Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 67
Filtrar
Mais filtros










Base de dados
Intervalo de ano de publicação
2.
Clin Exp Rheumatol ; 38(1): 74-81, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-30943131

RESUMO

OBJECTIVES: Patients with systemic lupus erythematosus (SLE) are under increased risk for cardiovascular events (CVE) and mortality. Aortic stiffness, as measured by carotid-femoral pulse wave velocity (cfPWV), has been shown to predict CVE and mortality in the general population. The aim of the present study was to examine the factors associated with cfPWV in patients with SLE and to determine differences of SLE patients in comparison to healthy controls. METHODS: 125 patients with SLE and 104 controls were included. Demographic, medication and cardiovascular risk factor data were collected from all participants. Furthermore, clinical and laboratory SLE associated parameters were documented in the patients' group. All subjects underwent measurements of blood pressure and cfPWV. RESULTS: Interestingly, only age (ß=0.55; p<0.001), mean arterial pressure (MAP) (ß=0.29; p<0.001) and estimated glomerular filtration rate (eGFR) (ß=-0.20; p=0.033) were associated independently with cfPWV in patients with SLE. Moreover, there was no difference of cfPWV between patients with SLE and controls before (p=0.301) and after adjustment for disparities between the groups (p=0.671). CONCLUSIONS: Vascular stiffness in patients with SLE seems to be independent from SLE-related factors and from most traditional CVRF and is mainly associated with age, MAP and renal function defined as eGFR. There is an independent correlation between eGFR and cfPWV in a SLE population with a widely normally ranged eGFR. There is no difference of cfPWV between patients with SLE and controls.

3.
J Med Internet Res ; 21(6): e12285, 2019 06 18.
Artigo em Inglês | MEDLINE | ID: mdl-31215515

RESUMO

BACKGROUND: As inpatient medical rehabilitation serves to promote work ability, vocational reintegration is a crucial outcome. However, previous Web-based trials on coping with work-related stress have been limited to Web-based recruitment of study participants. OBJECTIVE: The aim of our study was to evaluate the implementation of an empirically supported transdiagnostic psychodynamic Web-based aftercare program GSA (Gesund und Stressfrei am Arbeitsplatz [Healthy and stress-less at the workplace])-Online plus into the clinical routine of inpatient medical rehabilitation, to identify characteristics of patients who have received the recommendation for GSA-Online plus, and to determine helpfulness of the intervention and satisfaction of the participants as well as improvement in quality of life and mental health status of the regular users of GSA-Online plus. METHODS: GSA-Online plus was prescribed by physicians at termination of orthopedic psychosomatic inpatient rehabilitation. Participants' use of the program, work-related attitudes, distress, and quality of life were assessed on the Web. RESULTS: In 2 rehabilitation centers, 4.4% (112/2562) of rehabilitants got a recommendation for GSA-Online plus during inpatient rehabilitation. Compared with usual person aftercare, the Web-based aftercare program was rarely recommended by physicians. Recommendations were made more frequently in psychosomatic (69/1172, 5.9%) than orthopedic (43/1389, 3.1%) rehabilitation (χ21=11.845, P=.001, Cramér V=-0.068) and to younger patients (P=.004, d=0.28) with longer inpatient treatment duration (P<.001, r=-0.12) and extended sick leaves before inpatient medical rehabilitation (P=.004; Cramér V=0.072). Following recommendation, 77% (86/112) of rehabilitants participated in Web-based aftercare. Completers (50/86, 58%) reported statistically significant improvements between discharge of inpatient treatment and the end of the aftercare program for subjective work ability (P=.02, d=0.41), perceived stress (P=.01, d=-0.38), functioning (P=.002, d=-0.60), and life satisfaction (P=.008, d=0.42). CONCLUSIONS: Physicians' recommendations of Web-based aftercare are well accepted by patients who derive considerable benefits from participation. However, a low rate of prescription compared with other usual aftercare options points to barriers among physicians to prescribing Web-based aftercare.

