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1.
Clin Rheumatol ; 42(2): 385-390, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-36637636

RESUMO

BACKGROUND: This study aimed to assess the mortality of PsA before and during the COVID-19 pandemic. METHODS: From the prospective, multicenter PsART-ID (Psoriatic Arthritis Registry-International Database), patients from Turkey were analyzed by linking the registry to the Turkish Cause of Death Registry. The outcome of interest was death from any cause, pre-pandemic (since the onset of registry-March 2014-March 2020), and during the pandemic (March 2020-May 2021). The crude mortality rate and standardized mortality ratio (SMR) were determined. RESULTS: There were 1216 PsA patients with a follow-up of 7500 patient-years. Overall, 46 deaths (26 males) were observed. In the pre-pandemic period, SMR for PsA vs the general population was 0.95 (0.61-1.49), being higher in males [1.56 (0.92-2.63)] than females [0.62 (0.33-1.17)]. The crude mortality rate in PsA doubled during the pandemic (pre-pandemic crude mortality rate: 5.07 vs 10.76 during the pandemic) with a higher increase in females (2.9 vs 8.72) than males (9.07 vs 14.73). CONCLUSION: The mortality in PsA was found similar to the general population in the pre-pandemic era. The mortality rates in PsA doubled during the pandemic. Whether PsA patients have more risk of mortality than the general population due to COVID-19 needs further studies. Key Points • Decrease in mortality in PsA might be expected with the more effective treatment options and better disease control. • A crude mortality rate is comparable to the general population and not increased until the pandemic. • Currently, there is a 2-fold increase in crude mortality rate possibly due to the COVID-19.


Assuntos
Artrite Psoriásica , COVID-19 , Feminino , Humanos , Masculino , Artrite Psoriásica/mortalidade , COVID-19/epidemiologia , Pandemias , Estudos Prospectivos , Sistema de Registros , Turquia/epidemiologia
2.
Mod Rheumatol Case Rep ; 7(1): 271-275, 2023 01 03.
Artigo em Inglês | MEDLINE | ID: mdl-35538613

RESUMO

Castleman's disease (CD) is a rare, systemic disease with histopathological features of angiofollicular lymph node hyperplasia. In the literature, there are case-level reports that mimic or coexist with systemic lupus erythematosus (SLE) clinically and in the laboratory. Is this condition two separate diseases or is it an imitation of each other? A 73-year-old female patient was admitted to our clinic with arthritis, lymphadenopathy, fever, weight loss, and malar rash. He had a history of idiopathic thrombocytopenic purpura and thrombosis in the right leg tibialis posterior and dorsalis pedis arteries. Excisional lymphadenopathy biopsy indicated a diagnosis of hyaline-vascular-type CD. She had anti-nuclear antibody >1/80 homogeneous pattern, anti-double stranded DNA (anti-dsDNA), Anti-Smith (Sm) antibody positivity, hypocomplementemia (C3 and C4), pleural effusion, and pericardial effusion. For this reason, the classification criteria of the European League Against Rheumatism/American College of Rheumatology were studied. Clinical findings, idiopathic thrombocytopenic purpura history, antibody positivity, malar rash, and arthritis led us to the diagnosis of SLE. She was treated with 1 mg/kg/day prednisolone and hydroxychloroquine 200 mg 2 × 1. Azathioprine 2.5 mg/kg daily was added to the patient whose complaints did not improve. In the follow-ups, she completely recovered clinically and laboratory. SLE and CD are systemic diseases that overlap in many ways. The literature review shows that these two diseases may mimic each other or may coexist. This situation may be a reflection of a pathophysiological process that has not yet been clarified. This confusing process also affects the treatment decision. This confusing process also affects the treatment decision.


Assuntos
Artrite , Hiperplasia do Linfonodo Gigante , Lúpus Eritematoso Sistêmico , Linfadenopatia , Púrpura Trombocitopênica Idiopática , Masculino , Feminino , Humanos , Idoso , Hiperplasia do Linfonodo Gigante/complicações , Hiperplasia do Linfonodo Gigante/diagnóstico , Púrpura Trombocitopênica Idiopática/complicações , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Anticorpos Antinucleares , Linfadenopatia/complicações
3.
Mod Rheumatol Case Rep ; 7(1): 315-319, 2023 01 03.
Artigo em Inglês | MEDLINE | ID: mdl-36208300

