5% developed interstitial lung disease (ILD) and 18.75% were found to have malignancy.Conclusions: Steroids and immunomodulators are the mainstay of treatment in patients with idiopathic inflammatory myositis. All our patients improved with steroids. It is important to evaluate these patients during early stages and follow up to prevent complications.


Neurosurgery ◽  
2013 ◽  
Vol 73 (5) ◽  
pp. 816-824 ◽  
Author(s):  
Dale Ding ◽  
Chun-Po Yen ◽  
Zhiyuan Xu ◽  
Robert M. Starke ◽  
Jason P. Sheehan

Abstract BACKGROUND: Eloquent intracranial arteriovenous malformations (AVMs) located in the primary motor or somatosensory cortex (PMSC) carry a high risk of microsurgical morbidity. OBJECTIVE: To evaluate the outcomes of radiosurgery on PMSC AVMs and compare them with radiosurgery outcomes in a matched cohort of noneloquent lobar AVMs. METHODS: Between 1989 and 2009, 134 patients with PMSC AVMs underwent Gamma Knife radiosurgery with a median radiographic and clinical follow-up of 64 and 80 months, respectively. Seizure (40.3%) and hemorrhage (28.4%) were the most common presenting symptoms. Pre-radiosurgery embolization was performed in 33.6% of AVMs. Median AVM volume was 4.1 mL (range, 0.1-22.6 mL), and prescription dose was 20 Gy (range, 7-30 Gy). Cox regression analysis was performed to identify factors associated with obliteration. RESULTS: The overall obliteration rate, including magnetic resonance imaging and angiography, after radiosurgery was 63%. Obliteration was achieved in 80% of AVMs with a volume less than 3 mL compared with 55% for AVMs larger than 3 mL. No previous embolization (P = .002) and a single draining vein (P = .001) were independent predictors of obliteration on multivariate analysis. The annual post-radiosurgery hemorrhage risk was 2.5%. Radiosurgery-related morbidity was temporary and permanent in 14% and 6% of patients, respectively. Comparing PMSC AVMs with matched noneloquent lobar AVMs, the obliteration rates and clinical outcomes after radiosurgery were not statistically different. CONCLUSION: For patients harboring PMSC AVMs, radiosurgery offers a reasonable chance of obliteration with a relatively low complication rate. Eloquent location does not appear to confer the same negative prognostic value for radiosurgery that it does for microsurgery.


2014 ◽  
Vol 8 (11-12) ◽  
pp. 768 ◽  
Author(s):  
J. Matthew J. Andrews ◽  
James E. Ashfield ◽  
Michael Morse ◽  
Thomas F. Whelan

Introduction: We assessed oncological outcomes of active surveillance (AS) using a community database and identified factors associated with disease reclassification on surveillance biopsy.Methods: A retrospective review was performed on 200 men on AS. Prostate-specific antigen (PSA) was measured every 3 to 6 months. Prostate biopsies were performed every 1 to 4 years, and at the individual physician’s discretion. Disease reclassification was defined as clinical T1 to cT2 progression, or histologically as >2 cores positive, Gleason score >6, or >50% core involvement on surveillance biopsy. Multivariate Cox regression analysis evaluated factors associated with disease reclassification. Kaplan-Meier survival curves were plotted.Results: We assessed a heterogeneous cohort of 86 patients, with a median age 67.2 years, who received ≥1 surveillance biopsies. The median follow-up was 5.2 years. The median times to first and second surveillance biopsies were 730 and 763 days, respectively. Overall, 47% of patients were reclassified on surveillance biopsy after a median 2.1 years. Factors associated with disease reclassification were PSA density >0.20 (p < 0.0001, hazard ratio [HR] 4.55, 95% confidence interval [CI] 2.116–9.782) and ≥3 positive cores (p = 0.0152, HR 3.956, 95% CI 1.304–12.003) at diagnosis, and number of positive cores on surveillance biopsy. In total, 25 (29%) patients received delayed intervention, with a median time to intervention of 2.6 years. The median time on AS was 4.4 years, with an overall survival of 95% and prostate-specific survival of 100%.Conclusions: Our community study supports AS to reduce over treatmentof prostate cancer. PSA density >0.20 and ≥3 cores positive are associated with disease reclassification on surveillance biopsy.