4.
Ann Rheum Dis ; 78(9): 1226-1234, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31186256

RESUMO

OBJECTIVES: We explored the impact of circulating anti-N-methyl-D-aspartate receptor (NMDAR) antibodies on the severity of fatigue in patients with systemic lupus erythematosus (SLE). METHODS: Serum samples of 426 patients with SLE were analysed for the presence of antibodies to the NR2 subunit of the NMDAR. In parallel, the severity of fatigue was determined according to the Fatigue Scale for Motor and Cognitive functions questionnaire. In a subgroup of patients with SLE, the hippocampal volume was correlated with the levels of anti-NR2 antibodies. Isolated immunoglobulin G from patients with anti-NR2 antibodies were used for murine immunohistochemical experiments and functional assays on neuronal cell lines. Treatment effects were studied in 86 patients with lupus under belimumab therapy. RESULTS: We found a close correlation between the titre of anti-NR2 antibodies, the severity of fatigue, the clinical disease activity index (Systemic Lupus Erythematosus Disease Activity Index 2000) and anti-double stranded DNA antibodies-independently of the presence of neuropsychiatric lupus manifestations. Pathogenic effects could be demonstrated by (1) detection of anti-NR2 antibodies in the cerebrospinal fluid, (2) in situ binding of anti-NR2 antibodies to NMDAR of the hippocampus area and (3) distinct functional effects in vitro: downregulating the energy metabolism of neuronal cells without enhanced cytotoxicity. Treatment with belimumab for at least 6 months affected both the severity of fatigue and the levels of anti-NR2 antibodies. CONCLUSION: The presence of anti-NR2 antibodies in patients with SLE with fatigue is a helpful diagnostic tool and may offer a major approach in the therapeutic management of this important disabling symptom in patients with SLE.

5.
Clin Exp Rheumatol ; 37(6): 994-1002, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30943141

RESUMO

OBJECTIVES: Macrovascular involvement and cardiovascular (CV) risk has not been sufficiently studied in mixed connective tissue disease (MCTD). In particular, the gold standard assessment method of aortic stiffness carotid-femoral pulse wave velocity (cfPWV) has never been evaluated in patients with this disease. The aims of the present study were therefore to examine cfPWV in MCTD and to evaluate its associations with MCTD-associated markers and traditional CV risk factors. METHODS: Measurements of cfPWV were performed in 43 MCTD patients and 107 healthy controls. The difference between cfPWV in the two groups was statistically examined and subsequently controlled for the effect of possible confounding factors. The association of cfPWV with MCTD-associated organ involvement, routine laboratory parameters and immunoserological markers was also evaluated. Finally, the relationship of cfPWV with medications and traditional CV risk factors was examined. RESULTS: Adjusted statistical analyses for confounding factors showed significantly higher cfPWV values in MCTD patients in comparison to controls (padj<0.001). cfPWV correlated in both the patients and the control group significantly with age (rho=0.69, p<0.001 and rho=0.67, p<0.001 respectively) and diastolic arterial pressure (padj=0.024 and padj=0.032 respectively). Moreover, cfPWV correlated in the control group with systolic and mean arterial pressure (padj<0.001 and p=0.002 respectively). Finally, higher cfPWV values could be documented in the subset of MCTD patients without lung involvement (padj=0.007). CONCLUSIONS: Patients with MCTD have significantly higher aortic stiffness and thus CV risk in comparison to controls. Except for the disease itself, age and blood pressure were the main predictors of cfPWV.


Assuntos
Doenças Cardiovasculares , Doença Mista do Tecido Conjuntivo , Rigidez Vascular , Aorta/fisiopatologia , Pressão Sanguínea , Doenças Cardiovasculares/etiologia , Humanos , Doença Mista do Tecido Conjuntivo/complicações , Análise de Onda de Pulso , Fatores de Risco
6.
Dtsch Med Wochenschr ; 144(8): e51-e57, 2019 04.
Artigo em Alemão | MEDLINE | ID: mdl-30986856