RESUMO

Werner syndrome (WS), also known as adult progeria, is a premature ageing syndrome that can manifest itself with grey hair, hair loss, diabetes mellitus, hyperlipidaemia, hypertension, skin disorders, ocular cataracts, myocardial infarction, osteoporosis, and stroke, especially after puberty. Physical examination findings similar to systemic sclerosis may be seen. Therefore, it may mimic this disease as misleading. A 43-year-old female patient was admitted to our clinic with a pre-diagnosis of systemic sclerosis complaint of skin hardening up to the ankle. In the first physical examination, there were wrinkles and thinning of the lip, suggesting systemic sclerosis in the facial appearance. On her capillaroscopy, there was tortuosity and an old focus of microhemorrhage. She had a history of diabetes mellitus and chronic osteomyelitis. When all symptoms, clinical findings, and antibody results were combined, it was thought that the patient might have WS. WS was diagnosed with homozygous c.2221 C>P p.R741*(rs763089663) positive in genetic analysis. It is known that WS creates a predisposition to malignancies, and most patients die secondary to malignancies. Therefore, early diagnosis becomes essential. Early diagnosis is of vital importance both to prevent complications and to delay treatment. In particular, systemic sclerosis-like findings of this syndrome may cause delays in diagnosis. For this reason, small clues suggesting WS in the clinic should be well known and well defined.


Assuntos
Diabetes Mellitus , Osteoporose , Escleroderma Sistêmico , Síndrome de Werner , Adulto , Feminino , Humanos , Síndrome de Werner/complicações , Síndrome de Werner/diagnóstico , Síndrome de Werner/genética , Diabetes Mellitus/etiologia , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico , Pele/patologia , Osteoporose/complicações
4.
Eur J Rheumatol ; 7(2): 64-67, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31922480

RESUMO

OBJECTIVE: Our aim is to test the validity of the Psoriasis Symptom Inventory (PSI), a patient-reported outcome, to assess the psoriasis severity within the scope of rheumatology. METHODS: Within the PsA international database (PSART-ID), 571 patients had PSI, while 322 of these also showed body surface area (BSA). Correlations between PSI, BSA, and other patient- and physician-reported outcomes were investigated. RESULTS: There was a good correlation between PSI and BSA (r=0.546, p<0.001), which was even higher for mild psoriasis (BSA<3 (n=164): r=0.608, p<0.001). PSI significantly correlated with fatigue, pain, and patient and physician global parameters (p<0.001). CONCLUSION: PSI has a good correlation with other patient- and physician-reported outcomes, and our findings support its use in rheumatology practice.

5.
J Rheumatol ; 2019 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-31787608

RESUMO

OBJECTIVE: The effect of smoking in psoriatic arthritis (PsA) is under debate. Our aim was to test whether smoking is increased in axial PsA (axPsA). METHODS: Included in the analysis were 1535 patients from PsArt-ID (PsA-International Database). The effect of smoking on axPsA (compared to other PsA phenotypes) and radiographic sacroiliitis were investigated. RESULTS: Current smoking was more common in axPsA (28.6% vs 18.9%, p < 0.001). It also was found as an independent predictor of axPsA (OR 1.4) and radiographic sacroiliitis (OR 6.6). CONCLUSION: Current smoking is significantly associated with both axPsA and radiographic sacroiliitis in patients with PsA.

6.
Eur J Rheumatol ; 6(1): 34-37, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30489254

RESUMO

OBJECTIVE: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease. Most of the identified disease-causing mutations are located on exon 10. As the number of studies about the effect of the exonal location of the mutation and its phenotypic expression is limited, we aimed to investigate whether the exonic location of the Mediterranean fever (MEFV) mutation has an effect on the clinical manifestation in patients with FMF. METHODS: Study population was derived from the main FMF registry that included 2246 patients from 15 different rheumatology clinics. We categorized the mutations according to their exon locations and retrieved the clinical and demographic information from the database. RESULTS: Patients having the MEFV mutations on exon 2 or 10 (n:1526) were divided into three subgroups according to the location of the MEFV mutations: Group 1 (exon 2 mutations), Group 2 (exon 10 mutations), and Group 3 (both exon 2 and exon 10 mutations). Group 2 patients were of a significantly younger age at onset, and erysipel-like erythema, arthritis, amyloidosis, and a family history of FMF were more common in this group. CONCLUSION: Patients with FMF and exon 10 mutations show more severe clinical symptoms and outcome. Exon 2 mutations tend to have a better outcome.

