Improved differentiation between Churg-Strauss syndrome and Wegener's granulomatosis by an artificial neural network

OBJECTIVE: To examine the operating characteristics of the American College of Rheumatology (ACR) classification criteria for Churg-Strauss syndrome (CSS) and Wegener's granulomatosis (WG), and to develop and validate improved criteria for distinguishing CSS from WG. METHODS: The ACR classification criteria for WG and CSS were applied to 40 consecutive CSS patients age- and sex-matched with 40 patients with WG. Forty-three clinical, laboratory, and biopsy parameters were assessed. Artificial neural networks (ANNs) were trained and tested with all 43 parameters (set A) and with 15 solely clinical parameters documented at the initial manifestation of the disease (set B). The ANNs were trained with data from the first 27 CSS and 27 WG patients and validated with data from the next 13 consecutive CSS and 13 WG patients. To compare the ANNs with established methods, traditional format and classification tree criteria were generated using the same data sets. RESULTS: Fourteen of 40 CSS patients fulfilled the ACR criteria for WG, while 4 WG patients met the ACR criteria for CSS. The ANN, in contrast, reliably distinguished all CSS cases from WG cases (parameter set A, accuracy 100%). For parameter set B, the ANN achieved an accuracy of 100% in the training phase and 96% for validation. The newly formulated traditional format and classification tree criteria reached an accuracy of 81% and 88%, respectively. CONCLUSION: The ACR criteria for WG do not reliably differentiate between CSS and WG (specificity 65%). An ANN, however, could be trained to correctly allocate all but 1 patient on the basis of clinical data. Indeed, the ANN applied in this study proved superior to established methods of classification. We suggest that an ANN may be effectively applied in the classification of systemic vasculitides.     

Follicular lymphoma in early stages: high risk of relapse and usefulness of the Follicular Lymphoma International Prognostic Index to predict the outcome of patients

BACKGROUND AND OBJECTIVES: Patients with follicular lymphoma (FL) in advanced stages are currently deemed incurable with standard treatments. However, FL is considered to be eradicable in the small group of patients presenting with localized disease. The objective of this study was to analyze the clinical features and the outcome of a series of patients with FL in early stages with a long follow-up. PATIENTS AND METHODS: A total of 48 patients (25m/23f; median age: 50 yr) diagnosed consecutively with FL in Ann Arbor stage I (25 cases) or II (23) at a single institution with a median follow-up of 9.5 yr were included in the study. Main biological and clinical characteristics at diagnosis, including Follicular Lymphoma International Prognostic Index (FLIPI) were analyzed; treatment and response were assessed and analyzed for prognosis. RESULTS: The histologic subtypes were: FL type I, 20 cases (42%); type II, 24 (50%); type III, three (6%); and unclassifiable, one (2%). Distribution according to FLIPI was: low risk (36 cases) and intermediate risk (five cases). Treatment mainly consisted of combination chemotherapy (CHOP in 34 cases) plus involved-field radiotherapy in 26 cases. Forty patients (89%) achieved a complete response (CR), three (7%) a partial response, and two (4%) were non-responders; the remaining three patients did not receive therapy. No initial variable predicted CR achievement. About 57% of the patients in CR eventually relapsed with a relapse risk of 46% at 10 yr. Intermediate-risk FLIPI predicted failure-free survival. Histologic transformation was observed in six patients with a 10-yr risk of transformation of 13%. Twelve patients died during follow-up, in two cases as a result of unrelated causes. Overall survival (OS) at 10 yr was 79%. The FLIPI was the sole variable predicting OS. CONCLUSIONS: Although the majority of patients with localized FL achieve CR, the risk of relapse is high. The FLIPI is of prognostic value in these patients.     

