Mustafa Ozmen

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.

Subclinical Atherosclerosis in Systemic Sclerosis: Not Less Frequent Than Rheumatoid Arthritis and Not Detected With Cardiovascular Risk Indices.

To determine the frequency of subclinical atherosclerosis in patients with systemic sclerosis (SSc; scleroderma) compared to healthy subjects (HS) and rheumatoid arthritis (RA) patients and to determine the ability of cardiovascular (CV) risk indices in detecting SSc patients with subclinical atherosclerosis.

A rare case of systemic lupus erythematosus with chylous ascites and chylothorax.

During the course of the disease a patient with systemic lupus erythematosus (SLE) may develop inflammation of one or more serous membranes, resulting in pleural, peritoneal, or pericardial effusion. Chylous ascites and chylothorax have rarely been described in patients with SLE. Therefore, in parallel with the analysis of blood samples, detailed analysis of the effusions should be carried out. Supportive measures are often needed to relieve the symptoms of chylothorax or chylous ascites together with the treatment of the primary disease. The available literature had reported just 4 cases of chylous ascites and/or chylothorax in association with SLE, and this patient presented here is one of the rare cases apart from the reported ones.

Ground-glass-like hepatocellular inclusions in the course of adult-onset Still’s disease

Ground-glass hepatocytes are the cardinal biopsy feature of chronic hepatitis B virus (HBV) infection and may also be present in other specific conditions, including Lafora’s disease, cyanamide aversion therapy for alcohol use, patients with transplantation, uremia, and metabolic disorders. In this report, we present the case of a patient with adult-onset Still’s disease who underwent percutaneous liver biopsy, which revealed ground-glass-like cytoplasmic inclusions and which is a very unusual finding.

Serum procalcitonin levels in patients with ankylosing spondylitis

Procalcitonin is a marker of bacterial and fungal infection and sepsis. The present study evaluated the relationship between serum procalcitonin levels and disease activity in patients with ankylosing spondylitis (AS).

Clinical Factors Associated with the Diagnosis of Granulomatosis with Polyangiitis

Objective To determine the predictive value of nasal endoscopic findings and symptoms in the diagnosis of granulomatosis with polyangiitis (GPA). Study Design A cross-sectional study. Setting A tertiary university hospital. Subjects and Methods A total of 116 adults were enrolled in the study: 19 patients with GPA, 29 patients with other rheumatic diseases, and 68 healthy volunteers. All patients were examined with a flexible endoscope, and nasal endoscopic images were recorded and evaluated blindly. The medical history of each patient was taken by a physician blinded to the patient’s diagnosis. Results Univariate analysis indicated a statistically significant difference in rhinorrhea (P = .002), postnasal drip (P = .015), epistaxis (P < .001), and saddle nose (P = .017). However, binary logistic regression analysis demonstrated that only history of epistaxis (P = .012; odds ratio, 5.6) was statistically significant in predicting GPA. Univariate analysis showed a statistically significant difference in nasal secretion (P = .028), nasal septal perforation (P < .017), nasal crusting (P < .001), nasal adhesion (P < .001), nasal granuloma (P = .017), and hemorrhagic fragile nasal mucosa (P < .001). A binary logistic regression analysis demonstrated that only hemorrhagic fragile nasal mucosa (P < .001; odds ratio, 52.9) was a statistically significant predictor of GPA. Conclusions Given the results of this study, we believe that hemorrhagic fragile nasal mucosa and history of recurrent epistaxis may put patients at risk for GPA and should be investigated accordingly.

Retinal nerve fiber and optic disc morphology using spectral-domain optical coherence tomography in scleroderma patients.

Purpose To evaluate the optic nerve head parameters and peripapillary retinal nerve fiber layer using spectral-domain optical coherence tomography (SD-OCT) in a systemic sclerosis (SSc) cohort and age-matched controls to determine whether SSc patients have an increased risk of normal-tension glaucoma (NTG). Methods We examined 30 patients (3 male, 27 female) with SSc and 28 age- and sex-matched controls. Retinal nerve fiber and optic disc morphology were evaluated using Cirrus SD-OCT. Results Optic disc morphology measurements including disc area, rim area, average and vertical cup/disc (C/D) ratio, and cup volume were not significantly different between the study groups. The average and 4-quadrant retinal nerve fiber layer (RNFL) measurements of the C/D >0.3 subgroups were not significantly different in the patients and controls. These values were also similar for the C/D >0.5 subgroups except that the average inferior quadrant RNFL thickness of the right eyes in the patient subgroup was significantly thinner than in the control subgroup (p<0.05). Conclusions Our SSc cohort had relatively shorter disease duration but increased prevalence of early glaucomatous damage signs. Our findings indicate that SSc is a risk factor for developing normal-tension glaucoma. Further studies combined with visual field evaluation are necessary to identify the long-term glaucomatous effects of SSc.

Role of the mTOR pathway in minor salivary gland changes in Sjogren’s syndrome and systemic sclerosis

BackgroundTo examine the activity of the mammalian target of rapamycin (mTOR) pathway and its regulators, transforming growth factor (TGF)-β1 and phosphatase and tensin homolog (PTEN), in minor salivary gland biopsies of Sjogren’s syndrome (SS) and systemic sclerosis (SSc) patients.MethodsWe retrospectively evaluated SS, SSc, and SS-SSc overlap patients admitted to our outpatient rheumatology clinic between January 2007 and December 2015 who underwent a minor salivary gland biopsy. Patient demographics and some clinical features were obtained from hospital records. Immunohistochemistry was used to analyze total mTOR, total PTEN, and TGF-β1 expression in the biopsied tissues. The biopsy specimens were also examined for the presence and degree of fibrosis.ResultsMinor salivary gland biopsies of 58 SS, 14 SSc, and 23 SS-SSc overlap patients were included in the study. There was no significant difference in mTOR expression between these groups (P = 0.622). PTEN protein was expressed in 87.2% of patients with SS, 57.9% with overlap syndrome, and 100% of the SSC patients, and these differences were statistically different (P = 0.023). Although ductal epithelial TGF-β1 expression was similar between the groups (P = 0.345), acinar cell expression was found to be more frequent in the SSc (72.7%) and overlap patients (85.7%) in comparison with the SS cases (58.2%; P = 0.004).ConclusionmTOR may be one of the common pathways in the pathology of both SS and SSc. Hence, there may be a role for mTOR inhibitors in the treatment of both diseases. Additionally, PTEN and TGF-β1 expression may be a distinctive feature of SSc.

Evaluation of periostin and factors associated with new bone formation in ankylosing spondylitis: Periostin may be associated with the Wnt pathway

Periostin has been shown to be involved in bone anabolism through the regulation of Wnt‐β‐catenin signaling. It may be one of the pathogenic mechanisms in syndesmophyte formation in ankylosing spondylitis (AS). The aim of this study was to evaluate serum periostin levels in patients with AS and to assess relationships among biomarkers of bone formation and periostin in disease outcomes, particularly radiographic changes.

Choroidal and central foveal thickness in patients with scleroderma and its systemic associations

The aim was to investigate the morphological changes in the fovea and choroid in patients with scleroderma and its systemic associations.