7.
Rheumatology (Oxford) ; 56(2): 279-286, 2017 02.
Artigo em Inglês | MEDLINE | ID: mdl-27794533

RESUMO

OBJECTIVE: The aim was to assess the characteristics of PsA, find out how well the disease is controlled in real life, demonstrate the treatments and identify the unmet needs. METHODS: The PsA registry of Turkey is a multicentre Web-based registry established in 2014 and including 32 rheumatology centres. Detailed data regarding demographics for skin and joint disease, disease activity assessments and treatment choices were collected. RESULTS: One thousand and eighty-one patients (64.7% women) with a mean (sd) PsA duration of 5.8 (6.7) years were enrolled. The most frequent type of PsA was polyarticular [437 (40.5%)], followed by oligoarticular [407 (37.7%)] and axial disease [372 (34.4%)]. The mean (sd) swollen and tender joint counts were 1.7 (3) and 3.6 (4.8), respectively. Of these patients, 38.6% were on conventional synthetic DMARD monotherapy, 7.1% were on anti-TNF monotherapy, and 22.5% were using anti-TNF plus conventional synthetic DMARD combinations. According to DAS28, 86 (12.4%) patients had high and 105 (15.2%) had moderate disease activity. Low disease activity was achieved in 317 (45.7%) patients, and 185 (26.7%) were in remission. Minimal disease activity data could be calculated in 247 patients, 105 of whom (42.5%) had minimal disease activity. The major differences among sexes were that women were older and had less frequent axial disease, more fatigue, higher HAQ scores and less remission. CONCLUSION: The PsA registry of Turkey had similarities with previously published registries, supporting its external validity. The finding that women had more fatigue and worse functioning as well as the high percentage of active disease state highlight the unmet need in treatment of PsA.


Assuntos
Antirreumáticos/uso terapêutico , Artrite Psoriásica/tratamento farmacológico , Necessidades e Demandas de Serviços de Saúde , Sistema de Registros , Atividades Cotidianas , Adulto , Distribuição por Idade , Artrite Psoriásica/complicações , Artrite Psoriásica/epidemiologia , Artrite Psoriásica/fisiopatologia , Fadiga/etiologia , Fadiga/fisiopatologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Medidas de Resultados Relatados pelo Paciente , Indução de Remissão , Índice de Gravidade de Doença , Distribuição por Sexo , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Turquia/epidemiologia
8.
Rheumatol Int ; 36(7): 945-53, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-27221456

RESUMO

In this multicenter, retrospective study, we evaluated the efficacy and safety of biologic therapies, including anti-TNFs, in secondary (AA) amyloidosis patients with ankylosing spondylitis (AS) and rheumatoid arthritis (RA). In addition, the frequency of secondary amyloidosis in RA and AS patients in a single center was estimated. Fifty-one AS (39M, 12F, mean age: 46.7) and 30 RA patients (11M, 19F, mean age: 51.7) with AA amyloidosis from 16 different centers in Turkey were included. Clinical and demographical features of patients were obtained from medical charts. A composite response index (CRI) to biologic therapy-based on creatinine level, proteinuria and disease activity-was used to evaluate the efficacy of treatment. The mean annual incidence of AA amyloidosis in RA and AS patients was 0.23 and 0.42/1000 patients/year, respectively. The point prevalence in RA and AS groups was 4.59 and 7.58/1000, respectively. In RA group with AA amyloidosis, effective response was obtained in 52.2 % of patients according to CRI. RA patients with RF positivity and more initial disease activity tended to have higher response rates to therapy (p values, 0.069 and 0.056). After biologic therapy (median 17 months), two RA patients died and two developed tuberculosis. In AS group, 45.7 % of patients fulfilled the criteria of good response according to CRI. AS patients with higher CRP levels at the time of AA diagnosis and at the beginning of anti-TNF therapy had higher response rates (p values, 0.011 and 0.017). During follow-up after anti-TNF therapy (median 38 months), one patient died and tuberculosis developed in two patients. Biologic therapy seems to be effective in at least half of RA and AS patients with AA amyloidosis. Tuberculosis was the most important safety concern.