How to predict clinical relapse in inflammatory bowel disease patients

Inflammatory bowel diseases have a natural course characterized by alternating periods of remission and relapse. Disease flares occur in a random way and are currently unpredictable for the most part. Predictors of benign or unfavourable clinical course are required to facilitate treatment decisions and to avoid overtreatment. The present article provides a literature review of the current evidence on the main clinical, genetic, endoscopic, histologic, serologic and fecal markers to predict aggressiveness of inflammatory bowel disease and discuss their prognostic role, both in Crohn's disease and ulcerative colitis. No single marker seems to be reliable alone as a flare predictor, even in light of promising evidence regarding the role of fecal markers, in particular fecal calprotectin, which has reported good results recently. In order to improve our daily clinical practice, validated prognostic scores should be elaborated, integrating clinical and biological markers of prognosis. Finally, we propose an algorithm considering clinical history and biological markers to intercept patients with high risk of clinical relapse.     

Current prognostic and predictive factors in follicular lymphoma

Follicular lymphoma (FL) is generally considered an indolent disorder. With modern day treatments, long remissions are often achieved both in the front-line and relapsed setting. However, a subset of patients has a more aggressive course and a worse outcome. Their identification is the main purpose of modern day prognostic tools. In this review, we attempt to summarize the evidence concerning prognostic and predictive factors in FL, including (1) pre-treatment factors, from baseline clinical characteristics and imaging tests to histological grade, the microenvironment and genomic abnormalities; (2) post-treatment factors, i.e., depth of response, measured both by imaging tests and minimal residual disease; (3) factors at relapse and duration of response; and (4) prognostic factors in histological transformation. We conclude that, despite the existence of numerous tools, the availability of some of them is still limited; they generally suffer from notable downsides, and most have unproven predictive value, thus having scarce bearing on the choice of regimen at present. However, with the technological and scientific developments of the last few years, the potential for these prognostic factors is promising, particularly in combination, which will probably, in time, help guide therapeutic decisions.     

A significant diffuse component predicts for inferior survival in grade 3 follicular lymphoma,but cytologic subtypes do not predict survival

Grade 3 follicular lymphoma (FL3) is thought to have an aggressive clinical course. On the basis of possible biologic differences, the new World Health Organization (WHO) classification of lymphoma suggests further subdivision of FL3 into grades 3a and 3b and states that the percentage of involvement by diffuse large B-cell lymphoma (DLBCL) should also be reported. However, the clinical implications of these features are unclear. Therefore, we studied 190 newly diagnosed patients with lymph node-based FL3 who received anthracycline-containing combination chemotherapy. The follicular component was subclassified as grade 3a (FL3a) or grade 3b (FL3b) according to the WHO criteria, or as follicular large cleaved cell type (FLC). The percentage of a diffuse component, if present, was also recorded. Of the 190 cases, there were 107 FL3a (56%), 53 FL3b (28%), and 30 FLC (16%) cases. Diffuse areas were seen in 72 cases (31 FL3a, 28 FL3b, and 13 FLC). There were no significant differences in the clinical characteristics, overall survival, or event-free survival between patients with grades FL3a, FL3b, or FLC. However, those cases with a predominant diffuse component (> 50% diffuse) had a significantly worse overall survival (P =.0037) and event-free survival (P =.012). Therefore, we conclude that the subdivision of FL3 into cytologic subtypes does not appear to be important clinically. However, patients with FL3 having a diffuse component of more than 50% have an inferior survival that is similar to the survival of those with DLBCL.     

