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1.
Int J Gen Med ; 17: 2981-2991, 2024.
Article in English | MEDLINE | ID: mdl-39006915

ABSTRACT

Purpose: Primary Sjögren's syndrome (PSS) has many effects such as fatigue, pain, physical activity limitation and sleep disturbance, which limit patient's daily and social lives. The aim of our study was to assess fatigue, depression, physical activity status and quality of life in patients with PSS, and to determine the relationship between these data and disease-related parameters. Patients and Methods: This study was conducted with 117 primary Sjögren's syndrome patients. Demographic and anthropometric characteristics, disease activity (ESSDAI), quality of life scale (SF36), depression (Beck Depression Scale), physical activity status (International Physical Activity Questionnaire Short Form (IPAQ) score) and sleep status (Pittsburgh Sleep Quality Scale) of PSS patients were evaluated and relationships have been examined. Results: According to the results of our study, we found that sleep disorders are common in PSS patients (74.4%). Overweight patients, particularly higher lean mass sleep better (r:-0.201, p:0.043). Poor sleep causes fatigue (p=0.062) and depression (p=0.030). Sleep disturbance could not be explained by depressive state alone. However, after controlling for depression, the effect of sleep on fatigue seriously decreases (p=0.311). Exercise did not improve sleep quality (p=0.35) and the rate of poor sleep was higher among who exercised (p=0.192). Conclusion: Based on the results of our study, we believe that, treating depression in PSS patients is crucial for reducing fatigue. Patients need education on performing the correct exercises and weight gain should done in a professional manner. Gaining a deeper understanding of the multisystem involvement of the disease and the impact of exercise on the disease, will have positive effects on patient care and treatment decisions.

2.
Int J Rheum Dis ; 27(1): e14984, 2024 Jan.
Article in English | MEDLINE | ID: mdl-38014450

ABSTRACT

Systemic AA amyloidosis is associated with poorly controlled chronic inflammatory disorders. Chronic infections and inflammatory arthritis are the most common causes; however, they can also rarely occur as a complication of neoplastic disorders. The development of AA amyloidosis secondary to paraganglioma, which is a rare type of tumor, has rarely been reported in the literature. In this case, an 85-year-old female patient with a glomus tumor in the neck, who has been followed up over 50 years, applied with complaints of loss of appetite, nausea, and diarrhea for 5-6 months. While evaluating the patient, who had high levels of acute phase reactants, amyloidosis was diagnosed by salivary gland biopsy. No other cause was found to explain amyloidosis. The patient, who could not tolerate treatment with colchicine and azathioprine, is successfully treated with the interleukin-1 inhibitor anakinra. A rare relationship, systemic AA amyloidosis, which is thought to have developed as a result of long-standing jugular paraganglioma, is presented in this article. In addition, publications showing an association between paragangliomas and amyloidosis were reviewed.


Subject(s)
Amyloidosis , Glomus Tumor , Immunoglobulin Light-chain Amyloidosis , Paraganglioma , Female , Humans , Aged, 80 and over , Interleukin 1 Receptor Antagonist Protein/therapeutic use , Glomus Tumor/complications , Amyloidosis/complications , Amyloidosis/diagnosis , Amyloidosis/drug therapy , Immunoglobulin Light-chain Amyloidosis/complications , Paraganglioma/complications , Serum Amyloid A Protein
3.
Int J Rheum Dis ; 26(12): 2563-2566, 2023 Dec.
Article in English | MEDLINE | ID: mdl-37294068

ABSTRACT

There are very few cases in the literature on the coexistence of Sjögren's syndrome and pulmonary nodular amyloidosis being treated with rituximab. When nodules with central calcification and cystic lesions are seen on computed tomography, amyloid lung should be considered. Biopsy is recommended as it can be confused with malignancies. In this article, we present a 66-year-old female patient who has been followed up for Sjögren's syndrome for 26 years. Multiple cystic lesions with central calcification in the lung were detected and it was evaluated as amyloid nodule in the biopsy performed. The patient is being followed and is stable under rituximab treatment. Pulmonary noduler amyloidosis is very rare in Sjögren patients and there are very few cases where rituximab is used for treatment. We decided to publish in order to guide clinicians who will encounter similar cases.


