Orhan Küçükşahin

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 Stills 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.

Association of serum KL-6 levels with interstitial lung disease in patients with connective tissue disease: a cross-sectional study

It was aimed to evaluate KL-6 glycoprotein levels to determine if it may be a diagnostic marker for the connective tissue diseases (CTDs) predicting CTD-related interstitial lung diseases (ILDs) (CTD-ILD) development and to examine if there was a difference between patients and healthy controls. The study included 113 patients with CTD (45 CTD without lung involvement, 68 CTD-ILD) and 45 healthy control subjects. KL-6 glycoprotein levels were analyzed with ELISA in patients and the control group. The relationship between KL-6 glycoprotein levels and CTD-ILD was assessed. In the comparison of all the groups in the study, significantly higher levels of KL-6 were determined in the CTD-ILD group than in either the CTD without pulmonary involvement group or the healthy control group (p < 0.008 and p < 0.001, respectively). There was no statistically significant difference between the KL-6 levels in the healthy control group and the CTD without pulmonary involvement group (p = 0.289). The KL-6 levels did not differ significantly according to the connective tissue diseases in the diagnostic groups (systemic lupus erythematosus, Sjögren’s syndrome, rheumatoid arthritis, mixed connective tissue disease, scleroderma, polymyositis/ dermatomyositis). In the healthy control group, there was a statistically significant difference between KL-6 levels in smokers and non-smokers. Smokers had significantly higher serum KL-6 levels compared with non-smokers (p < 0.05). There was no statistically significant difference between smoking status (pack-year) and serum KL-6 levels. There was no statistically significant correlation between serum KL-6 levels and time since diagnosis of CTD and CTD-ILD. The level of KL-6 as a predictive factor could be used to identify the clinical development of ILD before it is detected on imaging modality. Further prospective clinical studies are needed to define whether levels of KL-6 might have prognostic value or might predict progressive ILD.

Behçet's Disease and Intracardiac Thrombosis: A Report of Three Cases

We present three patients with Behçets disease associated with intracardiac thrombus and pulmonary vascular involvement. One of these patients had also Budd-Chiari syndrome. All patients were treated with corticosteroid plus monthly intravenous cyclophosphamide as first line treatment and with no recurrences. Immunosuppressive therapy was successful in the treatment of intracardiac thrombus and also in the regression of pulmonary vascular thromboses in these patients. Intracardiac thrombus in Behçets disease is rarely seen. Behçets disease should be remembered in the differential diagnosis of the patients with intracardiac mass, especially in patients from the Mediterranean and Middle East populations.

Spontaneous Coronary Artery Dissection in a Male Patient with Takayasu’s Arteritis and Antiphospholipid Antibody Syndrome

We present a case of a 34-year-old male who presented to the emergency ward with fever and abdominal pain. The diagnosis of Takayasus arteritis and also antiphospholipid syndrome was made during an imaging workup of deep-vein thrombosis. A spontaneous coronary artery dissection was revealed in coronary CT angiography requested for chest pain and dyspnea. The patient was treated medically and discharged on close followup. The concurrence of spontaneous coronary artery dissection with antiphospholipid syndrome and Takayasus arteritis has not been reported in the previous literature. The possibility of a spontaneous coronary artery dissection should be considered in patients presenting with both diseases.

Treatment resistant severe digital ischemia associated with antiphospholipid syndrome in a male patient with systemic sclerosis.

We report the case of a male patient with limited cutaneous systemic sclerosis (SSc) that was complicated with severe digital ischemia, resistant to medical treatment. Due to the lack of treatment response, further laboratory and imaging studies were conducted. Findings were compatible with antiphospholipid syndrome and oral warfarin was added to the treatment regimen. After successful anticoagulation no further recurrences of digital ischemia were seen. An underlying etiology in SSc patients with treatment resistant digital ischemic necrosis should be suspected for accompanying antiphospholipid syndrome (APS).

Response to rituximab in a case of lupus associated digital ischemia.

We report the case of a 38-year-old female patient with systemic lupus erythematosus (SLE) and Jaccoud arthritis (JA) that sequentially developed digital ischemic lesions of the hands. In spite of follow-up treatment with glucocorticoids, immunosuppressant, antiaggregant, and potent vasodilatator agents, a serious progression to digital gangrene over a one-month period was observed. Surprisingly, her nonhealing digital lesions improved after two cycles of rituximab (RTX) administration.