Assuntos
Amiloidose/tratamento farmacológico , Artrite Reumatoide/tratamento farmacológico , Produtos Biológicos/uso terapêutico , Imunossupressores/uso terapêutico , Espondilite Anquilosante/tratamento farmacológico , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Adulto , Idoso , Amiloidose/diagnóstico , Amiloidose/epidemiologia , Amiloidose/imunologia , Artrite Reumatoide/diagnóstico , Artrite Reumatoide/epidemiologia , Artrite Reumatoide/imunologia , Produtos Biológicos/efeitos adversos , Progressão da Doença , Feminino , Humanos , Hospedeiro Imunocomprometido , Imunossupressores/efeitos adversos , Incidência , Masculino , Pessoa de Meia-Idade , Infecções Oportunistas/induzido quimicamente , Infecções Oportunistas/epidemiologia , Infecções Oportunistas/imunologia , Prevalência , Indução de Remissão , Estudos Retrospectivos , Fatores de Risco , Espondilite Anquilosante/diagnóstico , Espondilite Anquilosante/epidemiologia , Espondilite Anquilosante/imunologia , Fatores de Tempo , Resultado do Tratamento , Tuberculose/induzido quimicamente , Tuberculose/epidemiologia , Tuberculose/imunologia , Fator de Necrose Tumoral alfa/imunologia , Turquia/epidemiologia
9.
J Rheumatol ; 43(3): 524-9, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26773107

RESUMO

OBJECTIVE: Screening strategies for latent tuberculosis (TB) before starting tumor necrosis factor (TNF)-α inhibitors have decreased the prevalence of TB among patients who are treated with these agents. However, despite vigilant screening, TB continues to be an important problem, especially in parts of the world with a high background TB prevalence. The aim of this study was to determine the factors related to TB among a large multicenter cohort of patients who were treated with anti-TNF. METHODS: Fifteen rheumatology centers participated in this study. Among the 10,434 patients who were treated with anti-TNF between September 2002 and September 2012, 73 (0.69%) had developed TB. We described the demographic features and disease characteristics of these 73 patients and compared them to 7695 patients who were treated with anti-TNF, did not develop TB, and had complete data available. RESULTS: Among the 73 patients diagnosed with TB (39 men, 34 women, mean age 43.6 ± 13 yrs), the most frequent diagnoses were ankylosing spondylitis (n = 38) and rheumatoid arthritis (n = 25). More than half of the patients had extrapulmonary TB (39/73, 53%). Six patients died (8.2%). In the logistic regression model, types of anti-TNF drugs [infliximab (IFX), OR 3.4, 95% CI 1.88-6.10, p = 0.001] and insufficient and irregular isoniazid use (< 9 mos; OR 3.15, 95% CI 1.43-6.9, p = 0.004) were independent predictors of TB development. CONCLUSION: Our results suggest that TB is an important complication of anti-TNF therapies in Turkey. TB chemoprophylaxis less than 9 months and the use of IFX therapy were independent risk factors for TB development.


Assuntos
Antirreumáticos/efeitos adversos , Produtos Biológicos/efeitos adversos , Tuberculose Latente/diagnóstico , Tuberculose/epidemiologia , Tuberculose/etiologia , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Adulto , Antirreumáticos/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Produtos Biológicos/uso terapêutico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Risco , Espondilite Anquilosante/tratamento farmacológico
10.
J Rheumatol ; 42(9): 1661-6, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-26178288

RESUMO

OBJECTIVE: To assess bladder toxicity of cyclophosphamide (CYC) and uroprotective effect of mesna in rheumatic diseases. METHODS: Data of 1018 patients (725 women/293 men) treated with CYC were evaluated in this retrospective study. All of the following information was obtained: the cumulative CYC dose, route of CYC administration, duration of therapy, concomitant mesna usage, and hemorrhagic cystitis. Cox proportional hazard model was used for statistics. RESULTS: We identified 17 patients (1.67%) with hemorrhagic cystitis and 2 patients (0.19%) with bladder cancer in 4224 patient-years. The median time for diagnosis to hemorrhagic cystitis was 10 months (4-48) and bladder cancer was 8 years (6-10.9). There were 583 patients (57.2%) who received mesna with intravenous CYC therapy. We observed similar incidence rate for hemorrhagic cystitis in both patient groups concomitantly treated with or without mesna [9/583 (1.5%) vs 8/425 (1.8%) respectively, p = 0.08]. Cumulative CYC dose (HR for 10-g increments 1.24, p < 0.001) was associated with hemorrhagic cystitis. CONCLUSION: Cumulative dose was the only risk factor for hemorrhagic cystitis in patients treated with CYC. No proof was obtained for the uroprotective effect of mesna in our cohort.


