Clinical characteristics and disease course before and after SARS-CoV-2 infection in a large cohort of systemic sclerosis patients.

Background/aim: The objective of this study is to evaluate the clinical presentations and adverse outcomes of Coronavirus Disease 2019 (COVID-19) in patients with systemic sclerosis (SSc) and assess the impact of SSc features on the clinical course of COVID-19. Materials and methods: In this multicenter, retrospective study, SSc patients with COVID-19 were included. Clinical features of SSc, along with detailed COVID-19 data, were extracted from medical records and patient interviews. Results: The study included 112 patients (mean age 51.4 ± 12.8 years; 90.2% female). SSc-associated interstitial lung disease (ILD) was evident in 57.1% of the patients. The findings revealed hospitalization in 25.5%, respiratory support in 16.3%, intensive care unit admission in 3.6%, and a mortality rate of 2.7% among SSc patients with COVID-19. Risk factors for respiratory failure, identified through univariate analysis, included ILD (OR: 7.49, 95% CI: 1.63-34.46), ≥1 comorbidity (OR: 4.55, 95% CI: 1.39-14.88), a higher physician global assessment score at the last outpatient visit (OR 2.73, 95% CI: 1.22-6.10), and the use of mycophenolate at the time of infection (OR: 5.16, 95 %CI: 1.79-14.99). Notably, ≥1 comorbidity emerged as the sole significant predictor of the need for respiratory support in COVID-19 (OR: 5.78, 95% CI: 1.14-29.23). In the early post-COVID-19 period, 17% of patients reported the progression of the Raynaud phenomenon, and 10.6% developed new digital ulcers. Furthermore, progression or new onset of dyspnea and cough were detected in 28.3% and 11.4% of patients, respectively. Conclusion: This study suggests a potential association between adverse outcomes of COVID-19 and SSc-related ILD, severe disease activity, and the use of mycophenolate. Additionally, it highlights that having comorbidities is an independent risk factor for the need for respiratory support in COVID-19 cases.

Does decision tree analysis predict oral ulcer activity-related factors in patients with Behçet's syndrome?

OBJECTIVES: The study aimed to identify the interactions among treatment protocols and oral ulcer activity related factors in patients with Behçet's syndrome (BS) using the Classification and Regression Tree (CART) algorithm. METHODS: In this cross-sectional study, 979 patients with BS were included from16 centres in Turkey, Jordan, Brazil and the United Kingdom. In the CART algorithm, activities of oral ulcer (active vs. inactive), genital ulcer (active vs. inactive), cutaneous involvement (active vs. inactive), musculoskeletal involvement (active vs. inactive), gender (male vs. female), disease severity (mucocutaneous and musculoskeletal involvement vs. major organ involvement), smoking habits (current smoker vs. non-smoker), tooth brushing habits (irregular vs. regular), were input variables. The treatment protocols regarding immunosuppressive (IS) or non-IS medications were the target variable used to split from parent nodes to purer child nodes in the study. RESULTS: In mucocutaneous and musculoskeletal involvement (n=538), the ratio of IS use was higher in patients with irregular toothbrushing (ITB) habits (27.1%) than in patients with regular toothbrushing (RTB) habits (14.2%) in oral ulcer activity. In major organ involvement (n=441), male patients with ITB habits were more likely treated with IS medications compared to those with RTB habits (91.6% vs. 77.6%, respectively). CONCLUSIONS: Male BS patients on IS who have major organ involvement and oral ulcer activity with mucocutaneous and musculoskeletal involvement have irregular toothbrushing habits. Improved oral hygiene practices should be considered to be an integral part for implementing patient empowerment strategies for BS.

Age-related differences in the clinical phenotypes of Behçet's disease: The experience of two referral centres.

OBJECTIVES: Behçet's disease (BD) is a multisystem disease and frequently occurs during the second-fourth decades of life, although disease onset may be seen at any age. This study aimed to analyze the influence of the age of onset on clinical manifestations of BD. MATERIALS AND METHODS: This retrospective study analyzed two cohorts (paediatric and adult) to determine the association between the age of onset and clinical features in BD. Patients were classified into four groups to analyze the clinical characteristics according to the age of fulfilling the BD diagnostic criteria as follows: childhood onset (<12 years), adolescent onset (13-17 years), adult onset (18-39 years), and late onset (>40 years). RESULTS: The study included 801 patients with BD. Male predominance, pathergy test positivity, aphthosis (oral or genital), and skin and ocular involvements were higher among adult patients than paediatric patients. The presence of positive family history for BD, neuro-BD, and epididymitis were observed significantly common in the paediatric group. CONCLUSION: There may be differences in clinical manifestations with regard to the age of disease onset. Disease presentations may differ from adult patients, and clinicians should be aware of the high familial aggregation rate of BD, especially in countries where the disease is endemic.

