Validation of the International Criteria for Behçet’s disease (ICBD) in Iran

Aim: To evaluate the performance of the new International Criteria for Behçet’s Disease (ICBD) in Iran. The ICBD was created in 2006 to replace the International Study Group (ISG) criteria (created in 1990). In this study, 14 existing diagnosis/classification criteria (Curth, Hewitt, Mason and Barnes, Japan original, Hubault and Hamza, O’Duffy, Cheng and Zhang, Dilsen, Japan revised, International Study Group, Iran traditional, Iran Classification Tree, revised Dilsen, Korea), were evaluated and compared to ICBD by calculating their sensitivity, specificity and accuracy. Methods: All patients from the Behçet’s Disease registry (6128) and controls (3400), up to January 2008, entered the study. The diagnosis was clinical and not by any of the above‐mentioned criteria. Sensitivity, specificity, accuracy (percent agreement), and 95% confidence interval were calculated. The performance of ICBD and ISG were compared by the McNemar test. Results: The sensitivity was 98.2% for ICBD and 78.1% for ISG (P < 0.0001). The specificity was 95.6% for ICBD and 98.4% for ISG (P < 0.0001). The accuracy was 97.3% for ICBD and 85.5% for ISG (P < 0.0001). Discussion: In Iranian patients, ICBD has 20.1% higher sensitivity and 11.8% higher accuracy than ISG, while the specificity was only 2.8% lower. ICBD was better optimized than ISG (difference between sensitivity and specificity 2.6% vs 20.3%). Conclusion: ICBD has by far better performance than ISG in Iran. The difference is even more prominent in Iranian patients than in the cohort of the international patients upon whom the criteria were created.     

The saga of diagnostic/classification criteria in Behcet's disease

There are 17 sets of diagnosis/classification criteria for Behcet's disease: Curth (1946), Hewitt (1969), Mason (1971), Japan (1972), Hubault (1974), O'Duffy (1974), Cheng (1980), Dilsen (1986), Japan revised criteria (1988), International Study Group on Behcet's disease (ISG criteria, 1990), Iran traditional criteria (1993), Iran Classification Tree (1993), Dilsen revised criteria (2000), Korea Criteria (2003), International Criteria for Behcet's Disease (ICBD, 2006) and the revised ICBD (2010). This review is intended to show how to use them and show their performance in patients from different parts of the world. The major sets of patients (patient numbers, control numbers, year) on which the criteria were tested are: ISG set (886/97/1990), Iran (2069/1540/1993), Asia and Pacific League of Associations for Rheumatology (APLAR: 216/145/1998), Russia (105/233/2000), USA (50/NA/2000 [NA: not available]), India (50/NA/2004), Singapore (37/NA/2004), China (98/NA/2004), Korea (1454/NA/2004), Iran (4900/2020/2004), ICBD (2556/1163/2006), Germany (86/38/2008), China (322/118/2008), Iran (6128/3400/2010) and Iran (7011/5226/2013). For the following criteria sets (O'Duffy, Dilsen, Japan revised, ISG, Korea, ICBD, revised ICBD), the sensitivity in ISG cohort was 82/95/93/91/NA/NA/NA%, in APLAR 62.5/75/73/72/NA/NA/NA%, in Russia 91/92/92/86/NA/NA/NA%, USA 88/85/82/76/NA/NA/NA%, ICBD 83/87/88/82/90/96/96%, China 64/71/66/65/85/87/NA% and in Iran (2013) 69.5/81/86/77.5/86/98/97%. Specificity in ISG was 83/79/89/96/NA/NA/NA%, in APLAR 98/96/99/99/NA/NA/NA%, in Russia 88/91/92/100/NA/NA/NA%, ICBD 95/91/92/96/93/89/91%, China 97.5/95/98/99/97/94/NA% and in Iran (2013) 99/95/98/99/98/96/97%. Accuracy in ISG was 82.5/87/91/93.5/NA/NA/NA%, in APLAR 80/85/86/86/NA/NA/NA%, in Russia 89.5/92/92/93/NA/NA/NA%, ICBD 87/88/89/87/91/94/94.5%, China 72/78/74/74/88/89/NA% and in Iran (2013) 82/87/91/87/91/97/97%. ISG criteria has very good specificity, but lacks good sensitivity and accuracy. In contrast, ICBD has much better sensitivity, a little less specificity and better accuracy.     