Subject(s)
Amyloidosis , Lung Diseases , Sjogren's Syndrome , Female , Humans , Aged , Sjogren's Syndrome/complications , Sjogren's Syndrome/diagnosis , Sjogren's Syndrome/drug therapy , Rituximab/therapeutic use , Lung Diseases/complications , Lung Diseases/diagnosis , Lung Diseases/drug therapy , Lung/diagnostic imaging , Lung/pathology , Amyloidosis/complications , Amyloidosis/diagnosis , Amyloidosis/drug therapy
4.
Turk J Phys Med Rehabil ; 66(3): 299-306, 2020 Sep.
Article in English | MEDLINE | ID: mdl-33089086

ABSTRACT

OBJECTIVES: The aim of this study was to adapt the Assessment of Knowledge in Ankylosing Spondylitis Patients by a Self-Administered Questionnaire for the Turkish ankylosing spondylitis (AS) patients. PATIENTS AND METHODS: Between May 2016 and December 2016, a total of 100 AS patients (72 males, 28 females; mean age 43.4 years; range, 21 to 73 years) were included in the study. A forward (into Turkish) and backward translation of the questionnaire was performed. Reliability was evaluated using the Cronbach alpha (α) value, test-retest reliability, and intra-class correlations (ICCs). The correlations with demographic data including age, sex, time since diagnosis, and education status and with the disease-specific assessments including Bath Ankylosing Spondylitis Functional Index (BASFI), Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), and the Ankylosing Spondylitis Quality of Life (ASQOL) questionnaire were investigated. RESULTS: The Turkish version of the questionnaire showed a good reliability (Cronbach-α: >0.70, ICC: >0.90). A significant correlation was found with the education status (p<0.001). However, no significant correlation was observed between the questionnaire and the other parameters (p>0.05). CONCLUSION: Our study results show that the Turkish version of the questionnaire seems to be reliable for use in Turkish AS patients.

5.
Turk J Med Sci ; 48(3): 554-559, 2018 Jun 14.
Article in English | MEDLINE | ID: mdl-29914252

ABSTRACT

Background/aim: Abnormalities in oral mucosal immunity contribute to complex pathogenesis of primary Sjögren's syndrome (pSjS). We aimed to measure saliva and serum levels of caspase-1, tumor necrosis factor-alpha (TNF-α) and interferon gamma (IFN-γ) in patients with pSjS. Materials and methods: We studied 43 pSjS patients fulfilling the AECG criteria and 30 age/sex-matched healthy controls, as well as 39 rheumatoid arthritis (RA) patients as a disease control group. ESSDAI scores were less than seven in all patients with pSjS, indicating low disease activity. Quantitative analyses were made in serum and whole saliva samples. The statistical analysis was performed using SPSS 19.0. Results: While no significant difference was found in serum measurements, saliva levels of TNF-α and caspase-1 were significantly higher in pSjS patients versus healthy controls when using the Mann-Whitney U test. On the other hand, in the pSjS group, saliva levels of TNF-α and caspase-1 were also significantly higher compared to the RA group using Student's t-test. In the pSjS group, those parameters did not show any correlation with disease duration, seropositivity, and smoking. Conclusion: Despite low disease activity, saliva TNF-α and caspase-1 levels were found to be significantly higher in the pSjS group, which may suggest a possible advantage of local anticytokine treatments in selected cases.