The Risk of Presarcopenia Is Increased Among Female Patients With Primary Sjögren's Syndrome.

OBJECTIVES: Sarcopenia is a progressive and generalized loss of muscle mass and function. The aim of this study was to evaluate the frequency of sarcopenia among patients with primary Sjögren's syndrome (SS) and the factors related with sarcopenia. METHODS: Forty-four female patients with primary SS and 44 female control subjects were included in this cross-sectional study between February and August 2019. Sarcopenia was evaluated by the handgrip test, Skeletal Muscle Mass Index, and gait speed test. RESULTS: Eleven patients (25.0%) had presarcopenia in the SS group and 2 (4.5%) in the control group (p = 0.007). Compared with control subjects, SS patients had lower results of hand grip and gait speed tests (p = 0.005 and p < 0.001, respectively). According to the Mini Nutritional Assessment Short Form, patients with presarcopenia had higher risk of malnutrition compared with patients without sarcopenia (p = 0.043). Patients with presarcopenia had higher scores in the European League Against Rheumatism Sjögren's Syndrome Patient-Reported Index pain domain and patient visual analog scale for global disease activity compared with patients without sarcopenia (p = 0.044 and p = 0.036, respectively). In multivariate regression analysis, European League Against Rheumatism Sjögren's Syndrome Patient-Reported Index pain was associated with hand grip strength (p = 0.016, R2 = 0.13) and Mini Nutritional Assessment Short Form was associated with Skeletal Muscle Mass Index (p = 0.005). CONCLUSIONS: Risk of sarcopenia is increased in patients with SS. Pain and malnutrition may contribute to presarcopenia. Evaluating pain and patient's global disease activity may help physicians to determine patients with increased risk of sarcopenia. Controlling disease activity and pain and preventing malnutrition may reduce the risk of development of sarcopenia.

Physicians' Biological Drug Preference in Patients With Rheumatoid Arthritis and Spondyloarthritis With a History of Malignancy: Perspectives From the Treasure Database.

OBJECTIVE: Because of concerns about malignancy risks, using biological disease-modifying antirheumatic drugs (bDMARDs) in patients with a history of malignancy remains a challenging issue in rheumatology practice. This study aimed to investigate bDMARD preferences of physicians when treating of rheumatoid arthritis (RA) and spondyloarthritis (SpA) patients with a history of malignancy. METHODS: The data for this cross-sectional study were gathered from the TReasure database using a date range of December 2017 and January 2020. Biological disease-modifying antirheumatic drug preferences were analyzed for 40 RA patients and 25 SpA patients with a history of malignancy. RESULTS: The most frequently prescribed bDMARD was rituximab, which was given to 28 RA patients (70%). For 25 patients (62.5%), the time between the diagnosis of malignancy and starting on a bDMARD regimen was less than 60 months, with a median interval of 43.5 months. Among SpA patients, the preferred bDMARDs were secukinumab and etanercept, which were each administered to 7 patients (28%). For 13 SpA patients (52%), the time between the diagnosis of malignancy and starting on bDMARDs was less than 60 months, with a median interval of 97 months. CONCLUSIONS: The observed bDMARD preferences may be related to the therapeutic effects of rituximab on lymphoproliferative malignancies, the protective effects of secukinumab on tumor progression, and the short half-life of etanercept. Biological disease-modifying antirheumatic drugs should be used in RA and SpA patients with malignancy in case of high inflammatory activity.

In the era of disease-modifying antirheumatic drugs, how close are we to treating rheumatoid arthritis without the use of glucocorticoids?