Pathergy test in Behcet's disease: change in incidence over the time

Introduction: Pathergy phenomenon (PP) is used as a criterion in many diagnostic criteria for Behcet's disease (BD): Curth, Japan, Hubault and Hamza, Cheng and Zhang, Dilsen, International Study Group, Iran criteria (traditional format and the Classification Tree), Korea criteria, and the new International Criteria. PP is rather specific to BD. It has been reported with high frequency from most countries along the Silk Road, but with much lower frequency from other countries (UK, US). The incidence of PP has been reported from some countries (Japan, Turkey) to decrease gradually. The aim of this study was to test the above hypothesis in a large cohort of patients in Iran. Materials: Patients (6057) were divided into several groups by two methods. Method 1: patients were divided into six groups according to the time of their first visit. Method 2: patients were divided into four groups according to the date of the beginning of their first manifestation. Results: In method 1, the percentage of PP was 71.8% from patients 1–1000. The percentage became 55.9% from patients 1001–2000, 57.6% from patients 2001–3000, 58.8% from patients 3001–4000, 45.3% from patients 4001–5000, and 33.9% from patients 5001–6000. In method 2, the percentage of PP was 61.5% for patients with their disease onset before 1977. The percentage was 59.9% for patients with their disease starting between 1978 and 1987. The percentage dropped to 52.1% for patients with disease onset between 1988 and 1997. The percentage finally dropped to 41% for patients having their disease onset between 1998 and 2007. Conclusion: The incidence of PP is decreasing gradually. It is now 34%, which is rather low for a criterion used in many diagnosis criteria. It is important to find a surrogate for it because with the decrease in the incidence of PP the sensitivity of the diagnosis criteria will drop significantly.     

Impact of the positive pathergy test on the performance of classification/diagnosis criteria for Behcet’s disease

Background

The only diagnostic test that currently exists for Behcet’s disease (BD) is the pathergy test. A positive pathergy test (PPT) is an important component of many of the 16 sets of classification/diagnosis criteria used to diagnose BD. The aim of this study was to determine the importance of a PTT in the performance of the diagnosis/classification criteria for BD.

Patients and methods

All patients listed in the BD registry of the Rheumatology Research Center, Tehran (6,727) and 4,648 BD controls were enrolled in the study. The diagnosis was clinical when no other diagnosis could explain the patient’s manifestations. The criteria were tested with and without PPT results. Sensitivity, specificity, and accuracy were calculated.

Results

Without PPT, all sets of criteria lost sensitivity, gained specificity, and lost accuracy, with the exception of the Cheng–Zhang criteria. The largest loss in sensitivity was for the Hubault–Hamza (35 %) and Dilsen (17.3 %) criteria; the least was for the Curth (1.9 %) and ICBD (6.5 %) criteria. The largest gain on specificity was for the Dilsen (4.7 %) and Curth (3.1 %) criteria; the least was for the Japan (0.1 %) and Japan revised (0.1 %) criteria. The greatest loss in accuracy was for the Hubault–Hamza (20.4 %) and Dilsen revised (9.3 %) criteria; the least was for the ICBD (3.6 %), while Curth gained 0.3 %.

Conclusion

Without PPT as a criterion for the diagnosis of BD, the sensitivity and accuracy of the sets of classification/diagnosis criteria decrease, while the specificity improves.     

New simple way to use the classification tree for the diagnosis of Behcet's disease

Background: Behcet's Disease (BD) is a vasculitis of unknown origin. It is mainly seen around the Silk Road. The classification tree is the most sensitive and accurate criteria for the classification and diagnosis of the disease. The use of a classification tree necessitates having the diagram in hand. An arithmetic method was developed to replace the diagram by easy‐to‐remember figures. Methods: Each criterion was given an appropriate value: ocular lesions were given 5 points, oral aphthosis 4 points, genital aphthosis 3 points, pathergy phenomenon 2 points, and skin manifestations 1 point. Seven points or higher values were necessary to classify a patient as having Behcet's disease. The arithmetic method was tested in 5276 BD patients and in 2407 control patients. Results: The sensitivity of the classification tree was 97.2%, the specificity was 96.7%, and the accuracy was 97.1%. The arithmetic method gave exactly the same results. Discussion: The arithmetic extrapolation of the classification tree is a new and simple way for the diagnosis and the classification of Behcet's disease.     