Assuntos
Ciclofosfamida/efeitos adversos , Cistite/epidemiologia , Mesna/uso terapêutico , Substâncias Protetoras/uso terapêutico , Doenças Reumáticas/tratamento farmacológico , Adulto , Idoso , Ciclofosfamida/uso terapêutico , Cistite/induzido quimicamente , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
11.
Rev. bras. reumatol ; 54(5): 356-359, Sep-Oct/2014. tab
Artigo em Português | LILACS | ID: lil-725692

RESUMO

Introdução: Colchicina é a viga-mestra para o tratamento de FFM, que é uma doença autoinflamatória com polisserosite recidivante como principal manifestação. Apesar de doses diárias de 2 mg ou mais/dia, aproximadamente 5%-10% dos pacientes continuam a sofrer de seus ataques. Neste estudo, objetivamos investigar os aspectos da depressão e dos ataques em pacientes com FFM apresentando resistência à colchicina (RC). Pacientes e Métodos: Em pacientes com FFM, RC foi definida como dois ou mais ataques nos últimos seis meses, quando em medicação com colchicina 2 mg/dia. Dezoito pacientes (nove mulheres e nove homens) foram recrutados no grupo RC e 41 pacientes no grupo de controle (29 mulheres/12 homens). Foram avaliados os achados demográficos, clínicos e laboratoriais, a fidelidade ao tratamento e os escores do Beck Depression Inventory (BDI). Resultados: A idade de surgimento da FFM foi significativamente menor no grupo RC (12,3 anos vs. 16,9 anos, P = 0,03). A duração da doença foi maior no grupo RC (p = 0,01). Dores abdominais e nas pernas em decorrência do exercício foram significativamente mais frequentes no grupo RC versus controles (83% vs. 51%; p = 0,02 e 88% vs. 60%; p = 0,04, respectivamente). Pacientes com escores BDI > 17 pontos foram mais frequentes no grupo RC versus controles (50% vs. 34,1%; p < 0,001). Discussão: Verificamos que: (1) a idade do surgimento da doença foi mais baixa e (2) a duração da doença foi maior no grupo RC. Ataques pleuríticos, hematúria e proteinúria foram mais frequentes em pacientes com RC. Propomos que a depressão é fator importante a ser levado em consideração na sensibilidade à RC. .


Introduction: Colchicine is the mainstay for the treatment of FMF, which is an auto-inflammatory disease mainly with relapsing polyserositis. Despite daily doses of 2 mg or more each day, approximately 5% to 10% of the patients continue to suffer from its attacks. In this study, we aimed to investigate the depression and attack features in patients with FMF who have colchicine resistance (CR). Patients e Methods: CR was defined for FMF patients with 2 or more attacks within the last 6 months period while using 2 mg/day colchicine. Eighteen patients (9 Female/9 Male) were enrolled into the CR group and 41 patients were enrolled into the control group (12 Male/29 Female). Demographic, clinical e laboratory findings, treatment adherence, and the Beck Depression Inventory (BDI) scores were evaluated. Results: The age of onset of FMF was significantly lower in the CR group (12.3 yrs vs. 16.9 yrs, P = 0.03). Disease duration was longer in the CR group (P = 0.01). Abdominal and leg pain due to exercise were significantly more frequent in the CR group versus controls (83% vs. 51%; P = 0.02 e 88% vs. 60%; P = 0.04, respectively). Patients with BDI scores over 17 points were more frequent in the CR group compared to controls (50% vs. 34.1%; P < 0.001). Discussion: We found that: (1) the age of disease onset was lower and (2) the disease duration was longer in CR group. Pleuritic attacks, hematuria e proteinuria were more frequent in CR patients. We propose that depression is an important factor to consider in the susceptibility to CR. .