6.
J Periodontol ; 89(8): 959-965, 2018 08.
Article in English | MEDLINE | ID: mdl-29752727

ABSTRACT

BACKGROUND: The aim of the present study is to compare the clinical periodontal findings as well as gingival crevicular fluid (GCF) and plasma levels of tumor necrosis factor-alpha (TNF-α), interleukin-1beta (IL-1ß), interferon gamma (IFN-γ) and caspase-1 in primary Sjögren syndrome (pSS) and rheumatoid arthritis (RA) subjects. METHODS: In the present case control study plasma and GCF samples were collected, full-mouth recordings comprising plaque index (PI), bleeding on probing (BOP) and probing depth (PD) were performed in 44 subjects with pSS, 39 subjects with RA and 30 systemically healthy subjects. Plasma and GCF TNF-α, IL-1ß, IFN-gamma and caspase-1 levels were determined by enzyme-linked immunosorbent assay. RESULTS: There were no differences in GCF and plasma levels of IFN-γ and TNF-α in all the study groups (p > 0.05). GCF levels of IL-1ß were higher in pSS group than healthy group (p = 0.035). Caspase-1 GCF levels were significantly higher in pSS group than RA group (p = 0.032). Highest plasma IL-1ß levels were detected in pSS compared to RA and healthy groups (p < 0.001). Healthy group has higher caspase-1 plasma levels than pSS and RA groups (p < 0.001). CONCLUSIONS: The results of the present study reveal that the periodontal status of patients with pSS does not differ from systemically healthy subjects. Further studies involving longitudinal assessments on larger populations with standardized patient inclusion criteria are needed to confirm the findings.


Subject(s)
Arthritis, Rheumatoid , Sjogren's Syndrome , Case-Control Studies , Cytokines , Gingival Crevicular Fluid , Humans , Tumor Necrosis Factor-alpha
7.
Rheumatol Int ; 37(5): 675-684, 2017 May.
Article in English | MEDLINE | ID: mdl-28289872

ABSTRACT

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.


Subject(s)
Sjogren's Syndrome/diagnosis , Symptom Assessment , Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Rheumatology , Young Adult
8.
Eur J Rheumatol ; 3(3): 136-138, 2016 Sep.
Article in English | MEDLINE | ID: mdl-27733945

ABSTRACT

Segmental arterial mediolysis (SAM) is a rare, nonarteriosclerotic, noninflammatory vascular disease and mostly affects medium-to-large sized abdominal arteries as well as presents with hemorrhages in the abdominal cavity. We report the case of a patient with SAM of the celiac, right renal, jejunal branch of the superior mesenteric, left gastric, and splenic arteries who was diagnosed by excluding other causes and in whom transcatheter embolization was performed in two different sessions, but he died because of an undefined reason. SAM mimics systemic vasculitis and causes abdominal pain; it should be considered because abdominal hemorrhage or arterial infarction can result in death.

9.
Turk Patoloji Derg ; 32(2): 65-9, 2016.
Article in English | MEDLINE | ID: mdl-27136104

ABSTRACT

OBJECTIVE: Minor salivary gland biopsy is one of the objective tests used in the diagnosis of Sjögren syndrome. The aim of our study was to compare the clinical and laboratory data of primary and secondary Sjögren syndrome cases with a lymphocyte score 3 and 4 in the minor salivary gland biopsy. MATERIAL AND METHOD: Data from a total of 2346 consecutive minor salivary gland biopsies were retrospectively evaluated in this study. Clinical and autoantibody characteristics of 367 cases with lymphocyte score 3 or 4 and diagnosed with primary or secondary Sjögren syndrome were compared. RESULTS: There was no difference between lymphocyte score 3 and 4 primary Sjögren syndrome patients in terms of dry mouth, dry eye symptoms and Schirmer test results but Anti-Ro and Antinuclear Antibody positivity was statistically significantly higher in cases with lymphocyte score 4 (p= 0.025, p= 0.001). Anti-Ro test results were also found to be statistically significantly higher in secondary Sjögren syndrome patients with lymphocyte score 4 (p= 0.048). CONCLUSION: In this study, the high proportion of cases with negative autoantibody but positive lymphocyte score is significant in terms of showing the contribution of minor salivary gland biopsy to Sjögren syndrome diagnosis. Lymphocyte score 3 and 4 cases were found to have similar clinical findings but a difference regarding antibody positivity in primary Sjögren syndrome. We believe that cases with lymphocyte score 4 may be Sjögren syndrome cases whose clinical manifestations are relatively established and higher autoantibody levels are therefore found.