Evaluation of the international study group for Behçet's disease criteria in Brazilian patients

Summary Twenty-eight patients with Behçet's Disease (BD), diagnosed according to at least one of the published and internationally recognized diagnostic criteria (Japanese Committee, International Committee, O'Duffy, Mason & Barnes and James) were compared with 56 controls with other rheumatic diseases paired by sex and age.This study aimed at comparing the sensitivity and specificity of the five diagnostic criteria chosen as well as the validation of the use of the new set of diagnostic criteria proposed by the International Committee in our population.We conclude that, in general, Brazilian patients with BD present demographic and clinical characteristics similar to those described in the world literature. The criteria proposed by the International Committee, although not the most accurate in our study, are a valid proposal for the universal unification of diagnostic criteria for BD.     

Revised Japanese criteria for Sjögren's syndrome (1999): availability and validity

The Japanese criteria for diagnosing Sjögren's syndrome (SS) were revised in 1999, and consist of four major areas: histopathology, oral examination, ocular examination, and serological examination. A diagnosis of SS can be made when the patient meets at least two of these four criteria. This report describes how the revised Japanese criteria were established. After the publication of the revised Japanese criteria (1999), a research study which focused on evaluating its availability and validity was carried out in 2001 using funds from Grant-in-Aids for Scientific Research supported by the Japan Society for the Promotion of Science. The availability of the revised criteria was investigated by a questionnaire study through the Japanese Medical Society for Sjögren's Syndrome, and the use of the revised criteria for diagnosing SS in these medical facilities was found to be 76%. To evaluate the validity of the revised criteria, the records of 900 patients, including SS patients and non-SS controls, from 54 clinical centers were registered and analyzed to calculate the accuracy of the criteria. The revised Japanese criteria were found to have 96.0% sensitivity, 90.5% specificity, and 94.5% accuracy for diagnosing SS.     

Revised Japanese criteria for Sjögren’s syndrome (1999): availability and validity

Abstract

The Japanese criteria for diagnosing Sjögren's syndrome (SS) were revised in 1999, and consist of four major areas: histopathology, oral examination, ocular examination, and serological examination. A diagnosis of SS can be made when the patient meets at least two of these four criteria. This report describes how the revised Japanese criteria were established. After the publication of the revised Japanese criteria (1999), a research study which focused on evaluating its availability and validity was carried out in 2001 using funds from Grant-in-Aids for Scientific Research supported by the Japan Society for the Promotion of Science. The availability of the revised criteria was investigated by a questionnaire study through the Japanese Medical Society for Sjögren's Syndrome, and the use of the revised criteria for diagnosing SS in these medical facilities was found to be 76%. To evaluate the validity of the revised criteria, the records of 900 patients, including SS patients and non-SS controls, from 54 clinical centers were registered and analyzed to calculate the accuracy of the criteria. The revised Japanese criteria were found to have 96.0% sensitivity, 90.5% specificity, and 94.5% accuracy for diagnosing SS.     

A comparison of the performance characteristics of classification criteria for the diagnosis of psoriatic arthritis

