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1.
Int J Rheum Dis ; 27(1): e14984, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38014450

RESUMO

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.


Assuntos
Amiloidose , Tumor Glômico , Amiloidose de Cadeia Leve de Imunoglobulina , Paraganglioma , Feminino , Humanos , Idoso de 80 Anos ou mais , Proteína Antagonista do Receptor de Interleucina 1/uso terapêutico , Tumor Glômico/complicações , Amiloidose/complicações , Amiloidose/diagnóstico , Amiloidose/tratamento farmacológico , Amiloidose de Cadeia Leve de Imunoglobulina/complicações , Paraganglioma/complicações , Proteína Amiloide A Sérica
2.
Int J Rheum Dis ; 26(12): 2563-2566, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37294068

RESUMO

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.


Assuntos
Amiloidose , Pneumopatias , Síndrome de Sjogren , Feminino , Humanos , Idoso , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/tratamento farmacológico , Rituximab/uso terapêutico , Pneumopatias/complicações , Pneumopatias/diagnóstico , Pneumopatias/tratamento farmacológico , Pulmão/diagnóstico por imagem , Pulmão/patologia , Amiloidose/complicações , Amiloidose/diagnóstico , Amiloidose/tratamento farmacológico
3.
Turk J Phys Med Rehabil ; 66(3): 299-306, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-33089086

RESUMO

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.

4.
Turk J Med Sci ; 48(3): 554-559, 2018 Jun 14.
Artigo em Inglês | MEDLINE | ID: mdl-29914252

RESUMO

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.

5.
J Periodontol ; 89(8): 959-965, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-29752727

RESUMO

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.


Assuntos
Artrite Reumatoide , Síndrome de Sjogren , Estudos de Casos e Controles , Citocinas , Líquido do Sulco Gengival , Humanos , Fator de Necrose Tumoral alfa
6.
Rheumatol Int ; 37(5): 675-684, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28289872

RESUMO

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.


Assuntos
Síndrome de Sjogren/diagnóstico , Avaliação de Sintomas , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Reumatologia , Adulto Jovem
7.
Eur J Rheumatol ; 3(3): 136-138, 2016 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-27733945

RESUMO

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.

8.
Turk Patoloji Derg ; 32(2): 65-9, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27136104

RESUMO

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.


Assuntos
Autoanticorpos/sangue , Glândulas Salivares Menores/patologia , Síndrome de Sjogren/sangue , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/imunologia , Adulto , Idoso , Biópsia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
9.
Intern Med ; 55(5): 455-9, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-26935363

RESUMO

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.


Assuntos
Aterosclerose/fisiopatologia , Endotélio Vascular/fisiopatologia , Hipertensão/fisiopatologia , Síndrome de Sjogren/fisiopatologia , Rigidez Vascular , Adulto , Idoso , Aterosclerose/diagnóstico por imagem , Aterosclerose/epidemiologia , Pressão Sanguínea , Determinação da Pressão Arterial , Índice de Massa Corporal , Espessura Intima-Media Carotídea , Ecocardiografia , Feminino , Humanos , Hipertensão/epidemiologia , Pessoa de Meia-Idade , Análise de Onda de Pulso , Fatores de Risco , Síndrome de Sjogren/diagnóstico por imagem , Síndrome de Sjogren/epidemiologia
10.
Rheumatol Int ; 35(9): 1473-8, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-25749712

RESUMO

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.


Assuntos
Custos de Cuidados de Saúde , Qualidade de Vida , Espondilite Anquilosante/economia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Índice de Gravidade de Doença , Espondilite Anquilosante/diagnóstico , Espondilite Anquilosante/fisiopatologia , Inquéritos e Questionários , Turquia
11.
Spec Care Dentist ; 35(1): 1-7, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-24835826

RESUMO

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.


Assuntos
Saliva Artificial/uso terapêutico , Xerostomia/tratamento farmacológico , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Placebos , Método Simples-Cego , Inquéritos e Questionários , Resultado do Tratamento , Água
12.
Case Rep Rheumatol ; 2014: 549641, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-24551471

RESUMO

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.

13.
Clin Rheumatol ; 33(7): 955-61, 2014 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-24384825

RESUMO

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.