Subject(s)
Autoantibodies/blood , Salivary Glands, Minor/pathology , Sjogren's Syndrome/blood , Sjogren's Syndrome/diagnosis , Sjogren's Syndrome/immunology , Adult , Aged , Biopsy , Female , Humans , Male , Middle Aged , Retrospective Studies
10.
Intern Med ; 55(5): 455-9, 2016.
Article in English | MEDLINE | ID: mdl-26935363

ABSTRACT

OBJECTIVE: Primary Sjögren's syndrome (pSS) is a common chronic autoimmune disease that primarily affects the salivary and lacrimal glands. Arterial stiffness is one of the earliest detectable manifestations of adverse structural and functional changes within the vessel wall. The aim of this study was to evaluate the relationship between arterial stiffness and pSS. METHODS: In this study, 75 female patients with pSS who fulfilled the American European Consensus Criteria for Sjögren's syndrome, were included. A total of 68 age-, sex- and body mass index-matched subjects were recruited as the control population. Arterial stiffness was assessed by measurement of the carotid-femoral pulse wave velocity (PWV). RESULTS: The mean age of the patients was 54.0±9.3 years and the median duration of the disease was 10 years. Compared with the control subjects, patients with pSS had a higher mean PWV (8.2±1.5 m/s vs. 7.5±1.4 m/s; p=0.01). Correlation analysis showed that the PWV was positively correlated with age, body mass index, serum cholesterol, low-density lipoprotein (LDL) and C-reactive protein levels, blood pressure, mean arterial pressure (MAP), pulse pressure and left ventricular mass index. A multiple linear regression analysis revealed that arterial stiffness was associated with age, MAP and LDL levels in pSS patients. CONCLUSION: Although patients with pSS appear to have increased arterial stiffness, risk factors associated with arterial stiffness in these patients are similar to the general population. However, we cannot exclude the possibility that a higher PWV in pSS patients is caused, not by pSS itself, but by the use of steroids, hypertension and dyslipidemia.


Subject(s)
Atherosclerosis/physiopathology , Endothelium, Vascular/physiopathology , Hypertension/physiopathology , Sjogren's Syndrome/physiopathology , Vascular Stiffness , Adult , Aged , Atherosclerosis/diagnostic imaging , Atherosclerosis/epidemiology , Blood Pressure , Blood Pressure Determination , Body Mass Index , Carotid Intima-Media Thickness , Echocardiography , Female , Humans , Hypertension/epidemiology , Middle Aged , Pulse Wave Analysis , Risk Factors , Sjogren's Syndrome/diagnostic imaging , Sjogren's Syndrome/epidemiology
11.
Rheumatol Int ; 35(9): 1473-8, 2015 Sep.
Article in English | MEDLINE | ID: mdl-25749712

ABSTRACT

This study assessed quality of life, direct and indirect healthcare costs related to ankylosing spondylitis (AS). This study included 650 prevalent AS patients visiting seven centers at tertiary healthcare institutions in Turkey who were interviewed using a standard questionnaire to determine annual direct and indirect healthcare costs. Eligible patients were age ≥18 years with AS for at least 12 months. Direct costs were categorized as inpatient, outpatient and pharmacy, and AS-related consultation. Indirect costs were categorized as workday loss, additional AS-related costs, and caregiver costs. Clinical outcome measures were obtained, including Patients' Global Disease Activity (Pt-GDA); visual analog scale (Pain-VAS) for pain; Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Functional Index (BASFI), and Metrology Index (BASMI) scores, and EuroQoL 5 dimension (EQ-5D) health status survey scores. Mean (€,335.20) and median (€5,671.00) annual costs per patient were calculated. Pharmacy costs (€4,032.73) were highest among overall expenditures, followed by additional AS-related consultation (€2,480.38), outpatient (€225.02), and inpatient costs (€29.98). Over half of AS patients (54.8 %) experienced work loss. Related average annual costs were €414.16, based on income level. 10.3 % of AS patients incurred an additional €2,008.07 in 1 year. 6.8 % of patients required caregivers and incurred €778.70 in average annual patient paid costs. Mean Pt-GDA, Pain-VAS, EQ-5D, BASDAI, BASFI, and BASMI scores were 4.4, 40.5, 62.7, 3.6, 3.1, and 2.9, respectively. Direct and indirect AS-related costs are high and represent a considerable economic burden on Turkish AS patients.