OBJECTIVE: To compare the accuracy of published classification criteria for the diagnosis of psoriatic arthritis (PsA) and to see whether data-derived classification criteria would be more accurate. METHODS: Data were abstracted from case-note review and radiographic review of patients identified with PsA or rheumatoid arthritis (RA) from 2 clinical disease registers. Each patient was classified according to 7 criteria sets. The test performance characteristics were compared using conditional logistic regression analysis. In an attempt to overcome the problems of the diagnostic gold standard, latent class analysis also was used to calculate test-performance characteristics. Classification and regression-tree methodology was used to derive new criteria and to indicate the diagnostic importance of particular data items, especially rheumatoid factor (RF). RESULTS: Four hundred ninety-nine patients were identified with RA (n=156) or PsA (n=343). Excluding the criteria of Fournie, which could not be applied in 24% of subjects, 446 cases could be classified by all of the other 6 methods. The most sensitive criteria for the diagnosis of PsA were those of Vasey and Espinoza, McGonagle, and Gladman (99%), whereas the others were significantly less sensitive (between 56% and 94%). The specificity of the criteria was high and statistically similar (between 93% and 99%). The Fournie criteria were the most difficult to use, whereas the Vasey and Espinoza and Moll and Wright criteria were the easiest (98% of subjects were able to be classified). A 2-latent class model found very similar test-performance characteristics. Logistic regression and classification and regression-tree models suggested that negative RF was not necessary for diagnosis in the presence of other characteristic features of PsA. CONCLUSIONS: Apart from the Bennett and European Spondyloarthropathy Study Group criteria, which have inadequate sensitivity, the published classification criteria for PsA have similar test-performance characteristics. These data suggest that the criteria proposed by Vasey and Espinoza, Gladman, or McGonagle are the most accurate and feasible in distinguishing between PsA and RA. Relevance International agreement about classification criteria for PsA will assist the interpretation of clinical and epidemiologic research. However, further prospective studies on unselected patients with and without PsA, including controls with non-rheumatoid inflammatory arthritis, are required to confirm these findings.     

Comparative analysis of Behcet's disease in the APLAR region

Aims: Behcet's disease (BD) was originally a disease of the Silk Road. Some authors think that BD from the Silk Road is different from those seen in other countries. The aim of this study was to analyze the clinical manifestations of BD in APLAR countries, where some of them are in the Silk Road (SR) and some others not (NSR). Methods: Data from Australia (NSR), Hong Kong (NSR), India (SR), Iran (SR), and Singapore (SR) were selected and analyzed under the same protocol. Prior published data from China (SR) Japan (SR) and Korea (SR) were included in the analysis. Results: The mean age at the onset of the disease was under 30 for all countries except Japan and Singapore. The male gender was more frequent except in Australia and Korea. Oral aphthosis was the most frequent manifestation (90–100%). Genital aphthosis was less frequent (57–82%). Skin manifestations were also frequent (61–87%). Ocular manifestations were reported from 21–69% of patients. The difference was mainly due to patients’ selection bias and the low number of patients in some reports. The same was true for joint (30–87%), gastrointestinal (6–38%), neurological (2.5–29%) and vascular manifestations (5–28%). Conclusion: Despite the percentage difference among some countries, the general pattern of the disease was the same, suggesting that the minor differences seen in different parts of the world were not enough to call the disease a syndrome, or to differentiate Behcet's disease of the Silk Road from those seen in Western countries.     

Prevalence of Behcet's disease in Iran: a WHO‐ILAR COPCORD stage I study

Aim: To elucidate the prevalence of Behcet's disease (BD) during the World Health Organization‐International League of Associations for Rheumatology Community Oriented Program for Control of Rheumatic Disease study in Iran. The old estimate was 16 per 100,000 inhabitants, but this was just an estimation and a more precise figure was required. Materials and methods: Tehran, the capital of Iran with 1/10th of Iran's population, was selected as the field for the COPCORD study. The population of Iran is of mixed ethnicity (Caucasians 75.4%, Turks 22%, and Semites 2.6%). The same distribution was found in Tehran's general population. Tehran has 22 districts; 50 clusters were randomly selected from them. Interviewers passed an exam by interviewing 20 subjects. The observed agreement was 0.96. The chance expected agreement was 0.531325. The kappa coefficient was 0.919 (standard error: 0.112). The z‐score was 8.19. The one tailed P‐value was < 0.0001. Rheumatologists were selected from the ‘Rheumatology Subspecialty’, that is, those who were scheduled to become a rheumatology professor in one of Iran's medical schools. Results: The response rate was 75%. From 10,291 subjects interviewed all subjects with positive questionnaires were examined; seven had definitive BD and five probable BD. The prevalence was calculated on the seven definitive cases. It was 68 for 100,000 inhabitants. The confidence interval was 33.5–137. Four patients were female and three were male. All had oral and genital aphthosis, four had ocular lesions, three had pseudo‐folliculitis, and one had joint manifestations. Conclusions: The prevalence of BD in Iran is 68 per 100,000 inhabitants, which is the second highest prevalence after Turkey (80–370) in the world, and far behind comes Saudi Arabia with 20, China 14, and Japan 13.4 per 100,000 inhabitants.     