A Case of Amyopathic Dermatomyositis with Pneumomediastinum and Subcutaneous Emphysema

A 34-year-old man was admitted with dyspnea, cough, and fever. Thorax computed tomography revealed ground glass opacities and pneumomediastinum. The patient was diagnosed as amyopathic dermatomyositis due to skin lesions and radiological findings. Despite immunosuppressive treatment clinical deterioration and radiological progression were observed and the patient died because of severe hypoxemic respiratory failure. The patient presented with extremely rare occurrence of pneumomediastinum and subcutaneous emphysema in amyopathic dermatomyositis with a poor prognosis.

Autoimmune disease, tumor necrosis factor inhibitors and acute leukemia: possible associations in two patients?

articles do not necessarily reflect those of Informa Healthcare (the Publisher), the Editors or the journal. The Publisher does not assume any responsibility for any injury and/or damage to persons or property arising from or related to any use of the material contained in these articles. The reader is advised to check the appropriate medical literature and the product information currently provided by the manufacturer of each drug to be administered to verify the dosages, the method and duration of administration, and contraindications. It is the responsibility of the treating physician or other health care professional, relying on his or her independent experience and knowledge of the patient, to determine drug dosages and the best treatment for the patient.

Serum interleukin-37 level and interleukin-37 gene polymorphism in patients with Behçet disease

Behçet’s disease (BD) is a chronic inflammatory disease. The etiopathogenesis of BD is not well understood and several cytokines and genetic factors have been investigated. Interleukin (IL)-37, which a member of IL-1 family is an anti-inflammatory cytokine. The aim of the study was to analyze serum IL-37 level and IL-37 gene polymorphisms to assess its possible role in BD. Two hundred twenty-three patients with BD and 80 healthy controls (HC) were enrolled. Serum IL-37 level was measured using an enzyme-linked immunosorbent assay (ELISA). Deoksiribo Nucleic acids (DNA) were extracted using a genomic DNA isolation kit. Single nucleotide polymorphism (SNP) of IL-37 gene (rs3811047) was performed using polymerase chain reaction-restriction fragment length polymorphism (PCR/RFLP) methods. Serum IL-37 level was not significantly different in BD and HC (p > 0.05). Serum IL-37 level was not associated with the disease activity (p > 0.05). However, its level was higher in mucocutaneous involvement compared with systemic involvement (p = 0.002) and HC (p = 0.005). IL-37 gene polymorphisms were similar in BD and HC (p > 0.05). IL-37 may play a role in the etiopathogenesis of BD by contributing to manifestation with more moderate clinical symptoms.

Do the European League Against Rheumatism (EULAR) Sjögren’s syndrome outcome measures correlate with impaired quality of life, fatigue, anxiety and depression in primary Sjögren’s syndrome?

Introduction The aim of the study was to investigate whether there is a relationship between the European League Against Rheumatism (EULAR) outcome measures and quality of life (QoL), fatigue, anxiety and depression in patients with pSS and to define determinants which could affect quality of life. Material and methods The study included 105 pSS patients and 72 age/sex-matched healthy controls (HCs). Cross-sectional clinical data were collected, including the Hospital Anxiety and Depression Scale (HADS), the Multidimensional Assessment of Fatigue (MAF) scale, the Short Form (SF-36), EULAR Sjögren’s syndrome disease activity index (ESSDAI) and EULAR Sjögren’s syndrome patient reported index (ESSPRI). Results The SF-36 scores were significantly lower and anxiety, depression and fatigue scores were significantly higher in the pSS group than in the control group (all p-value < 0.05). ESSDAI was negatively correlated with SF-36 scores and positively with MAF. ESSPRI was negatively correlated with SF-36 scores except for the mental health subdimension, and a positive correlation was determined with MAF, HADS-A and HADS-D. Multiple linear regression analysis revealed that HADS-A, HADS-D, MAF, ESSPRI and ESSDAI were associated with most SF-36 subscales. Conclusions The results of this study provide further evidence supporting the use of ESSDAI and ESSPRI in daily practice. Quality of life was diminished in patients with pSS and was associated with different symptoms. This should be taken into account when managing patients with pSS.