We wanted to see how close we could get to our goal of treating rheumatoid arthritis (RA) without the use of glucocorticoids (GCs) in the disease-modifying antirheumatic drugs (DMARDs) era using real-life data. Established in 2017, the TReasure database is a web-based, prospective, observational cohort for Turkey. As of May 2019, there were 2,690 RA patients recorded as receiving biologic and targeted synthetic DMARDs (bDMARDs and tsDMARDs) therapy. At the start of the bDMARDs or tsDMARDs, patients with follow-up visits of at least 3 months were registered. At the time of registration and the last visit, doses of GCs were recorded and it was determined if the target dose of ≤ 7.5 mg was achieved. During registration and follow-up, 23.4% of the patients did not receive GCs and 76.5% of the patients received GCs at any time. GCs could be stopped after 59 (25-116) months in 28.4% of these patients, but 71.6% of patients were still using GC. The target GC dose could not be achieved in 18.2% of these patients (n = 352). The rate of continuing to use GC was significantly higher in women, in the elderly, those with rheumatoid factor (RF) positive, with higher Visual Analog Scale (VAS) pain and Disease Activity Score (DAS)-28. The initial GC dose of ≥ 7.5 mg/day was found to be crucial in not reaching the GC target dose (p < 0.001, OR 39.0 (24.1-63.2)). The initial GC dose of ≥ 7.5 mg/day, female gender, age, RF positivity, high DAS28, and VAS pain level were all highly related for GC continuation. Despite the use of DMARDs, our data revealed that we are still far from achieving our goal of treating RA without using steroids.

The assessment of tocilizumab therapy on recurrent attacks of patients with familial Mediterranean fever: A retrospective study of 15 patients.

AIM: Interleukin-6 receptor antagonist, tocilizumab (TCZ), is known to be effective in the treatment of amyloidosis in patients with familial Mediterranean fever (FMF). But there are limited data about the effect of TCZ on frequency of attacks. In the current study, we aimed to find out whether TCZ therapy could decrease the frequency of recurrent attacks of FMF or not. MATERIALS AND METHODS: The recorded files of 15 patients who had received intravenous TCZ for the improvement of amyloidosis associated with FMF, were evaluated retrospectively. Data of demographic and clinical characteristics of patients were archived from those files. RESULTS: Three female and 12 male patients received TCZ due to amyloidosis were included to the study. The mean age was 42.07 ± 14.37 years. All of the patients were in full compliance with colchicine treatment. According to international severity scoring system for FMF, all of the patients had severe disease. The frequency of attacks recorded was evaluated during TCZ treatment, and it was reported that one patient had no response, six patients had decreased attack frequency and eight patients had no attacks. DISCUSSION: Tocilizumab is found to be efficient on improvement of amyloidosis and decreasing the frequency of recurrent attacks in patients with FMF. Besides, TCZ is well tolerated among the patients. Further and prospective studies with larger sample are needed to support these results.

Comparison of vitamin D levels between patients with pterygium and healthy subjects.

PURPOSE: The purpose of this study was to compare vitamin D (VD) deficiency frequency among patients with pterygium to that of healthy subjects and to investigate the VD deficiency among patients with pterygium. METHODS: One-hundred eight pterygium patients and 94 healthy subjects were included in the study. Blood samples were collected from groups during the same time interval and the samples were saved. Serum 25-hydroxyvitamin D (25(OH)D) and parathormone (PTH) levels were measured and analyzed. RESULTS: The pterygium group consisted of 57 female and 51 male patients, while the control group consisted of 47 female and 47 male patients. The mean age, sex and mean BMI were similar in the two groups (p > 0.05). The percentage of time that individuals spent outdoors was higher in the pterygium group (p = 0.02). The percentage of VD deficiency was 83.3% in the pterygium group and 61.7% in the control group (p = 0.001). There was a positive correlation between VD and time spent outdoors (p < 0.05). CONCLUSIONS: VD deficiency can play a role in pterygium etiopathogenesis. Wide population-based studies in different regions are needed to evaluate this result.

Non-adherence to colchicine treatment is a common misevaluation in familial Mediterranean fever

Background/aim: Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease that requires lifelong colchicine treatment. Colchicine is the mainstay of the treatment, which decreases the frequency and the severity of recurrent FMF attacks and prevents the development of amyloidosis. This study aimed to investigate the rates of colchicine treatment adherence in patients with FMF and the factors related to treatment non-adherence. Materials and methods: This observational study was conducted with 179 patients with FMF between November 2018 and April 2019 in a tertiary rheumatology outpatient clinic. The sociodemographic and clinical data were recorded. Compliance Questionnaire on Rheumatology (CQR) was used to assess the treatment adherence and the Beliefs About Medicines Questionnaire (BMQ-T) was used to assess a patient's beliefs about colchicine. The factors associated with adherence to the treatment were evaluated. Results: The study included 113 male (63.1%) and 66 (36.9%) female patients with a mean age of 30 (25­44) years. The rate of the patients who declared regular colchicine usage was 66.5%. The frequency of non-adherent patients was 83.8% according to CQR. Treatment adherence was better in patients with comorbid diseases than those without (41.4% vs. 22%, respectively, p = 0.028). The frequency of married patients in the adherent group (72.4%) was higher than the non-adherent group (47.3%) (p = 0.013). The colchicine dose used in the adherent group was 1.5 (1.3­1.8) mg/day, whereas it was 1.5 (1.0­1.5) mg/day in the non-adherent group (p = 0.033). The adherence rate was rising with increasing scores of BMQ-T Specific Necessity. As the scores of BMQ-T General Overuse and General Harm increased, non-adherence to colchicine increased. Conclusion: Evaluating adherence to colchicine treatment with objective methods is crucial to ensure sufficient treatment and prevent amyloidosis. Determining beliefs about colchicine may increase patients' adherence to treatment.