Early diagnosis of granulomatosis with polyangiitis: An introduction to the newly designed Iran criteria

BackgroundsIn the absence of practical diagnostic criteria for diagnosis of granulomatosis with polyangiitis (GPA), a new diagnostic criteria for GPA is proposed based on literature review for characteristic manifestations of GPA and expert opinion. The sensitivity of the new criteria, Iran criteria for GPA, is assessed in comparison with 1990 American College of Rheumatology (ACR) criteria for Wegener's granulomatosis (WG).MethodsEvaluation of three organs (ear, nose and throat (ENT); lung; kidney) and two laboratory findings (anti-neutrophil cytoplasmic antibody; biopsy), abbreviated mnemonically as ELKAB, is suggested in our criteria. A retrospective sensitivity analysis was performed based on medical records of 35 patients. Clinical diagnosis of GPA by a single rheumatologist was used as the gold standard.ResultsRecords of a total of 15 male and 20 female patients with a mean follow-up duration of 21.26 ± 4.13 months were considered. Mean age at diagnosis and mean disease duration were 32.37 ± 2.33 years and 19.06 ± 5.41 months, respectively. The sensitivity for Iran criteria for GPA and 1990 ACR classification criteria for WG were calculated as 100% and 80%, respectively.ConclusionsIran criteria for GPA is a highly sensitive instrument for detecting GPA patients in comparison with 1990 ACR classification criteria for WG.     

Duffy blood group genotypes among malaria Plasmodium vivax patients of Baoulch population in southeastern Iran

Objective:To determine the distribution of Duffy blood group genotypes in Balouch population as a major ethnic group that living in a sub-tropical area in south East of Iran.Methods:In this study,the Duffy blood group FY phenolypes were determined using indireet anti-globulin technique and also genotype by PCR-RFLP in 160 vivax malaria patients and 160 control individuals.Results:The results showed that the most common Duffy geitolype was FYA/FYB(46.6%)followed by FYA/FYA(15.3%),FYA/FYO(14.4%),FYB/FYO(11.9%,),FYB/FYB(10%)and FYO/FYO(1.9%).In case individuals,frequency of FY A.FYB and FYO alleles were 0.471,0.431and 0.097,respectively compaired to 0.444,0.353 and 0.203,respectively in control(non-infected)group.Conclusions:This data provide evidence that individuals with the FYA/FYB genotype have higher susceptibility to malaria and there are significant associations between Duffy blood group variants and susceptibility or resistance to vivax malaria.     

Evolving concepts in classification of systemic vasculitis: where are we and what is the way forward?

The classification of the systemic vasculitides has been controversial for several decades. The Chapel Hill consensus Conference definitions originally developed in 1994, but revised and extended in 2012 are now widely accepted. The American College of Rheumatology (ACR) criteria were first published in 1990, are now generally accepted to be out of date and new criteria are needed. More recently the classical division of the ANCA vasculitides using clinical phenotype has come under scrutiny with evidence from epidemiological, genetic and outcome studies that perhaps these conditions should be classified on the basis of ANCA specificity into PR3‐ANCA positive and MPO‐ANCA positive groups. The traditional distinction between giant cell arteritis and Takayasu arteritis has been questioned and some recent studies of GCA have included patients with only extra‐cranial disease. The Diagnostic and Classification Criteria of Vasculitis study (DCVAS) will provide new validated classification criteria for the systemic vasculitides.     