Assuntos
Doenças Autoimunes/complicações , Espondilite Anquilosante/complicações , Doenças da Glândula Tireoide/complicações , Adolescente , Adulto , Estudos de Casos e Controles , Comorbidade , Estudos Transversais , Feminino , Doença de Hashimoto/imunologia , Humanos , Masculino , Pessoa de Meia-Idade , Testes de Função Tireóidea , Glândula Tireoide/diagnóstico por imagem , Ultrassonografia , Adulto Jovem
14.
Int J Rheum Dis ; 16(1): 88-92, 2013 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-23441777

RESUMO

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.


Assuntos
Escleroderma Sistêmico/epidemiologia , Síndrome de Sjogren/epidemiologia , Xeroftalmia/epidemiologia , Xerostomia/epidemiologia , Comorbidade , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Glândulas Salivares/metabolismo , Glândulas Salivares/patologia , Glândulas Salivares/fisiopatologia , Escleroderma Sistêmico/patologia , Escleroderma Sistêmico/fisiopatologia , Síndrome de Sjogren/patologia , Síndrome de Sjogren/fisiopatologia , Xeroftalmia/diagnóstico , Xeroftalmia/fisiopatologia , Xerostomia/diagnóstico , Xerostomia/fisiopatologia
15.
Genet Test Mol Biomarkers ; 15(7-8): 475-82, 2011.
Artigo em Inglês | MEDLINE | ID: mdl-21413889

RESUMO

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.


Assuntos
Proteínas do Citoesqueleto/genética , Febre Familiar do Mediterrâneo/genética , Testes Genéticos/métodos , Heterozigoto , Mutação , Febre Familiar do Mediterrâneo/diagnóstico , Feminino , Frequência do Gene , Genótipo , Humanos , Masculino , Hibridização de Ácido Nucleico/métodos , Pirina , Análise de Sequência de DNA/métodos , Turquia
16.
Rheumatol Int ; 31(12): 1567-70, 2011 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-20490802

RESUMO

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.


Assuntos
Ceratoconjuntivite Seca/terapia , Próteses e Implantes , Síndrome de Sjogren/terapia , Adulto , Feminino , Seguimentos , Humanos , Aparelho Lacrimal , Masculino , Pessoa de Meia-Idade , Termodinâmica , Resultado do Tratamento
17.
Artigo em Inglês | MEDLINE | ID: mdl-20610299

RESUMO

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.


Assuntos
Inibidores Enzimáticos/uso terapêutico , Hidroxicloroquina/uso terapêutico , Saliva/efeitos dos fármacos , Síndrome de Sjogren/tratamento farmacológico , Adulto , Idoso , Síndrome da Ardência Bucal/tratamento farmacológico , Síndrome da Ardência Bucal/psicologia , Queilite/tratamento farmacológico , Queilite/psicologia , Inibidores da Colinesterase/uso terapêutico , Deglutição/fisiologia , Feminino , Seguimentos , Humanos , Mastigação/fisiologia , Pessoa de Meia-Idade , Satisfação do Paciente , Saliva/metabolismo , Taxa Secretória/efeitos dos fármacos , Síndrome de Sjogren/psicologia , Resultado do Tratamento , Xerostomia/tratamento farmacológico , Xerostomia/psicologia
18.
Mod Rheumatol ; 20(6): 588-97, 2010 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-20585824

RESUMO

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.


Assuntos
Ansiedade/psicologia , Depressão/psicologia , Qualidade de Vida , Síndrome de Sjogren/psicologia , Atividades Cotidianas , Ansiedade/epidemiologia , Ansiedade/fisiopatologia , Comorbidade , Depressão/epidemiologia , Depressão/etiologia , Depressão/fisiopatologia , Feminino , Nível de Saúde , Humanos , Pessoa de Meia-Idade , Índice de Gravidade de Doença , Síndrome de Sjogren/epidemiologia , Síndrome de Sjogren/fisiopatologia , Inquéritos e Questionários , Turquia/epidemiologia
19.
Rheumatol Int ; 29(12): 1523-7, 2009 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-19593568

RESUMO

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.


Assuntos
Antirreumáticos/uso terapêutico , Metotrexato/uso terapêutico , Articulação Sacroilíaca , Espondilartrite/tratamento farmacológico , Espondilite Anquilosante/tratamento farmacológico , Sulfassalazina/uso terapêutico , Adulto , Quimioterapia Combinada , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Espondilartrite/etiologia , Espondilite Anquilosante/complicações , Resultado do Tratamento
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