RESUMO

BACKGROUND: To examine clinical, comorbidity and demographic aspects of gout and to explore the routine clinical practice of gout treatment among general practitioners (G.P.'s) in southwest Germany. METHODS: Gout specific questionnaires were sent to all G.P.'s in Rhineland Palatinate (RL-P), through the Panel physicians' Association. Questionnaires consisted of items exploring epidemiological, medication and comorbidity data. Moreover, questions regarding clinical gout manifestations were included with an extra focus on therapy-refractory cases. Finally, G.P.'s were asked to rate the current care status of gout. RESULTS: Data from 4016 gout patients (age at diagnosis: 62.8 years, IQR 55 - 67.8) were collected. The majority of patients were male (75 %) with podagra being the most common gout manifestation (85 %). Chronic tophaceous courses were reported in 15 % (median 10 %, IQR 2 - 20) and spinal involvement in 2.7 % (median 0 %, IQR 0 - 2) of patients respectively. An average of 11.3 % cases (median 10 %, IQR 2.3 - 20) were defined as "hard-to-treat". However, biologic agents were not namely reported as applied treatments. 32 % of patients were diagnosed with gout by their G.P., whereas 68 % had to visit further physicians. A definite diagnosis could be reached after 3.1 months on average (median 0.3, IQR 0.1 - 1). CONCLUSIONS: In the era of biologic therapies there is a need for optimization of gout management. Important targets are the shortening of diagnostic periods and the prevention of chronic disease courses. Critical awareness of the disease and its comorbidities, standardized treatment and patient-training could be important steps toward this direction.


Assuntos
Medicina Geral/métodos , Gota/epidemiologia , Gota/terapia , Idoso , Artrite Gotosa/diagnóstico , Artrite Gotosa/epidemiologia , Artrite Gotosa/terapia , Comorbidade , Feminino , Medicina Geral/estatística & dados numéricos , Alemanha/epidemiologia , Gota/complicações , Gota/diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Educação de Pacientes como Assunto , Encaminhamento e Consulta/estatística & dados numéricos , Insuficiência Renal/complicações , Fatores de Risco , Inquéritos e Questionários , Fatores de Tempo
7.
Rheumatology (Oxford) ; 58(6): 987-996, 2019 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-30597077

RESUMO

OBJECTIVE: Fatigue is reported in up to 90% of patients with SLE. This study was conducted to identify the determinants associated with fatigue in a large cohort of patients with SLE, as well as to provide a systematic review of the literature. METHODS: Patients from the Lupus BioBank of the upper Rhein, a large German-French cohort of SLE patients, were included in the FATILUP study if they fulfilled the 1997 ACR criteria for SLE and had Fatigue Scale for Motor and Cognitive Functions scores collected. Multivariate logistic regression analyses were performed to assess the determinants of fatigue and severe fatigue. RESULTS: A total of 570 patients were included (89.1% female). The median age was 42 years (interquartile range 25-75: 34-52). The median value of the SAfety of Estrogens in Lupus Erythematosus National Assessment (SELENA)-SLEDAI was 2 (0-4). Fatigue was reported by 386 patients (67.7%) and severe fatigue by 209 (36.7%). In multivariate analyses, fatigue was associated with depression [odds ratio (OR): 4.72 (95% CI: 1.39-16.05), P = 0.01], anxiety [OR: 4.49 (95% CI: 2.60-7.77), P < 0.0001], glucocorticoid treatment [OR: 1.59 (95% CI 1.05-2.41), P = 0.04], SELENA-SLEDAI scores [OR: 1.05 (95% CI: 1.00-1.12) per 1 point increase, P = 0.043] and age at sampling [OR: 1.01 (95% CI: 1.00-1.03) per 1 year increase, P = 0.03]. Severe fatigue was independently associated with anxiety (P < 0.0001), depression (P < 0.0001), glucocorticoid treatment (P = 0.047) and age at sampling (P = 0.03). CONCLUSION: Both fatigue and severe fatigue are common symptoms in SLE, and are strongly associated with depression and anxiety. Disease activity and the use of glucocorticoids were also independently associated with fatigue, although more weakly.