2013 ◽  
Vol 95 (4) ◽  
pp. 252-257 ◽  
Author(s):  
B Ip ◽  
M Jones ◽  
P Bassett ◽  
R Phillips

Introduction The aim of this study was to establish patient and procedural factors associated with the development of an unhealed perineum in patients undergoing a proctectomy or excision of an ileoanal pouch. Methods A review of 194 case notes for procedures performed between 1997 and 2009 was carried out. All patients had at least 12 months’ follow-up. Univariate and multivariate analyses were performed in 16 parameters. For those patients who developed an unhealed perineum, Cox regression analysis was performed to establish healing over a 12-month period. Results Two hundred patients were included in the study, of which six had unknown wound status and were subsequently excluded. This left 194 study patients. Of these, 86 (44%) achieved primary wound healing with a fully healed perineum and 108 (56%) experienced primary wound failure. With reference to the latter, 63 (58%) healed by 12 months. Comparing patients with an initially intact perineum with those with initial wound failure showed pre-existing sepsis was highly relevant (odds ratio: 4.32, 95% confidence interval [CI]: 2.16–8.62, p<0.001). In patients who had an unhealed perineum initially, perineal sepsis and surgical treatment were both significantly associated with time to healing (hazard ratio [HR]: 0.54, 95% CI: 0.31–0.93, p=0.03; and HR: 0.42, 95% CI: 0.21–0.84, p=0.01). Conclusions The presence of pre-existing perineal sepsis is associated with an unhealed perineum following proctectomy in inflammatory bowel disease (IBD) and non-IBD surgery. Further studies are indicated to establish perineal sepsis as a causative factor.


2018 ◽  
Vol 26 (5) ◽  
pp. 320-324 ◽  
Author(s):  
Juan Pablo Zumárraga ◽  
Felipe Augusto Ribeiro Batista ◽  
André Mathias Baptista ◽  
Marcelo Tadeu Caiero ◽  
Luis Pablo de la Rosa Martino ◽  
...  

ABSTRACT Objective: Myxofibrosarcoma (MFS) is a common soft tissue sarcoma (STS) that affects the extremities in elderly patients. The objective was to analyze the prognostic factors and outcomes of patients with MFS treated at a single institution. Methods: We retrospectively reviewed the records of 75 patients with MFS. We compared age, sex, tumor size and location, grade and stage of the disease. Median age was 49.7 years (range, 1 to 88 y). Location: upper extremity (25.4%), lower extremity (66.6%) and pelvis (8%). Patients had high-grade tumors in 46.7% of the reports. Margins were negative in 76% of the cases. Bivariate Cox regression analysis was used to determine associations between clinical and treatment factors with local recurrence (LR). Results: Median follow-up time was 30.7 months (range, 1.8 to 383.8 m). We found 26.7% of LR. Distant metastasis (DM) was reported in 27 (36%) patients. Lung was the most common site of DM, reported in 92.6% of patients. Overall survival (OS) with metastasis was 21.2 months (range, 4.8 to 114.8 m). Predictors of OS were grade, LR (hazard ratio [HR] 5.13, 95% confidence interval, 2.15-12.24, P <0.001), and DM (HR 540.97, 95% confidence interval, 5.04-58112.03, P< 0.001). Conclusions: Tumor grade, LR, positive margins and DM were significant predictors of poor OS prognosis. Level of Evidence IV, Case Series.