Role of bowel ultrasound in the management of postoperative Crohn's disease

The use of biological and immunosuppressive therapy in Crohn's disease(CD) changed favorably the course of the disease and is currently suggested in the prevention of clinical recurrence. Symptomatic exacerbation is a feature of the natural course of the disease. Endoscopic recurrence may occur earlier than clinical manifestations and its rate is still high ever since the first year after surgery. The severity of mucosal lesions is highly predictive of a new flare of the disease so that the early detection of recurrence warrants strong therapeutic changes or a closer monitoring of the case. Endoscopy is at present the gold-standard technique for the diagnosis and grading of recurrence severity, but is poorly accepted by patients for its invasiveness. A simple and easy repeatable examination able to detect early signs of recurrence could be useful in the follow-up as an alternative or as a backing in the choice of the right timing for endoscopy in questionable cases. The use of bowel ultrasound(B-US) in the management of CD has grown in the past twenty years. Its accuracy in the real time detection of the disease and its complications, known since the 80's, together with the non-invasiveness, low cost and wide availability of the technique have influenced the extension of its clinical use in many referral centers in Europe. The latest generation of ultrasound scannersallows a precise and reproducible morphological assessment of the intestinal tract and the surrounding tissues and enables a complete evaluation of the disease. This review analyzes the literature history about B-US in the diagnosis of postoperative recurrence of CD and outlines the clinical implications of its use. Published works confirm a very good accuracy of B-US in the diagnosis of CD recurrence compared to endoscopy, also in the early phase. B-US shows a good correlation with Rutgeert's score grading, but does not prove significant association with C-reactive protein or CD Activity Index values. A wider use of B-US in the daily practice could allow to set a prompt diagnosis and an earlier and targeted treatment, probably sparing more invasive tests.     

Improved differentiation between Churg‐Strauss syndrome and Wegener's granulomatosis by an artificial neural network

Objective

To examine the operating characteristics of the American College of Rheumatology (ACR) classification criteria for Churg‐Strauss syndrome (CSS) and Wegener's granulomatosis (WG), and to develop and validate improved criteria for distinguishing CSS from WG.

Methods

The ACR classification criteria for WG and CSS were applied to 40 consecutive CSS patients age‐ and sex‐matched with 40 patients with WG. Forty‐three clinical, laboratory, and biopsy parameters were assessed. Artificial neural networks (ANNs) were trained and tested with all 43 parameters (set A) and with 15 solely clinical parameters documented at the initial manifestation of the disease (set B). The ANNs were trained with data from the first 27 CSS and 27 WG patients and validated with data from the next 13 consecutive CSS and 13 WG patients. To compare the ANNs with established methods, traditional format and classification tree criteria were generated using the same data sets.

Results

Fourteen of 40 CSS patients fulfilled the ACR criteria for WG, while 4 WG patients met the ACR criteria for CSS. The ANN, in contrast, reliably distinguished all CSS cases from WG cases (parameter set A, accuracy 100%). For parameter set B, the ANN achieved an accuracy of 100% in the training phase and 96% for validation. The newly formulated traditional format and classification tree criteria reached an accuracy of 81% and 88%, respectively.

Conclusion

The ACR criteria for WG do not reliably differentiate between CSS and WG (specificity 65%). An ANN, however, could be trained to correctly allocate all but 1 patient on the basis of clinical data. Indeed, the ANN applied in this study proved superior to established methods of classification. We suggest that an ANN may be effectively applied in the classification of systemic vasculitides.

     

BCL6 gene translocation in follicular lymphoma: a harbinger of eventual transformation to diffuse aggressive lymphoma

Follicular lymphoma (FL) is characterized by a relatively indolent clinical course, but the disease often transforms into a more aggressive large cell lymphoma with a rapidly progressive clinical course. In the present study, we analyzed 41 cases of FL known to have subsequently transformed to aggressive lymphoma and an additional 64 FL samples from patients not subsequently transformed. We studied BCL6 gene rearrangement by the methodology of long-distance inverse polymerase chain reaction (LDI-PCR). Of the 41 cases known to transform, 16 (39.0%) harbored BCL6 translocation or deletion at the time of FL diagnosis. Among 64 cases not known to transform, BCL6 translocation was detected in 9 (14.1%). The prevalence of BCL6 translocation in the group known to transform was significantly higher (P =.0048). Among the transformation cases, the partners of the BCL6 translocation were identified in 13 cases and included IGH, CIITA, U50HG, MBNL, GRHPR, LRMP, EIF4A2, RhoH/TTF, and LOC92656 (similar to NAPA), whereas in the control group the BCL6 partners were IGH, CIITA, SIAT1, and MBNL. In 13 cases paired specimens before and after transformation were available. Among these paired specimens, a loss (3 cases) or a gain (1 case) of BCL6 translocation was observed after the transformation. Analysis of clonality showed that all of these cases represented the evolution of a subclone of the original tumor population. Our study demonstrated that BCL6 translocation is not necessary for transformation but that BCL6 translocation in FL may constitute a subgroup with a higher risk to transform into aggressive lymphoma.     