Subject(s)
Health Care Costs , Quality of Life , Spondylitis, Ankylosing/economics , Adult , Aged , Female , Humans , Male , Middle Aged , Severity of Illness Index , Spondylitis, Ankylosing/diagnosis , Spondylitis, Ankylosing/physiopathology , Surveys and Questionnaires , Turkey
12.
Spec Care Dentist ; 35(1): 1-7, 2015.
Article in English | MEDLINE | ID: mdl-24835826

ABSTRACT

OBJECTIVE: The purpose of this single blind study was to investigate effects of Buccotherm® spray on subjective symptoms of xerostomia patients. METHODS: Twenty patients with dry mouth complaint were instructed to use placebo six times a day for 2 weeks. After a wash period, mineral water spray was provided. Patients were asked to reply questions regarding dry mouth using visual analog scale (VAS). Baseline and subsequent subjective findings on 1 hour after the application of the materials at the end of 1st, 7th, and 14th days were recorded. RESULTS: At the end of 14-day treatment, no statistically significant differences were observed between the efficacy of placebo and commercial mineral water dental spray (p > 0.05). The VAS scores revealed that difficulty in mastication (p = 0.006), difficulty in swallowing (p = 0.00), need to sip liquids while eating (p = 0.000), difficulty in speech (p = 0.003), and waking up at night to sip water (p = 0.005) were statistically lower for placebo than commercial mineral water spray. CONCLUSION: The commercial mineral water dental spray was not more efficient than placebo in the management of dry mouth-related symptoms. CLINICAL RELEVANCE: This study emphasizes the fundamental role of saliva in oral health and evaluates the clinical utility of a commercial dental spray.


Subject(s)
Saliva, Artificial/therapeutic use , Xerostomia/drug therapy , Adult , Aged , Female , Humans , Male , Middle Aged , Placebos , Single-Blind Method , Surveys and Questionnaires , Treatment Outcome , Water
13.
Case Rep Rheumatol ; 2014: 549641, 2014.
Article in English | MEDLINE | ID: mdl-24551471

ABSTRACT

Temporal arteritis is most common vasculitis in elderly and imitated by miscellaneous disorders. Temporal artery biopsy is the gold standard test in the diagnosis of giant cell arteritis (GCA). Hereby, we describe a case of a 67-year-old man who presented initially with temporal arteritis; however, a lip biopsy then revealed AL amyloidosis. In this respect, temporal artery biopsy should be performed for definitive diagnosis of GCA particularly patients with systemic symptoms and treatment resistant.

14.
Clin Rheumatol ; 33(7): 955-61, 2014 Jul.
Article in English | MEDLINE | ID: mdl-24384825

ABSTRACT

Although autoimmune thyroid disease is well known to be associated with primary Sjögren's syndrome (SjS) and with various autoimmune diseases, it is less clear whether a similar association also exists for ankylosing spondylitis (AS). Therefore, we investigated the frequency of autoimmune thyroid disease in patients with AS. In this cross sectional study, 80 patients with AS fulfilling the 1984 Modified New York Criteria and 80 healthy subjects, age and sex-matched with AS patients, were included. As the positive control group, 62 female patients with primary SjS were also studied. All cases underwent thyroid ultrasonography (USG) by a single endocrinologist. Thyroid function tests and thyroid autoantibodies were measured. The diagnosis of Hashimoto's thyroiditis (HT) was made if the patient had thyroid autoantibody positivity plus at least one of the following criteria: diffuse goiter with physical examination, abnormality in thyroid function tests, and parenchymal heterogeneity with USG. The chi-squared test and Fisher's exact test were used to compare cases and controls. The p values <0.05 were considered statistically significant. The frequencies of parenchymal heterogeneity with USG (30 vs 11.3 %, p = 0.045), thyroid autoantibody positivity (13.8 vs 2.5 %, p = 0.017), and concomitant diagnosis of HT (10 vs 1.3 %, p = 0.034) were significantly higher in AS group compared to healthy controls. Among AS patients having HT, subclinical hypothyroidism was detected only in a single patient. Frequency of autoimmune thyroid disease was significantly higher in AS group, compared to healthy controls. Prospective studies are needed to see the clinical relevance of these findings and outcome in the long term.