Assuntos
Humanos , Masculino , Feminino , Adulto , Febre Familiar do Mediterrâneo/tratamento farmacológico , Colchicina/uso terapêutico , Febre Familiar do Mediterrâneo/etiologia , Resistência a Medicamentos , Estudos Prospectivos , Depressão/complicações
12.
Mod Rheumatol ; 24(2): 327-30, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-24593208

RESUMO

OBJECTIVES: Ankylosing spondylitis (AS) is a chronic multi-systemic inflammatory rheumatic disorder. Several studies have suggested that the interval from the peak to the end of the electrocardiographic T wave (Tp-e) may correspond to the transmural dispersion of repolarization and that increased Tp-e interval and Tp-e/QT ratio are associated with malignant ventricular arrhythmias. The aim of this study was to evaluate ventricular repolarization by using Tp-e interval and Tp-e/QT ratio in patients with AS, and to assess the relation with inflammation. METHODS: Sixty-two patients with AS and 50 controls were included. Tp-e interval and Tp-e/QT ratio were measured from a 12-lead electrocardiogram, and the Tp-e interval corrected for heart rate. The plasma level of high sensitive C-reactive protein (hsCRP) was measured. These parameters were compared between groups. RESULTS: In electrocardiographic parameters analysis, QT dispersion (QTd) and corrected QTd were significantly increased in AS patients compared to the controls (31.7 ± 9.6 vs 28.2 ± 7.4 and 35.8 ± 11.5 vs 30.6 ± 7.9 ms, P = 0.03 and P = 0.007, respectively). cTp-e interval and Tp-e/QT ratio were also significantly higher in AS patients (92.1 ± 10.2 vs 75.8 ± 8.4 and 0.22 ± 0.02 vs 0.19 ± 0.02 ms, all P values <0.001). cTp-e interval and Tp-e/QT ratio were significantly correlated with hsCRP (r = 0.63, P < 0.001 and r = 0.49, P < 0.001, respectively). CONCLUSIONS: Our study revealed that Tp-e interval and Tp-e/QT ratio were increased in AS patients. These electrocardiographic ventricular repolarization indexes were significantly correlated with the plasma level of hsCRP.


Assuntos
Arritmias Cardíacas/fisiopatologia , Sistema de Condução Cardíaco/anormalidades , Sistema de Condução Cardíaco/fisiologia , Espondilite Anquilosante/fisiopatologia , Adulto , Arritmias Cardíacas/complicações , Síndrome de Brugada , Doença do Sistema de Condução Cardíaco , Eletrocardiografia , Feminino , Sistema de Condução Cardíaco/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Espondilite Anquilosante/complicações
14.
Rheumatology (Oxford) ; 53(4): 741-5, 2014 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24369413

RESUMO

OBJECTIVE: The primary aim of this study was to investigate the prevalence of amyloidosis and its related factors in a large number of FMF patients. METHODS: Fifteen centres from the different geographical regions of Turkey were included in the study. Detailed demographic and medical data based on a structured questionnaire and medical records were collected. The diagnosis of amyloidosis was based on histological proof of congophilic fibrillar deposits in tissue biopsy specimens. RESULTS: There were 2246 FMF patients. The male/female ratio was 0.87 (1049/1197). The mean age of the patients was 34.5 years (S.D. 11.9). Peritonitis was the most frequent clinical finding and it was present in 94.6% of patients. Genetic testing was available in 1719 patients (76.5%). The most frequently observed genotype was homozygous M694V mutation, which was present in 413 (24%) patients. Amyloidosis was present in 193 patients (8.6%). Male sex, arthritis, delay in diagnosis, M694V genotype, patients with end-stage renal disease (ESRD) and family history of amyloidosis and ESRD were significantly more prevalent in patients with amyloidosis compared with the amyloidosis-negative subjects. Patients with homozygous M694V mutations had a 6-fold higher risk of amyloidosis compared with the other genotypes (95% CI 4.29, 8.7, P < 0.001). CONCLUSION: In this nationwide study we found that 8.6% of our FMF patients had amyloidosis and homozygosity for M694V was the most common mutation in these patients. The latter finding confirms the association of homozygous M694V mutation with amyloidosis in Turkish FMF patients.