Current diagnosis and treatment of polymyositis and dermatomyositis

Abstract

Idiopathic inflammatory myopathies (IIMs) are heterogeneous disorders that affect the skeletal muscles. Polymyositis, dermatomyositis, and inclusion body myositis are major IIM subsets. Immune-mediated necrotizing myopathy became recognized as a potentially new IIM subset. Since the new classification criteria published by the International Myositis Classification Criteria Project have higher sensitivity and specificity for IIM classification and subclassification than the previous criteria, they should help precise diagnosis. It should be noted that several tests available in current clinical practice, such as electromyography, magnetic resonance imaging, and other myositis-specific autoantibodies than anti-Jo-1 antibodies, were not included in the new criteria. As for treatment, glucocorticoids are used empirically as the first-line treatment despite their various adverse effects. Concomitant treatment with steroid-sparing immunosuppressive agents, including methotrexate, azathioprine, calcineurin inhibitors, mycophenolate mofetil, and cyclophosphamide, reduces successfully initial glucocorticoid doses for the remission induction, the relapse risk during glucocorticoid tapering, and adverse effects of glucocorticoids. Treatment with biologics, including rituximab and abatacept, seems promising in some IIM patients. Multi-target treatment with glucocorticoids and several steroid-sparing immunosuppressive agents is effective in refractory IIM patients. Considering proven steroid-sparing efficacy and tolerability of multi-target treatment in patients with other autoimmune diseases, it should be a good therapeutic option for IIMs.     

Classification of Japanese patients with mild/early systemic sclerosis (SSc) by the 2013 ACR/EULAR classification criteria for SSc

Objective: To classify Japanese patients with mild/early systemic sclerosis (SSc) by the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) Classification Criteria (new 2013 criteria).

Methods: We assessed 120 patients who visited Kanazawa University Hospital suspected of SSc and who did not meet the 1980 ACR preliminary classification criteria for SSc. We clinically diagnosed 16 patients with primary Raynaud’s disease and 104 with mild/early SSc prior to being assessed by the new 2013 criteria.

Results: None of the 16 patients with primary Raynaud’s disease met the new 2013 criteria. On the other hand, 94 out of the 104 patients (90.3%) with mild/early SSc by our clinical diagnosis met the new 2013 criteria. Among the 94 SSc patients, sclerodactyly was detected in 58 (62%), puffy fingers in 62 (66%), abnormal nailfold capillaries in 89 (95%), Raynaud’s phenomenon in 93 (99%), and SSc-related autoantibodies (Abs) in 85 (90%). The median (range) score of these 94 patients was 12 (9–14). Ten mild/early SSc patients who did not meet the new 2013 criteria had the following clinical features: puffy fingers in 1 (10%), abnormal nailfold capillaries in 8 (80%), Raynaud’s phenomenon in 9 (90%), and SSc-related autoAbs in 8 (80%). The median (range) score of these 10 patients was 7 (5–8).

Conclusion: The new 2013 criteria can classify most mild/early Japanese SSc patients, which may contribute to early treatment interventions.     

Behcet’s disease: from east to west

Behcet's disease (BD) is classified among vasculitides. There are actually five nationwide surveys of BD: Iran, Japan, China, Korea, and Germany. Among case series, four are on more than 200 cases (Turkey, Morocco, Tunisia, and UK). BD was classically seen around the Silk Route. Now, it is seen everywhere. The male to female ratios were, respectively (in nationwide surveys), 1.19, 0.98, 1.34, 0.63, and 1.40 to 1. The mean age at onset was 26.2, 35.7, 33.8, 29, and 26 years. Major manifestations were seen, respectively, in nationwide surveys: mucous membrane (oral aphthosis in 97%, 98%, 98%, 99%, and 98%; genital aphthosis in 65%, 73%, 76%, 83%, and 64%); skin manifestations (pseudo-folliculitis in 57%, N/A, 31%, N/A, and 62%; erythema nodosum in 22%, N/A, 38%, N/A, and 42%; ocular manifestations in 55%, 69%, 35%, 51%, and 53%). Minor manifestations were seen, respectively, in nationwide surveys: joint manifestations in 33%, 57%, 30%, 38%, and 53%; neurological manifestations in 9%, 11%, 6.5%, 4.6%, and 11%; gastrointestinal manifestations in 7%, 15.5%, 9%, 7.3%, and 12%; vascular involvement in 8.9%, 8.9%, 7.7%, 1.8%, and 13%; pulmonary manifestations in 0.3%, N/A, 2.2%, N/A, and 3.6%; cardiac manifestations in 0.5%, N/A, 4%, N/A, and 3.2%. Laboratory tests are not useful except the pathergy test, which was positive in 54%, 44%, N/A, 40%, and 34% of cases. ESR was normal in many patients. Diagnosis is based upon clinical manifestations. The International Criteria for Behcet's Disease (ICBD, 2006) may be of help, having a sensitivity of 98.2% and a specificity of 95.6% in Iranian patients.     