Subject(s)
Autoimmune Diseases/complications , Spondylitis, Ankylosing/complications , Thyroid Diseases/complications , Adolescent , Adult , Case-Control Studies , Comorbidity , Cross-Sectional Studies , Female , Hashimoto Disease/immunology , Humans , Male , Middle Aged , Thyroid Function Tests , Thyroid Gland/diagnostic imaging , Ultrasonography , Young Adult
15.
Int J Rheum Dis ; 16(1): 88-92, 2013 Feb.
Article in English | MEDLINE | ID: mdl-23441777

ABSTRACT

OBJECTIVE: The objectives are to detect the frequency of sicca symptoms and Sjögren's syndrome (SS) in patients with systemic sclerosis (SSc) based on the diagnostic criteria of the American-European Consensus Group (AECG) and to evaluate demographic, clinical and serologic characteristics. PATIENTS AND METHOD: One hundred and eighteen SSc patients referred to our hospital were included in this study. All SSc patients were questioned with respect to sicca symptoms. Levels of rheumatoid factor (RF), anti-nuclear antibodies (ANA), anti-Ro and anti-La antibodies were measured; non-stimulated saliva amounts were recorded and Schirmer test and break-up time were applied to all patients. Minor salivary gland biopsy samples were obtained from those patients giving ≥ 3 positive answers to sicca symptom questions, patients with positive xerostomia/xerophthalmia test results, and patients with at least one antibody being positive. Patients presenting with grade 3 and/or grade 4 sialoadenitis based on Chisholm criteria were considered pathological. RESULTS: Sicca symptoms were present in 84 of 118 patients with SSc (71.2%). Minor salivary gland biopsy samples were obtained from 74 patients. Grade 3 and/or grade 4 sialoadenitis was detected in 40 (33.9%) patients and they were diagnosed with SS. Compared to patients diagnosed with SSc alone, systemic sclerosis patients diagnosed with SS had lower pulmonary hypertension and less diffuse lung involvement. Statistically significant difference was detected in terms of sclerodactylia and telangiectasia between SSc-SS and SSc patient groups (P = 0.045 and P = 0.011, respectively). Serological assessments revealed that in the SSc-SS group, 13 patients were anti-Ro antibody positive, six were anti-La antibody positive and 37 were anti-topoisomerase 1 antibody positive. RF, ANA and anti-centromere antibody levels were higher in the SSc-SS group. CONCLUSION: In the present study, highly frequent sicca symptoms and Sjögren's syndrome based on AECG criteria were noted in patients with systemic sclerosis. The SSc-SS patient group had less severe clinical course and lung involvement.


Subject(s)
Scleroderma, Systemic/epidemiology , Sjogren's Syndrome/epidemiology , Xerophthalmia/epidemiology , Xerostomia/epidemiology , Comorbidity , Female , Humans , Incidence , Male , Middle Aged , Salivary Glands/metabolism , Salivary Glands/pathology , Salivary Glands/physiopathology , Scleroderma, Systemic/pathology , Scleroderma, Systemic/physiopathology , Sjogren's Syndrome/pathology , Sjogren's Syndrome/physiopathology , Xerophthalmia/diagnosis , Xerophthalmia/physiopathology , Xerostomia/diagnosis , Xerostomia/physiopathology
16.
Genet Test Mol Biomarkers ; 15(7-8): 475-82, 2011.
Article in English | MEDLINE | ID: mdl-21413889

ABSTRACT

Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disorder characterized by episodes of inflammation in the absence of high-titer autoantibodies or antigen-specific T cells. The Mediterranean fever (MEFV) gene located on chromosome 16p13.3, which encodes the 781-amino-acid protein pyrin, is the causative gene for this monogenic Mendelian disease. This study presents the molecular analysis of an MEFV gene mutation screen of 5518 Turkish individuals with clinical diagnoses of FMF. Patients were genetically diagnosed using the FMF StripAssay and DNA sequencing analysis. Contrary to the results achieved by the FMF StripAssay, DNA sequencing analysis identified large-scale coding and noncoding novel sequence variants, together with a significant group (76%) of individuals who were receiving colchicine and had a single heterozygous mutation, despite the recessive inheritance of FMF. In conclusion, sequence analysis, unlike other routine laboratory techniques, may enable screening for a broad range of nucleotide variations and may prevent less common, population-restricted, novel sequence variants from being overlooked.