Assuntos
Amiloidose/etiologia , Febre Familiar do Mediterrâneo/complicações , Adulto , Amiloidose/genética , Artrite/etiologia , Proteínas do Citoesqueleto/genética , Diagnóstico Tardio , Febre Familiar do Mediterrâneo/genética , Feminino , Genótipo , Homozigoto , Humanos , Falência Renal Crônica/etiologia , Masculino , Pessoa de Meia-Idade , Pirina , Estudos Retrospectivos , Fatores de Risco , Fatores Sexuais , Turquia , Adulto Jovem
15.
Rev Bras Reumatol ; 53(4): 335-40, 2013 Aug.
Artigo em Inglês, Português | MEDLINE | ID: mdl-24217664

RESUMO

OBJECTIVE: The aim of the present study is to determine the frequency of enthesopathy in fibromyalgia (FM) by using a newly developed ultrasonography (US) method, the Madrid Sonography Enthesitis Index (MASEI). METHODS: This study was conducted on 38 consecutive patients with FM and 48 healthy sex- and age-matched controls. Six entheseal sites (olecranon tuberosity, superior and inferior poles of patella, tibial tuberosity, superior and inferior poles of calcaneus) on both lower limbs were evaluated. All US findings were identified according to MASEI. Scores of patients and controls were compared by Student's t-test and Mann-Whitney U-test. Validity was analysed by receiver operating characteristic curve. Values of P < 0.05 were considered significant. RESULTS: Total enthesitis score was 7.39 ± 4.99 (mean ± SD) among FM patients and 3.7 ± 3.22 among healthy controls (P < 0.001). The receiver operating characteristic curve established an ultrasound score of > 3.5 in the FM group as the best cut-off point to differentiate between cases and controls. No statistically significant correlation was found between the MASEI score and the FM disease duration, and the location of the tender points. CONCLUSIONS: Misdiagnoses of FM are harmful to patients and the community, and the presence of enthesopathy among FM patients increases. Its detection with the MASEI score may help to discriminate FM patients presenting with ill-defined symptoms and signs, in order to prevent mistreatment.


Assuntos
Fibromialgia/complicações , Doenças Reumáticas/diagnóstico por imagem , Doenças Reumáticas/etiologia , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Reumáticas/epidemiologia , Ultrassonografia , Adulto Jovem
16.
Rev. bras. reumatol ; 53(4): 335-340, ago. 2013. ilus, tab
Artigo em Português | LILACS | ID: lil-690714

RESUMO

OBJETIVO: Determinar a frequência de entesopatia na fibromialgia (FM) utilizando um método de ultrassonografia (US) recém-desenvolvido, o escore Madrid Sonography Enthesitis Index (MASEI). MÉTODOS: Este estudo foi realizado em 38 pacientes com FM consecutivos e 48 controles saudáveis pareados para idade e sexo. Seis sítios de ênteses (tuberosidade do olécrano, polos superior e inferior da patela, tuberosidade tibial, polos superior e inferior do calcâneo) nos dois membros inferiores foram avaliados. Todos os achados da US foram identificados de acordo com o escore MASEI. Os escores de pacientes e controles foram comparados usando-se o teste t de Student e o teste U de Mann-Whitney. A validade foi analisada pela curva receiver operating characteristic (ROC). Valores de P < 0,05 foram considerados significativos. RESULTADOS: O escore total de entesite foi 7,39 ± 4,99 (média ± DP) para os pacientes com FM e 3,7 ± 3,22 para os controles saudáveis (P < 0,001). A curva ROC estabeleceu um escore de US > 3,5 no grupo de FM como o melhor ponto de corte para diferenciar casos de controles. Não houve correlação estatisticamente significativa entre o escore MASEI e a duração da FM, e a localização dos pontos dolorosos. CONCLUSÕES: Erros no diagnóstico de FM são prejudiciais aos pacientes e à comunidade, e a presença de entesopatia entre pacientes com FM é crescente. Sua detecção por meio do escore MASEI pode ser útil para discriminar pacientes com FM, cujos sintomas e sinais são mal definidos, para evitar equívoco de tratamento.


OBJECTIVE: The aim of the present study is to determine the frequency of enthesopathy in fibromyalgia (FM) by using a newly developed ultrasonography (US) method, the Madrid Sonography Enthesitis Index (MASEI). METHODS: This study was conducted on 38 consecutive patients with FM and 48 healthy sex- and age-matched controls. Six entheseal sites (olecranon tuberosity, superior and inferior poles of patella, tibial tuberosity, superior and inferior poles of calcaneus) on both lower limbs were evaluated. All US findings were identified according to MASEI. Scores of patients and controls were compared by Student's t-test and Mann-Whitney U-test. Validity was analysed by receiver operating characteristic curve. Values of P < 0.05 were considered significant. RESULTS: Total enthesitis score was 7.39 ± 4.99 (mean ± SD) among FM patients and 3.7 ± 3.22 among healthy controls (P < 0.001). The receiver operating characteristic curve established an ultrasound score of > 3.5 in the FM group as the best cut-off point to differentiate between cases and controls. No statistically significant correlation was found between the MASEI score and the FM disease duration, and the location of the tender points. CONCLUSIONS: Misdiagnoses of FM are harmful to patients and the community, and the presence of enthesopathy among FM patients increases. Its detection with the MASEI score may help to discriminate FM patients presenting with ill-defined symptoms and signs, in order to prevent mistreatment.