Treatment of idiopathic granulomatous mastitis and factors related with disease recurrence

Background/aim: Idiopathic granulomatous mastitis is a rare, benign inflammatory disease of breast. There is no general agreement on the appropriate treatment choice. The aim of the study was to investigate the immunosuppressive administer for idiopathic granulomatous mastitis and risk factors related with disease recurrence. Materials and methods: The data of 53 patients with idiopathic granulomatous mastitis were evaluated for this cross-sectional retrospective study. Demographic features and clinical characteristics and course of the patients were obtained from file records. Results: The mean age of the patients was 37.2 ± 6.6 years. Fifty-one of 53 patients received immunosuppressive treatment with or without surgery. Forty-seven (88.6%) of the patients received only immunosuppressive treatment without surgery, while 4 (7.54%) patients received immunosuppressive treatment after surgery. Forty-one (77.3%) of 47 patients who had no surgical resection received methotrexate as immunosuppressive treatment. The other 6 (11.3%) patients received azathioprine or corticosteroid treatment. Complete or partial remission was observed in 50 (98%) of 51 patients who received immunosuppressive treatment, while only 1 (2%) patient did not reach remission. No factors were found related with recurrence of disease. Conclusion: Methotrexate seems to be efficient in the treatment of idiopathic granulomatous mastitis and provides drug-free remission.

Oral ulcer activity assessment with the composite index according to different treatment modalities in Behçet's syndrome: a multicentre study.

OBJECTIVES: The aim of this multicentre study was to understand patients' needs and to evaluate the oral ulcer activity with the Composite Index (CI), according to different treatment modalities in Behçet's syndrome (BS). METHODS: BS patients (n=834) from 12 centres participated in this cross-sectional study. Oral ulcer activity (active vs. inactive) and the CI (0: inactive vs. 1-10 points: active) were evaluated during the previous month. The effects of treatment protocols [non-immunosuppressive: non-IS vs. immunosuppressive: (ISs)], severity (mild vs. severe), disease duration (<5 years vs. ≥5 years) and smoking pattern (non-smoker vs. current smoker) were analysed for oral ulcer activity. RESULTS: Oral ulcer activity was observed in 65.1% of the group (n=543). In both genders, the activity was higher in mild disease course with non-IS treatment group compared to severe course with ISs (p<0.05). As a resistant group, patients with mild disease course whose mucocutaneous symptoms were unresponsive to non-IS medications were treated with ISs in a limited period and achieved the highest CI scores in females. Oral ulcer activity and poor CI score were associated with disease duration less than 5 years compared to others in male patients (p<0.05). CONCLUSIONS: Oral ulcer activity pattern is affected by both the combination of disease course, treatment protocols and disease duration. CI scores reflected the oral clinical activity and CI might be a candidate scale to evaluate the efficacy of treatments during the follow-up of oral ulcer activity in BS.

Comparison of nitric oxide level in Behçet's disease patients with or without psychiatric comorbidity.

Both Behçet's disease (BD) and major depressive disorder (MDD) are diseases associated with nitric oxide (NO). We hypothesized that the comorbidity of MDD affects the levels of NO in BD. In this study, we investigate whether there was a difference in NO levels among BD patients with accompanying MDD, BD patients with no depressive symptoms and healthy control group. There was a significant difference in NO levels among BD and control group (P < 0.05). Also, there was a significant difference between the BD group with MDD and BD group without psychiatric comorbidity in terms of NO levels (P < 0.05). This study is interesting as it demonstrates that accompanying psychiatric comorbidity puts an additional NO burden on the shoulders of BD patients.

The Spectrum of ß-Thalassemia Mutations in Siirt Province, Southeastern Turkey.