Subject(s)
Cytoskeletal Proteins/genetics , Familial Mediterranean Fever/genetics , Genetic Testing/methods , Heterozygote , Mutation , Familial Mediterranean Fever/diagnosis , Female , Gene Frequency , Genotype , Humans , Male , Nucleic Acid Hybridization/methods , Pyrin , Sequence Analysis, DNA/methods , Turkey
17.
Rheumatol Int ; 31(12): 1567-70, 2011 Dec.
Article in English | MEDLINE | ID: mdl-20490802

ABSTRACT

To evaluate the efficacy of a thermo-sensitive punctum plug, (SmartPlug™) in Primary Sjogren's Syndrome (pSS) patients with dry eyes, whose symptoms persist despite preservative-free artificial tear treatment. In this study, 22 Primary Sjögren's Syndrome (pSS), as defined by American-European Consensus Group Classification Criteria. All patients being followed up by Ege University Departments of Rheumatology and Ophthalmology. The patients had positive Schirmer test results (<5 mm without anesthesia). SmartPlug™ (Medennium, Irvine, California, USA) was inserted into the inferior lacrimal canaliculi of both eyes. Visual acuity measurements, Schirmer I test measurements, lissamine green staining scores, and tear-film breakup times (BUT) were noted before plug insertion and at the 1st, 6th, and 12th months following the procedure. Minimum follow-up period was 6 months for 19 patients and 12 months for 16 patients. Significant improvements were seen in the Schirmer I test scores (before insertion: 1.98 ± 2.67; 1st month: 5.68 ± 6.69; 6th month: 5.35 ± 5.38; 12th month 6.43 ± 5.14 P = 0.006), tear-film BUT in seconds (before insertion: 4.64 ± 3.7; 1st month: 5.80 ± 2.36; 6th month: 7.53 ± 2.92; 12th month 7.50 ± 2.52, P < 0.0001), respectively. Thermodynamic punctum plug insertion only in inferior canaliculus is a simple, effective, and comfortable option for treatment of severe aqueous tear deficiency that cannot be controlled using preservative-free tears.


Subject(s)
Keratoconjunctivitis Sicca/therapy , Prostheses and Implants , Sjogren's Syndrome/therapy , Adult , Female , Follow-Up Studies , Humans , Lacrimal Apparatus , Male , Middle Aged , Thermodynamics , Treatment Outcome
18.
Article in English | MEDLINE | ID: mdl-20610299

ABSTRACT

OBJECTIVE: The objective of this study was to evaluate whether hydroxychloroquine (HCQ) therapy effects subjective and/or objective complaints and salivary flow rates of patients with primary Sjögren's syndrome (PSS). STUDY DESIGN: Thirty women recently diagnosed with PSS, scheduled for HCQ treatment (400 mg daily), participated and were clinically examined before initiation of 30 weeks of HCQ treatment. During baseline evaluation, both the objective and/or subjective oral findings were recorded. Unstimulated (uSFR) and stimulated salivary flow rates (sSFR) were determined. After initiation of HCQ treatment, study parameters were assessed at 6, 12, 18, 24, and 30 weeks. Each patient served as her own control; measurements of the baseline and control times were analyzed by ANOVA. RESULTS: uSFR values increased significantly with HCQ treatment, but sSFR values, objective and/or subjective complaints did not change considerably. CONCLUSION: A positive impact of 30 weeks of HCQ treatment only on uSFRs of SS patients was revealed.


Subject(s)
Enzyme Inhibitors/therapeutic use , Hydroxychloroquine/therapeutic use , Saliva/drug effects , Sjogren's Syndrome/drug therapy , Adult , Aged , Burning Mouth Syndrome/drug therapy , Burning Mouth Syndrome/psychology , Cheilitis/drug therapy , Cheilitis/psychology , Cholinesterase Inhibitors/therapeutic use , Deglutition/physiology , Female , Follow-Up Studies , Humans , Mastication/physiology , Middle Aged , Patient Satisfaction , Saliva/metabolism , Secretory Rate/drug effects , Sjogren's Syndrome/psychology , Treatment Outcome , Xerostomia/drug therapy , Xerostomia/psychology
19.
Mod Rheumatol ; 20(6): 588-97, 2010 Dec.
Article in English | MEDLINE | ID: mdl-20585824