The impact of histologic grade on the outcome of high-dose therapy and autologous stem cell transplantation for follicular lymphoma

The impact of the follicular lymphoma (FL) histologic grade on outcomes after high-dose therapy (HDT) and autologous stem cell transplantation (ASCT) is unknown. We evaluated 219 consecutive patients with grades 1-3 FL who underwent HDT and ASCT at our center. Overall survival (OS), progression-free survival (PFS), relapse and non-relapse mortality (NRM) was estimated for each grade after controlling for other predictive factors. The number of patients with grades 1, 2 and 3 FL was 106 (48%), 75 (34%) and 38 (17%), respectively. Five-year outcome estimates for the entire cohort included 60% OS, 39% PFS and 46% relapse (median follow-up=7.8 years). PFS and relapse were nearly identical among patients with grade 3 FL versus grades 1-2 FL after adjusting for other contributing factors (hazard ratio (HR)=0.90, P=0.68; HR=1.07, P=0.80, respectively). The hazard for mortality (HR=0.70, P=0.23) and NRM (HR=0.33, P=0.07) was non-significantly lower among patients with grade 3 FL compared to patients with grades 1-2 disease. Factors associated with inferior PFS included elevated lactate dehydrogenase (HR=1.52, P=0.03), chemoresistance (HR=1.82, P=0.02), > or =2 prior therapies (HR=1.8, P=0.03) and prior radiation (HR=1.99, P=0.003). These data suggest that the histologic grade of FL does not impact PFS or relapse following HDT and ASCT.     

Spirometric criteria for hospital admission of patients with acute exacerbation of COPD

Recent studies have demonstrated that there is a high relapse rate for patients discharged from the Emergency Department (ED) following treatment of acute exacerbation of chronic obstructive pulmonary disease (COPD). Objective criteria have not been established to determine when to hospitalize these patients. This study evaluated spirometric criteria for that use. Eighty-three patients with an acute exacerbation of COPD were studied; 45 percent were admitted to the hospital while 17 percent of the patients who were discharged suffered a relapse. An FEV1 of less than 40 percent of predicted normal identified patients who required hospital admission or suffered a relapse with a sensitivity of 0.96, specificity of 0.58, and overall accuracy of 0.78. Combining clinical assessment with spirometry led to an improvement in specificity to 0.73 with a minimal decrease in sensitivity. Patients with an FEV1 of 40 percent or greater of predicted normal or no clinical evidence of respiratory distress after treatment may be safely discharged from the hospital. Patients not meeting these criteria are at high risk for relapse and should either be admitted or have further aggressive ED therapy.     

Follicular lymphoma prognostic factors in the modern era: what is clinically meaningful?

Follicular lymphomas (FL) account for 30% of non-Hodgkin’s lymphomas (NHL). Their evolution is heterogeneous. Some patients present with indolent forms undergoing several relapses while in other patients the disease evolves abruptly toward aggressive NHL. This is why accurate prognostic indices are required so that treatment strategies may be optimized for each patient and so that trials may be conducted in groups of patients that are as homogeneous as possible. The Follicular Lymphoma International Prognostic Index (FLIPI) has been designed to separate patients into 3 groups with significantly different hazard ratios for death. Its accuracy has been confirmed in several studies. The FLIPI2 was designed more recently to separate patients with significantly different hazard ratios for progression/relapse in the era of anti-CD20 monoclonal antibody treatments. Gene profile studies have shown that the prognosis of FL is mainly related to the type, number, and activation of immune cells in the microenvironment of lymphomatous follicles. Immunohistochemical studies suggest that macrophages, CD4+ T cells and among them T-regulatory cells (T-regs) and programed death-1 cells (PD-1 cells) play a major role in the outcome of FLs. However, additional confirmatory studies are required due to discrepancies in results. Up to now, these biological study results are more useful for approaching the pathophysiology of FL rather than to be used as prognostic tools in clinical practice.     