Assuntos
Ansiedade/complicações , Depressão/complicações , Fadiga/etiologia , Lúpus Eritematoso Sistêmico/complicações , Qualidade de Vida , Adulto , Idoso , Feminino , Humanos , Lúpus Eritematoso Sistêmico/diagnóstico , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Índice de Gravidade de Doença , Adulto Jovem
8.
Z Rheumatol ; 78(7): 677-684, 2019 Sep.
Artigo em Alemão | MEDLINE | ID: mdl-29869153

RESUMO

BACKGROUND: Giant cell arteritis (GCA) is one of the most common forms of inflammatory vasculitis in older patients. Because of possible irreversible vision deterioration, a fastest possible diagnosis and therapy is of absolute importance. To date, there are still no reliable data to obtain an initial assessment of the outpatient health care situation of patients diagnosed with GCA in Rhineland-Palatinate. METHODS: The specialists (neurologists, rheumatologists, ophthalmologists and general practitioners) participating in the statewide rheumatology network ADAPTHERA were questioned with the help of a questionnaire regarding disease frequency, activity, drug therapy and possible comorbidities. In addition, the collected data were compared and supplemented by the ambulatory coding of the Association of Statutory Health Insurance Physicians in Rhineland-Palatinate. RESULTS: Based on the information provided by general practitioners, 272 GCA patients were treated in Rhineland-Palatinate during the survey period. The average duration of the disease until diagnosis was 3.6 (SD ± 4.8) months. Drug therapy in the form of glucocorticoids was in first place followed by methotrexate, acetylsalicylic acid (ASA) and azathioprine. Cardiovascular diseases, chronic pain syndromes, depression, osteoporosis and diabetes mellitus were also described as comorbidities. CONCLUSION: The majority of patients with GCA are being cared for by general practitioners (GP). Long-term therapy and timely rheumatological co-treatment seem to be problematic. The primary care providers expressed their wishes for rheumatological training and further education measures. In terms of diagnosis and treatment, there is a demand to implement a "vasculitis fast-track" module.


Assuntos
Educação Médica Continuada/organização & administração , Arterite de Células Gigantes , Polimialgia Reumática , Atenção Primária à Saúde , Reumatologia , Idoso , Alemanha , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/terapia , Glucocorticoides , Humanos , Assistência ao Paciente
10.
J Rheumatol ; 46(3): 279-284, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30442823

RESUMO

OBJECTIVE: To describe nailfold videocapillaroscopy (NVC) features of patients with antisynthetase syndrome (AS) and to investigate possible correlations with clinical and serological features of the disease. METHODS: We retrospectively analyzed NVC images of 190 patients with AS [females/males 3.63, mean age 49.7 ± 12.8 yrs, median disease duration 53.7 mos (interquartile range 82), 133 anti-Jo1 and 57 non-anti-Jo1-positive patients]. For each patient, we examined number of capillaries, giant capillaries, microhemorrhages, avascular areas, ramified capillaries, and the presence of systemic sclerosis (SSc)-like pattern. Finally, we correlated NVC features with clinical and serological findings of patients with AS. Concomitantly, a historical cohort of 75 patients with antinuclear antibody-negative primary Raynaud phenomenon (RP) and longterm followup was used as a control group (female/male ratio 4.13/1, mean age 53.9 ± 17.6 yrs) for NVC measures. RESULTS: NVC abnormalities were observed in 62.1% of AS patients compared with 29.3% of primary RP group (p < 0.001). An SSc-like pattern was detected in 67 patients (35.3%) and it was associated with anti-Jo1 antibodies (p = 0.002) and also with a longer disease duration (p = 0.004). Interestingly, there was no significant correlation between the presence of SSc-like pattern and RP, and only 47% of patients with SSc-like pattern had RP. CONCLUSION: NVC abnormalities are commonly observed in AS, independently from the occurrence of RP. The presence of an SSc-like pattern could allow identification of a more defined AS subtype, and prospective studies could confirm the association with clinical and serological features of AS.

12.
Methods Mol Biol ; 1802: 11-29, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29858799

RESUMO

The aim of this review is to provide a brief overview of the role and current clinical relevance of HLA-B27 in spondyloarthritis and HLA-B51 in Behcet's disease as well as HLA-DQ2/DQ8 in celiac disease and HLA-DRB1 in rheumatoid arthritis and to discuss possible future implications.