ABSTRACT

The aim of this study was to evaluate health-related quality of life (HR-QOL) in patients with primary Sjögren's syndrome (pSS) using both Short-Form 36 (SF-36) and World Health Organization Quality of Life Assessment-BREF (WHOQOL-BREF) questionnaires and to determine the effects of anxiety and depression on HR-QOL using the Hospital Anxiety-Depression Scale (HADS). In this cross-sectional study, 107 female patients with pSS (mean age 54.10 ± 10.2 years), fulfilling US-European Consensus Criteria and 109 female controls (mean age 53.4 ± 10.9 years) were included. Student's t test, Mann-Whitney U test, and analysis of variance (ANOVA) were used for statistical analysis. P values > 0.05 were accepted as significant. All domains of the SF-36, with the exception of "Vitality", and all domains of the WHOQOL-BREF with the exception of "Environment", were significantly lower in pSS patients compared with healthy controls. In pSS patients having anxiety according to HADS, the scores of all domains of WHOQOL-BREF were significantly lower, and in patients having depression according to HADS, three of four domains of WHOQOL-BREF were significantly lower compared with the rest of the group. However, the scores of two domains of the SF-36, namely "Role-Physical" and "Role-Emotional" domains, were significantly higher in pSS patients having depression according to HADS. We confirmed the presence of impaired HR-QOL in pSS. Whereas the presence of anxiety and/or depression generally showed a negative affect on HR-QOL, interestingly, depression seemed to improve the scores of "Role-Physical" and "Role-Emotional" domains of the SF-36. This surprising finding might be related to adaptation to changing health. Social support based upon cultural traditions might also have contributed.


Subject(s)
Anxiety/psychology , Depression/psychology , Quality of Life , Sjogren's Syndrome/psychology , Activities of Daily Living , Anxiety/epidemiology , Anxiety/physiopathology , Comorbidity , Depression/epidemiology , Depression/etiology , Depression/physiopathology , Female , Health Status , Humans , Middle Aged , Severity of Illness Index , Sjogren's Syndrome/epidemiology , Sjogren's Syndrome/physiopathology , Surveys and Questionnaires , Turkey/epidemiology
20.
Rheumatol Int ; 29(12): 1523-7, 2009 Oct.
Article in English | MEDLINE | ID: mdl-19593568

ABSTRACT

Sacroiliitis is an important sign of spondylarthritis (SpA) of which the prototype disease is ankylosing spondylitis. The radiographic changes required for diagnosing AS occur as late as 8-11 years after the onset of clinical symptoms. Nonsteroid anti-inflammatory drugs (NSAIDs) have been the main treatment for spondylitis of AS. For patients refractory or intolerant to NSAIDs, disease-modifying antirheumatic drugs (DMARDs) have been used as a second-line approach. Sulphasalazine (SLZ) is known as the best DMARD in treatment of peripheral arthritis; also methotrexate (MTX) is currently one of the most widely used DMARDs. But there was no objective information about inflammation of sacroiliac joints during treatment with these DMARDS that are the first places of the beginning point of SpA. For this purpose, in this study, the effect of SLZ and MTX, which are used alone and combination in 6 months, on treatment of active sacroiliitis, which is shown by dynamic magnetic resonance and acute phase reactants in laboratory has been investigated. 55 patients (F:M = 34:21) with active sacroiliitis [mean age = 37.05 + 13.03 year (n = 55)] were evaluated and determined by dynamic magnetic resonance imaging in this study. The better response in the SLZ treatment group than the other two groups has been obtained. Nevertheless, those changes were not statistically found different. In conclusion, the ratio of treatment of active sacroiliitis, especially early period, with SLZ as a DMARD is better than MTX or MTX + SLZ, but this difference is not statistically significant. A prospective study of the treatment of active sacroiliitis by DMARDs may be more illustrative.


Subject(s)
Antirheumatic Agents/therapeutic use , Methotrexate/therapeutic use , Sacroiliac Joint , Spondylarthritis/drug therapy , Spondylitis, Ankylosing/drug therapy , Sulfasalazine/therapeutic use , Adult , Drug Therapy, Combination , Female , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Retrospective Studies , Spondylarthritis/etiology , Spondylitis, Ankylosing/complications , Treatment Outcome
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