ß-Thalassemia (ß-thal) is the most common hereditary genetic blood disorder. The aims of this study were: (i) to determine the mutation types and the frequency of these mutations in ß-thal patients to obtain the ethnic origins of the population in Siirt Province; (ii) to evaluate the pathogenicity of these mutations by performing in silico analysis; (iii) to reveal the genotype-phenotype correlation by comparing the clinical manifestation of our patients to the specific mutations in this population. This study included 34 patients (18 males and 16 females) with a mean age of 9.1 ± 3.6 years (range 3-16 years). All mutations were determined using sequence analysis methods, and the mutations were analyzed using bioinformatics tools. Thirteen different mutations were detected in the patients: IVI-I-110 (G>A) (HBB: c.93-21G>A) (38.9%); IVS-II-1 (G>A) (HBB: c.315_1G>A) (11.1%); -30 (T>A) (HBB: c.-80T>A) (9.25%) and IVS-I-1 (G>A) (HBB: c.92 + 1G>A) (9.25%), were the most common, and these mutations constituted 68.5% of the cases. Missense codon 6 (A>T) (HBB: c.20A>T) was not pathogenic; however, all the intronic mutations (IVS-I-1, IVS-I-110, IVS-II-1) and frameshift mutations [codon 44 (-C) (HBB: c.135delC) and codons 36/37 (-T) (HBB: c.112delT)] resulted in disease. These mutations can be used to determine the ethnic origin of the Siirt population and, in affected pregnant women, to develop prenatal strategies. A fatal phenotype can be identified by in silico analysis; however, mutations that are unknown prior to marriage, pregnancy, and childbirth or new mutations can be less accurately identified.

The distribution of MEFV mutations in Turkish FMF patients: multicenter study representing results of Anatolia

Background/aim: The distribution of Mediterranean fever (MEFV) gene mutations in Turkish familial Mediterranean fever (FMF) patients varies according to geographic area of Turkey. There is a need for highly representative data for Turkish FMF patients. The aim of our study was to investigate the distribution of the common MEFV mutations in Turkish FMF patients in a nationwide, multicenter study. Materials and methods: Data of the 2246 FMF patients, from 15 adult rheumatology clinics located in different parts of the country, were evaluated retrospectively. The following mutations have been tested in all patients: M694V, M680I, M694I, V726A, and E148Q. Results: There were 1719 FMF patients with available genetic testing. According to the genotyping, homozygous M694V, present in 413 patients (24%), was the most common mutation . One hundred and fifty-four (9%) of patients had no detectable mutations. Allele frequencies of common mutations were: M694V (n = 1529, 44.5%), M680I (n = 423, 12.3%), V726A (n = 315, 9.2%), E148Q (n = 214, 1%), and M694I (n = 12, <1%). Conclusion: In this large-scale multicenter study, we provided information about the frequencies of common MEFV gene mutations obtained from adult Turkish FMF patients. Nearly half of the patients were carrying at least one M694V mutations in their alleles.

Methodology of a new inflammatory arthritis registry: TReasure

Background/aim: The TReasure registry, created in 2017, is an observational multicenter cohort that includes inflammatory arthritis patients. This article reviews the methodology and objectives of the TReasure registry established to collect data from rheumatoid arthritis (RA) and spondyloarthritis (SpA) patients. Methodology: Fifteen rheumatology centers in Turkey will contribute data to the TReasure database. The actual proprietor of the database is the Hacettepe Rheumatology Association (HRD) and Hacettepe Financial Enterprises. Pharmaceutical companies that operate in Turkey (in alphabetical or er), Abbvie, Amgen, BMS, Celltrion Healthcare, Novartis, Pfizer, Roche, and UCB, support the TReasure registry. TReasure is a web-based database to which users connect through a URL (https://www.trials-network.org/treasure) with their unique identifier and passwords provided for data entry and access. TReasure records demographic and clinical features, comorbidities, radiology and laboratory results, measures of disease activity, and treatment data. Discussion: TReasure will provide us with various types of data, such as a cross-sectional view of the current nationwide status of the patients currently receiving these treatments, and retrospective data as much as allowed by the participating centers' records. Finally, a high-quality prospective dataset will be built over the ensuing years from patients with a new diagnosis of RA or SpA.

Criteria sets for primary Sjogren's syndrome are not adequate for those presenting with extraglandular organ involvements as their dominant clinical features.