Analysis of the evolution to defined connective tissue diseases of patients with "early unidifferentiated connective tissue diseases (UCTD)"

The term undifferentiated connective tissue diseases (UCTD) is used to identify systemic autoimmune diseases not fulfilling classificative criteria for defined connective tissue diseases (CTD). Aim of the present study was to evaluate the evolution to defined CTD of an historical cohort of 91 UCTD patients followed at our Unit and to describe clinical and serological characteristics of stable UCTD patients with a disease duration of more than 5 years. Patients, previously described, were selected for having an undifferentiated profile after 1 year of follow up. These patients have been regularly followed at our Unit and their diagnosis has been reassessed annually based on the existing classificative criteria. Seven UCTD patients with a follow up of less than 5 years have been excluded from the study, therefore 84 patients (F: 81, M: 3) have been analysed. During the follow up 28 patients (33%) developed a defined CTD. In particular 22 patients developed systemic lupus erythematosus (SLE), while the remaining 6 patients developed other CTDs (2 primary Sj?gren's syndrome, 2 overlap syndromes, 1 Systemic Sclerosis, 1 rheumatoid arthritis). The evolution to a defined CTD occurred after a mean disease duration of 80.6+/- 66.8 months (min 14, max 336, median 72); the evolution to SLE occurred after a mean disease duration of 66.8+/-43.3 months (min 17, max 216, median 57). Anti-cardiolipin antibodies were the only variable correlated with the evolution to SLE (p<0.05). Stable UCTD were characterized by a simplified clinical picture with no major organ involvement and by a simplified autoantibody profile (anti-Ro/SSA antibodies and anti-RNP antibodies were the single antibody specificities observed in 22% and 13% of patients respectively). These results confirm previous data showing that about 30% of UCTD patients will develop a defined CTD, the predictive role of anti-cardiolipin antibodies for the evolution to SLE, and the existence of stable UCTD, distinct clinical entities with a simplified clinico-serological profile. The early identification of stable UCTD is very important both from a clinical and a research point of view. Future research is needed to define a new set of classification criteria.     

The significance of DNA distribution pattern in rectal carcinoma

The DNA distribution pattern was determined retrospectively in 25 rectal carcinomas and the possible correlation to clinical outcome evaluated. The DNA content in individual cells was measured according to a cytophotometric method based on light transmission measurement of Feulgen-stained nuclei. Tumor cells with DNA content exceeding an upper limit,i.e., the 90 percentile of the control cells, were considered to be nondiploid (aneuploid). Virtually all long-term survivors had less than 50 per cent of the tumor cells exceeding the upper diploid level, whereas those developing only a local recurrence had 50 to 70 per cent. Patients with disseminated disease and short survival time had all of their tumor cells exceeding the upper diploid level. There was a highly significant correlation between Dukes' stage and aneuploidy and probably a significant correlation between histologic grading and aneuploidy. The clinical significance of these results lies in the fact that DNA can be measured in biopsy specimens. It might thus be possible to “tailor” the operation according to the future clinical course to be expected. It could be hypothetically argued that patients with a DNA profile heralding disseminated disease and short life expectancy should have surgery that preserves quality of life, where-as those tending to develop a local recurrence should have more aggressive surgery. It may also be possible to define groups of patients thought to prosper from a more intense postoperative surveillance. The scientific basis for these suggestions is still lacking and further studies on a prospective basis are currently in progress.     