Assuntos
Doenças Autoimunes/imunologia , Antígenos HLA/metabolismo , Epitopos/metabolismo , Antígenos de Histocompatibilidade Classe II/metabolismo , Humanos
13.
Rheumatol Ther ; 5(2): 371-381, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-29860570

RESUMO

INTRODUCTION: The aim of this study was to determine the prevalence of methicillin-resistant Staphylococcus aureus (MRSA) both in rheumatologic and non-rheumatologic rehabilitation centers. In addition, we sought to evaluate the practice value of existing screening recommendations of the German Commission for Hospital Hygiene and Infection Prevention (KRINKO). METHODS: The analysis was performed in four rehabilitation clinics (rheumatology, psychosomatic medicine, oncology, and cardiology) with at least 200 patients per clinic tested for MRSA. RESULTS: Nine (1.1%) of the 842 patients were colonized with MRSA. Only five of them should have been tested according to the commission's recommendations. The prevalence was 0.5% (n = 207) in rheumatologic, 0.9% (n = 224) in psychosomatic, 1.4% (n = 209) in oncologic and 1.5% (n = 202) in cardiologic patients. We found a greater exposure to risk factors in cardiologic and oncologic patients. Among patients with carrier status, a higher percentage was exposed to three potential risk factors not applied by the commission. CONCLUSIONS: The prevalence of MRSA in our cohort correlates with data from previous studies. The low percentage among rheumatologic patients suggests that they are not more likely to reveal MRSA carrier status than other patient groups and that long-term immunosuppression does not necessarily represent a risk factor for MRSA colonization. Since only five out of nine patients with carrier status would have been detected following the recommendations of the KRINKO, further studies on potential risk factors are warranted.

14.
Rheumatol Ther ; 5(1): 5-20, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29516402

RESUMO

Since the discovery of HLA 60 years ago, it has contributed to the understanding of the immune system as well as of the pathogenesis of several diseases. Aside from its essential role in determining donor-recipient immune compatibility in organ transplantation, HLA genotyping is meanwhile performed routinely as part of the diagnostic work-up of certain autoimmune diseases. Considering the ability of HLA to influence thymic selection as well as peripheral anergy of T cells, its role in the pathogenesis of autoimmunity is understandable. The aim of this paper is to provide a brief overview of the role and current clinical relevance of HLA-B27 in spondyloarthritis and HLA-B51 in Behçet's disease as well as HLA-DQ2/DQ8 in celiac disease and HLA-DRB1 in rheumatoid arthritis and to discuss possible future implications.

15.
Clin Exp Rheumatol ; 36(1): 44-49, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-28770709

RESUMO

OBJECTIVES: To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD). METHODS: The patients were selected from a retrospective large international cohort of ASSD patients regularly followed-up in centres referring to AENEAS collaborative group. Patients were eligible if they had an antisynthetase antibody testing positive in at least two determinations along with arthritis occurring either at ASSD onset (Group 1) or during the course of the disease (Group 2). RESULTS: 445 (70%; 334 females, 110 males, 1 transsexual) out of the 636 ASSD we collected had arthritis, in the majority of cases (367, 83%) from disease onset (Group 1). Patients belonging to Group 1 with respect to Group 2 had an arthritis more commonly polyarticular and symmetrical (p=0.015), IgM-Rheumatoid factor positive (p=0.035), erosions at hands and feet plain x-rays (p=0.036) and more commonly satisfying the 1987 revised classification criteria for rheumatoid arthritis (RA) (p=0.004). Features such as Raynaud's phenomenon, mechanic's hands and fever (e.g. accompanying findings) were more frequently reported in Group 2 (p=0.005). CONCLUSIONS: In ASSD, the timing of appearance with respect to disease onset influences arthritis characteristics. In particular, RA features are more common when arthritis occurs from ASSD onset, suggesting an overlap between RA and ASSD in these patients. When arthritis appears during the follow-up, it is very close to a connective tissue disease-related arthritis. Also, the different prevalence of accompanying features between these two groups is in line with this possibility.