Patients with primary Sjogren's syndrome (pSS) may go undiagnosed or be misclassified due to the insidious nature and wide spectrum of the disease. The available several classification criteria emphasize glandular findings. We aimed to analyze the efficiency of various classification criteria sets in patients diagnosed on the clinical basis by expert opinion and to compare those pSS patients who fulfilled these criteria with those who did not. This is a multicenter study in which 834 patients from 22 university-based rheumatology clinics are included. Diagnosis of pSS was made on the clinical basis by the expert opinion. In this study, we only interviewed patients once and collected available data from the medical records. The European criteria, American-European Consensus Group (AECG) and American College of Rheumatology (ACR) Sjogren's criteria were applied. Majority of the patients were women (F/M was 20/1). The median duration from the first pSS-related symptom to diagnosis was significantly shorter in men (2.5 ± 2.3 vs 4.3 ± 5.9 years) (p = 0 < 0.016). When the European, AECG and ACR Sjogren's criteria were applied, 666 patients (79.9%) satisfied at least one of them. In total, 539 patients (64.4%) satisfied the European, 439 (52.6%) satisfied the AECG, and 359 (43%) satisfied the ACR criteria. Among the entire group, 250 patients (29.9%) satisfied all and 168 (20.1%) met none of the criteria. The rates of extraglandular organ involvements were not different between patients who met at least one of the criteria sets and those who met none. There is an urgent need for the modification of the pSS criteria sets to prevent exclusion of patients with extraglandular involvements as the dominant clinical features.

Metformin HCl has curative effect on rebuilding of ventricular diastolic functions in high-fat-diet fed rats.

Myocardial lipid accumulation due to diabetes and/or obesity plays a role in the progression of left ventricular diastolic dysfunction. Our aims were to exhibit the correlation between histopathologic stage of the liver and cardiac functions, and to evaluate the effects of metformin HCl and rosiglitazone on myocardial functions. Thirty-two male Sprague-Dawley rats were divided into four groups to exhibit the correlation between histopathologic stage of the liver and cardiac functions and to determine whether metformin HCl and rosiglitazone have effects on cardiac functions. For 20 weeks, one group was fed standard rat basic diet, whereas the other groups were on high-fat-diet. During the last 4 weeks, metformin HCl was given to the third group, rosiglitazone to the fourth group. Histological evaluation of rat livers yielded significantly higher steatosis grade in high-fat-diet group and different fibrosis stages among groups. Also, there was significant correlation between diastolic functions and steatosis grade/fibrosis stage of rat liver. Electrophysiological study of hearts via Langendorff technique showed better coronary perfusion pressures and diastolic functions in standard-diet and metformin HCl groups compared to other groups. Metformin HCl improves LV diastolic dysfunction and coronary perfusion pressures.

Response rate of initial conventional treatments, disease course, and related factors of patients with adult-onset Still's disease: Data from a large multicenter cohort.

BACKGROUND: Adult-onset Still's disease (AOSD) is a rare condition, and treatment choices are frequently dependent on expert opinions. The objectives of the present study were to assess treatment modalities, disease course, and the factors influencing the outcome of patients with AOSD. METHODS: A multicenter study was used to reach sufficient patient numbers. The diagnosis of AOSD was based on the Yamaguchi criteria. The data collected included patient age, gender, age at the time of diagnosis, delay time for the diagnosis, typical AOSD rash, arthralgia, arthritis, myalgia, sore throat, lymphadenopathy, hepatomegaly, splenomegaly, pleuritis, pericarditis, and other rare findings. The laboratory findings of the patients were also recorded. The drugs initiated after the establishment of a diagnosis and the induction of remission with the first treatment was recorded. Disease patterns and related factors were also investigated. A multivariate analysis was performed to assess the factors related to remission. RESULTS: The initial data of 356 patients (210 females; 59%) from 19 centers were evaluated. The median age at onset was 32 (16-88) years, and the median follow-up time was 22 months (0-180). Fever (95.8%), arthralgia (94.9%), typical AOSD rash (66.9%), arthritis (64.6%), sore throat (63.5%), and myalgia (52.8%) were the most frequent clinical features. It was found that 254 of the 306 patients (83.0%) displayed remission with the initial treatment, including corticosteroids plus methotrexate with or without other disease-modifying antirheumatic drugs. The multivariate analysis revealed that the male sex, delayed diagnosis of more than 6 months, failure to achieve remission with initial treatment, and arthritis involving wrist/elbow joints were related to the chronic disease course. CONCLUSION: Induction of remission with initial treatment was achieved in the majority of AOSD patients. Failure to achieve remission with initial treatment as well as a delayed diagnosis implicated a chronic disease course in AOSD.