A clinicopathologic correlation of the idiopathic hypereosinophilic syndrome. II. Clinical manifestations

The idiopathic hypereosinophilic syndrome, a disorder characterized by peripheral blood and bone marrow eosinophilia associated with single or multiple organ system dysfunction attributable to tissue invasion by eosinophils has, in the past, been associated with an extremely poor prognosis. Recently, we reported the favorable impact of a therapeutic protocol consisting of prednisone and/or hydroxyurea on the morbidity and mortality of this syndrome. We have reviewed the clinical and hematologic features upon admission and the subsequent clinical courses of 32 patients with this disease referred to the NIH between 1965 and 1979 in an effort to determine which features suggest a more rapidly progressive course. A grading system based on 22 clinical features involving the 8 organ systems commonly affected by the illness was devised. The disease followed a more aggressive course in patients with evidence of cardiac or neurologic dysfunction at the time of initial NIH evaluation. Although splenomegaly, in and of itself, caused little morbidity, splenic enlargement at presentation appeared to be a predictor of a more aggressive course. The clinical grading system accurately predicted which patients would require no specific antihypereosinophilic therapy, which patients would respond adequately to corticosteroids, and which patients would require therapy with cytotoxic agents. It is proposed that this clinical grading system, and the hematologic grading system outlined in the accompanying report be used as aids in the selection of initial therapy in this group of patients.     

Comparison of operational criteria for treatment outcome in gambiense human African trypanosomiasis

Objective  To develop a simple and standard operational decision tool for the diagnosis of relapse after treatment for human African trypanosomiasis (HAT), by evaluating the performance of several criteria currently used by HAT control programs and research projects.
Methods  We identified 10 different criteria for relapse, based on trypanosome presence and/or white blood cell count in cerebrospinal fluid, and compared their specificity, sensitivity and time to diagnosis on a data set containing 63 relapsed and 247 cured T.b. gambiense patients.
Results  At any time point, the criterion 'Trypanosomes present and/or a cerebrospinal white blood cell count ≥50/μl' allowed accurate and timely detection of HAT relapse, irrespective of disease stage. This criterion was 13–25% more sensitive ( P  ≤   0.013) than trypanosome detection alone and was >97% specific. Lumbar punctures at the end of treatment and at 3-month post-treatment provided limited clinical information.
Conclusions  Adequate detection of relapse was possible with a simple criterion but these findings should be validated in a prospective study before adoption in clinical practice.     

Nodular sclerosing Hodgkin disease: new grading predicts prognosis in intermediate and advanced stages

The prognostic value of histologic classification and single histomorphologic parameters in Hodgkin disease has been widely debated in the literature. Whereas several former studies identified single parameters to be of clinical relevance, some recent reports have doubted the prognostic value of histology using modern treatment. Grading of the largest histologic category of Hodgkin disease, nodular sclerosis (NS), has been controversially discussed concerning clinical relevance. In this study, 965 cases of NS were reviewed to assess 9 histomorphologic parameters. The histologic results were correlated with laboratory and clinical findings and with overall survival and disease-free survival. Based on these results, a new grading of the NS category was established. The new grading, based on the 3 criteria eosinophilia, lymphocyte depletion, and atypia of the Hodgkin/Reed-Sternberg cells, was a significant indicator of prognosis in intermediate and advanced stages. Patients investigated in this study represent an outstanding collection because all of them were enrolled in the prospective multicenter clinical trial of the German Hodgkin Lymphoma Study Group. All of them had been staged uniformly according to the Ann Arbor system and had received stage-adapted modern treatment according to multimodality protocols. A subtle analysis of histology could represent a possible way to identify patients with a significantly better or worse prognosis. This new grading should help to avoid overtreatment to reduce severe therapy-related side effects such as acute toxicity and chronic sequelae such as cardiopulmonary complications and secondary neoplasias.     