2019 ◽  
Author(s):  
Victor Tak Lung Wong ◽  
Ho SO ◽  
Ronald Man Lung Yip

Abstract Background The aims of the study were to investigate the prevalence of myositis specific autoantibodies (MSAs) and their associated complications in a cohort of patients with idiopathic inflammatory myopathies (IIMs). Methods A total of 201 consecutive patients with IIMs being followed up in the Rheumatology clinics of the participating regional hospitals in Hong Kong from July 2016 to January 2018 were recruited. Clinical characteristics, treatment history and disease complications were documented. Immunoblot assay was used to detect the MSAs.Results Out of the 201 patients, at least one MSA was found in 63.2% of patients. The most common MSAs were the anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab) and the anti-transcriptional intermediary factor 1-gamma antibody (anti-TIF1-γ Ab) (both 13.9%), followed by anti-Jo-1 antibody (12.4%). Anti-MDA5 Ab was present exclusively in dermatomyositis (DM) and was strongly associated with digital ulcers, the clinically amyopathic phenotype and rapidly progressive interstitial lung disease (RP-ILD). Anti-TIF1γ Ab was strongly associated with refractory rash and malignancy. Multivariate analysis showed that the independent risk factors of RP-ILD included anti-MDA5 Ab (OR 14.5, p=0.001), clinically amyopathic DM (OR 13.9, p=0.015) and history of pulmonary tuberculosis (OR 12.2, p=0.026). Cox regression analysis showed that the independent predictors of malignancy included anti-TIF1γ Ab (HR 3.55, p=0.002), DM (HR 3.82, p=0.009) and family history of cancer (HR 3.40, p=0.038). Conclusions MSA testing enables dividing of patients with IIMs into phenotypically homogenous subgroups and prediction of potentially life-threatening complications.


Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 2465-2465
Author(s):  
Xiao-qin Wang ◽  
Zi-xing Chen ◽  
Shu-chang Chen ◽  
Guo-wei Lin ◽  
Mei-rong Ji ◽  
...  

Abstract Several reports indicated a striking difference in age, chromosome abnormalities and prognosis between Western and Eastern MDS patients, even in the same subtype. The prognostic factors in Chinese MDS patients remain unclear. To investigate the prognostic factors and survival rate in Chinese cases with refractory anemia (RA) in adult MDS based on FAB classification, and to evaluate the applicability of international prognostic scoring system (IPSS) for Chinese MDS-RA patients by comparing with the clinical features of Western cases, Three hundred and seven MDS-RA cases were registered and followed-up in Shanghai, Suzhou and Beijing from 1995 to 2006. The longest follow-up duration was 103 months, and the median follow-up duration was 26.7 months. Kaplan-Meier curve, Log-rank and COX regression model were used to analyze the prognostic factors and survival rate. The median age of 307 MDS-RA cases was 52 years. The frequency of 2 or 3 lineage cytopenias was 85.6%. Abnormal karyotype occurred in 35.7% of all MDS-RA patients. By IPSS cytogenetic risk groups, 165 cases (70.2%) were in the good IPSS cytogenetic subgroup, 44 cases (18.7%) in the intermediate IPSS cytogenetic subgroup and 26 cases (11.1%) in the poor IPSS cytogenetic subgroup. According to IPSS, 20 cases (8.5%) were categorized as low risk, 195 cases (83.0%) as intermediate–I risk and 20 cases (8.5%) as intermediate–II risk. The 1–year, 2–year, 3–year, 4–year and 5–year survival rate were 90.8%, 85.7%, 82.9%, 74.9% and 71.2%, respectively. Fifteen cases (4.9%) transformed to acute myeloid leukemia and the median transformation time was 15.9 months (range 3–102 months). Lower white blood cells count (&lt;1.5 × 109/L), platelet count (&lt;30 × 109/L) and cytogenetic abnormalities were identified as independent prognostic factors by multivariate analysis, while the age (≥65 years), hemoglobin level(&lt;60g/L), IPSS cytogenetic subgroup and IPSS risk subgroup were not independent prognostic factors associated with survival time by COX regression analysis. In summary, Chinese patients were younger and had lower incidence of cytogenetic abnormalities and more severe cytopenias than that of the Western patients. However, the Chinese patients have more favorable prognosis than Western patients do. WBC count, platelet count and karyotype are major prognostic factors for prediction of survival and can be helpful in identifying patients with different prognosis and consequently designing the optimal therapeutic strategies. The present IPSS scores based on conditions of western patients may not be perfectly applicable to MDS-RA patients in Asia and need to be modified.


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