Evaluation of the alternative classification criteria of systemic lupus erythematosus established by Systemic Lupus International Collaborating Clinics (SLICC)

Objective: To evaluate the performance of the 2012 Systemic Lupus International Collaborating Clinics criteria (SLICC-12) on classifying systemic lupus erythematosus (SLE) in an uncontrolled multi-centered study with real-life scenario of the patients in Japan.

Methods: This study comprised 495 patients with SLE or non-SLE rheumatic diseases and allied conditions from 12 institutes in Japan. Chart review of each patient was performed by the 27 expert rheumatologists and diagnosis of 487 cases reached to the consensus. Value of the SLICC-12 on SLE classification was analyzed comparing with the 1997 revised American College of Rheumatology SLE classification criteria (ACR-97) employing the expert-consented diagnoses.

Results: Compared to the ACR-97, the SLICC-12 had a higher sensitivity (ACR-97 vs. SLICC-12: 0.88 vs. 0.99, p?Conclusion: Although employment of SLICC-12 for the classification for SLE should be carefully considered, the SLICC-12 showed the higher sensitivity on classifying SLE in Japanese population.     

A comparison of the International Consensus and 5th WHO classifications of T-cell lymphomas and histiocytic/dendritic cell tumours

Since the publication in 2017 of the revised 4th Edition of the World Health Organization (WHO) classification of haematolymphoid tumours, here referred to as WHO-HAEM4, significant clinicopathological, immunophenotypic and molecular advances have been made in the field of lymphomas, contributing to refining the diagnostic criteria of several diseases, upgrading entities previously defined as provisional and identifying new entities. This process has resulted in two recent classification proposals of lymphoid neoplasms: the International Consensus Classification (ICC) and the 5th edition of the WHO classification (WHO-HAEM5). In this paper, we review and compare the two classifications in terms of diagnostic criteria and entity definition, focusing on T-cell lymphomas and histiocytic/dendritic cell tumours. Moreover, we update the genetic data of the various pathological entities. The main goal is to provide a tool to facilitate the work of the pathologists, haematologists and researchers involved in the diagnosis and treatment of these haematological malignancies.     

Diagnostic difficulties,therapeutic strategies,and performance of scoring systems in patients with autoimmune hepatitis and concurrent hepatitis B/C

Abstract

Background. The diagnosis of autoimmune hepatitis (AIH) is already difficult, and that of AIH with chronic viral hepatitis including hepatitis B (HBV) or hepatitis C (HCV) is even more challenging. To date, only a few case-based studies have described this association. Aim. The aim was to retrospectively assess diagnostic difficulties, therapeutic approaches, and performance of the scoring systems in AIH patients with concurrent HBV and HCV. Methods. A total of 25 patients from United States, Sweden, Italy, and Turkey were retrospectively evaluated. Both revised and simplified criteria suggested by the International Autoimmune Hepatitis Group were applied for each patient. All study data were obtained from medical records. Results. Of the 25 patients, 20 (80%) had concomitant HCV and 5 (20%) had HBV. Based on the revised scoring system and simplified criteria, 18 (72%) and 12 (48%) patients were diagnosed as “probable” AIH. None of the patients were diagnosed as “definite” AIH according to both scoring systems. Patients with HCV initially were treated with immunosuppressive agents, and antiviral therapy was commenced when biochemical remission occurred. AIH patients with HBV were first treated with antiviral and thereafter, immunosuppressive therapy was started. Conclusions. This large case series describes concurrent AIH and chronic viral hepatitis. The revised scoring system for AIH had a better performance than the simplified scoring system. However, neither scoring system is optimal for diagnosing AIH alone. In these patients, a definitive diagnosis of AIH should be based on a combination of serological profiles, histological findings, scoring systems, treatment response, and outcomes.