Interferon alpha with or without rituximab achieves a high response rate and durable responses in relapsed FL: 17 years’ experience in a single centre

Maintenance interferon alpha (IFN-α) immunotherapy after induction chemotherapy prolongs progression-free survival (PFS) in untreated follicular lymphoma (FL). Little information is available about IFN-α use in relapsed FL. This study aims to evaluate the benefit of IFN-α as a treatment of low-burden FL relapse. This single-centre retrospective study identified 20 patients treated in 27 cases with IFN-α. We analysed all cases of IFN-α treatment in patients with low-burden FL in clinical relapse (11), partial response (5) or only with molecular minimal residual disease (MRD; 5). The treatment schedule was 3MIU IFN-α three times a week alone (16) or combined with four weekly rituximab (R; 11), according to the institution’s policy. Except for the molecular relapses, responses were evaluated according to the IWG 1999 criteria. MRD was defined as a repeatedly detectable BCL2-IgH rearrangement in peripheral blood or bone marrow. In 22 cases of clinical relapses or partial responders, overall response rate was 68 %, with 55 % complete responses. Median PFS was 20.9 months (95 % confidence interval (95 % CI), 0–64.9) with 20.9 and 48.7 months in the IFN and R-IFN groups, respectively (p?=?0.4). The median PFS of the five MRD cases was 133 months (95 % CI, 103–165). The Follicular Lymphoma International Prognostic Index score calculated at initiation of IFN-α treatment was predictive of time to relapse (p?=?0.036). These results compare favourably with previous reports of the efficacy of R alone, and of R with IFN-α in relapse. Further research is required to explore the role of IFN-α in the management of FL.     

Clinical characteristics and outcomes of Takayasu's arteritis: analysis of 108 patients using standardized criteria for diagnosis, activity assessment, and angiographic classification

OBJECTIVES: To investigate the clinical characteristics and outcomes of Takayasu's arteritis (TA) using standardized criteria for diagnosis, disease activity, and angiographic classification, and to identify the predictive factors for remission, angiographic progression, and mortality in patients with TA. METHODS: One hundred and eight patients who fulfilled the 1990 American College of Rheumatology (ACR) classification criteria for TA were studied. Their clinical features, laboratory findings, angiographic findings, and clinical outcomes were evaluated retrospectively. The disease activities were assessed using the National Institutes of Health (NIH) criteria for active disease, and the angiographic types were classified using the International TA Conference in Tokyo 1994 angiographic classification. RESULTS: Angiographic classification showed that type I was the most common, followed by types V and IV. Ninety-one patients had active disease at diagnosis, and remission was achieved in 81.3% of them. Among those who experienced remission and those who had stable disease at diagnosis, 28.6% experienced a relapse. A low erythrocyte sedimentation rate (ESR) at diagnosis and treatment with glucocorticoid were found to be independent predictors for remission, and the stable disease activity at diagnosis was an independent predictor for the quiescence of vascular lesions on follow-up angiography. Survival rates were 92.9% at the fifth year and 87.2% at the tenth year, and the presence of two or more complications was a risk factor for mortality. CONCLUSIONS: These findings could provide useful information on the clinical features, angiographic findings, and outcomes in TA, particularly on the assessment of patients at risk of a poor outcome.     

Liver histology according to the presence of metabolic syndrome in nonalcoholic fatty liver disease cases

AIM： To investigate the histologic features of the liver in nonalcoholic fatty liver disease （NAFLD） cases according to the presence of metabolic syndrome or its individual components. METHODS： We enrolled 81 patients （40 male, 41 female） who were diagnosed with fatty liver by ultrasonographic scan and fulfilled the inclusion criteria. First anamnesis, anthropometric, clinical, laboratory and imaging features of all participants were recorded and then liver biopsy was performed after gaining consent from patients. Diagnosis of metabolic syndrome was dependent on patients having 3 or more out of 5 risk criteria defined by the WHO. Biopsy specimens were assessed according to Brunt etal＇s classification. RESULTS： Sixty-nine of the 81 patients had nonalcoholic steatohepatitis （NASH）, 11 had simple fatty liver and 1 had cirrhosis according to histologic evaluation. Comparisons were made between two groups of NASH patients, those with and without metabolic syndrome. We did not detect statistically significant differences in liver histology between NASH patients with and without metabolic syndrome. CONCLUSION： NASH can progress without metabolic risk factors or the presence of metabolic syndrome.