Assuntos
Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem , Fibromialgia/complicações , Doenças Reumáticas/etiologia , Doenças Reumáticas , Doenças Reumáticas/epidemiologia
17.
Mod Rheumatol ; 2013 Apr 12.
Artigo em Inglês | MEDLINE | ID: mdl-23579501

RESUMO

OBJECTIVES: Ankylosing spondylitis (AS) is a chronic multi-systemic inflammatory rheumatic disorder. Several studies have suggested that the interval from the peak to the end of the electrocardiographic T wave (Tp-e) may correspond to the transmural dispersion of repolarization and that increased Tp-e interval and Tp-e/QT ratio are associated with malignant ventricular arrhythmias. The aim of this study was to evaluate ventricular repolarization by using Tp-e interval and Tp-e/QT ratio in patients with AS, and to assess the relation with inflammation. METHODS: Sixty-two patients with AS and 50 controls were included. Tp-e interval and Tp-e/QT ratio were measured from a 12-lead electrocardiogram, and the Tp-e interval corrected for heart rate. The plasma level of high sensitive C-reactive protein (hsCRP) was measured. These parameters were compared between groups. RESULTS: In electrocardiographic parameters analysis, QT dispersion (QTd) and corrected QTd were significantly increased in AS patients compared to the controls (31.7 ± 9.6 vs 28.2 ± 7.4 and 35.8 ± 11.5 vs 30.6 ± 7.9 ms, P = 0.03 and P = 0.007, respectively). cTp-e interval and Tp-e/QT ratio were also significantly higher in AS patients (92.1 ± 10.2 vs 75.8 ± 8.4 and 0.22 ± 0.02 vs 0.19 ± 0.02 ms, all P values <0.001). cTp-e interval and Tp-e/QT ratio were significantly correlated with hsCRP (r = 0.63, P < 0.001 and r = 0.49, P < 0.001, respectively). CONCLUSIONS: Our study revealed that Tp-e interval and Tp-e/QT ratio were increased in AS patients. These electrocardiographic ventricular repolarization indexes were significantly correlated with the plasma level of hsCRP.

18.
BMJ Case Rep ; 20122012 Nov 30.
Artigo em Inglês | MEDLINE | ID: mdl-23203187

RESUMO

Granulomatosis with polyangiitis (GPA) (Wegener's) is a multiorgan system disease of unknown aetiology characterised by granulomatous inflammation, tissue necrosis and vasculitis. The characteristic lung parenchymal lesions of GPA are firm spherical nodules that may cavitate. Pneumothorax (PX) can develop as a quiet rare complication of cavitary nodules. Our case admitted to our clinic with the diagnosis of GPA showing cavitary pulmonary mass. While taking immunosuppressive treatment, spontaneous PX on left lung was developed. A closed chest tube was inserted to the left lung for expansion of PX. Even after 30 days, the left lung did not re-expand and wedge resection with thoracotomy was conducted and the closed chest tube was still in the left lung. On the seventh day, empyema emerged as a complication and, with appropriate treatment, the patient became well. In GPA patients taking immunosuppressive medication, PX is a serious complication and requires aggressive therapy with broad-spectrum antibiotics.


Assuntos
Empiema Pleural/tratamento farmacológico , Granulomatose com Poliangiite/complicações , Pneumopatias/etiologia , Pneumotórax/etiologia , Pseudomonas aeruginosa , Adulto , Antibacterianos/uso terapêutico , Anti-Inflamatórios/uso terapêutico , Cilastatina/uso terapêutico , Combinação Imipenem e Cilastatina , Ciclofosfamida/uso terapêutico , Combinação de Medicamentos , Empiema Pleural/microbiologia , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Imipenem/uso terapêutico , Imunossupressores/uso terapêutico , Pneumopatias/patologia , Masculino , Pneumotórax/cirurgia , Prednisona/uso terapêutico
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