Assuntos
Artrite/epidemiologia , Miosite/epidemiologia , Adulto , Artrite/diagnóstico , Artrite/imunologia , Autoanticorpos/sangue , Biomarcadores/sangue , Europa (Continente)/epidemiologia , Feminino , Humanos , Masculino , México/epidemiologia , Pessoa de Meia-Idade , Miosite/diagnóstico , Miosite/imunologia , Fenótipo , Prevalência , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo
16.
Arthritis Rheumatol ; 69(12): 2370-2379, 2017 12.
Artigo em Inglês | MEDLINE | ID: mdl-29193819

RESUMO

OBJECTIVE: To determine the effect of selexipag, an oral, selective IP prostacyclin receptor agonist, on the frequency of attacks of Raynaud's phenomenon (RP) in patients with systemic sclerosis (SSc). METHODS: Patients with SSc-related RP were randomized 1:1 to placebo (n = 38) or selexipag (n = 36) in individualized doses (maximum of 1,600 µg twice daily) during a 3-week titration period. The primary end point was the weekly average number of RP attacks during the study maintenance period, analyzed using a Bayesian approach with a negative binomial model adjusted for baseline number of RP attacks. Other outcome measures included Raynaud's Condition Score (RCS), RP attack duration, and treatment-emergent adverse events (AEs). RESULTS: Baseline characteristics were comparable between treatment groups. For 83.3% of patients, the individualized maintenance dosage of selexipag was ≤800 µg twice daily. No significant difference was observed between placebo and selexipag in weekly average number of electronic diary (eDiary)-recorded RP attacks during the maintenance period (14.2 attacks during the maintenance period and 21.5 attacks during the baseline week in the placebo group [n = 32] versus 18.0 attacks during the maintenance period and 22.4 attacks during the baseline week in the selexipag group [n = 27]; adjusted mean treatment difference of 3.4 in favor of placebo). No significant treatment effect was observed on RCS or RP attack duration. In the double-blind period, 86.8% of placebo-treated patients and 100% of selexipag-treated patients reported ≥1 AE; 55.3% and 91.7%, respectively, reported ≥1 prostacyclin-associated AE. CONCLUSION: Treatment with selexipag did not reduce the number of RP attacks compared with placebo. The safety profile of selexipag was similar to that previously reported. This study provides important information about the feasibility of eDiary reporting of RP attacks in clinical trials.


Assuntos
Acetamidas/administração & dosagem , Anti-Hipertensivos/administração & dosagem , Pirazinas/administração & dosagem , Doença de Raynaud/tratamento farmacológico , Escleroderma Sistêmico/complicações , Adulto , Teorema de Bayes , Método Duplo-Cego , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Raynaud/etiologia , Índice de Gravidade de Doença , Resultado do Tratamento
18.
Z Gastroenterol ; 55(5): 473-478, 2017 May.
Artigo em Alemão | MEDLINE | ID: mdl-28376538

RESUMO

Background Elevated liver values are the most common pathological laboratory result in Germany. Frequent findings, especially in younger patients, are nutritive- or medicamentous- toxic reasons, viral or autoimmune hepatitis. A macrophage activation syndrome (MAS) may manifest like a viral infectious disease with fever, hepatosplenomegaly and pancytopenia and is associated with a high mortality. It is based on an enhanced activation of macrophages with increased cytokine release, leading to organ damage and multi-organ failure. In addition to genetic causes, MAS is commonly associated with infections and rheumatic diseases. We report the case of a 26-year-old female patient suffering from MAS as a rare cause of elevated liver enzymes. Methods Patient characteristics, laboratory values, liver histology, bone marrow and radiological imaging were documented and analyzed. Case Report After an ordinary upper airway infection with bronchitis, a rheumatic arthritis appeared and was treated with leflunomide und methotrexate. In the further course of the disease, the patient developed an acute hepatitis with fever, pancytopenia and massive hyperferritinemia. Immunohistochemistry of the liver biopsy revealed hemophagocytosis and activation of CD68-positive macrophages. In the radiological and histological diagnostics of the liver and bone marrow, an MAS was diagnosed as underlying disease of the acute hepatitis. Under therapy with prednisolone, the fever disappeared and transaminases and ferritin rapidly normalized. Conclusion Aside from the frequent causes of elevated liver values in younger patients, such as nutritive toxic, drug induced liver injury, viral or autoimmune hepatitis, especially in case of massive hyperferritinemia, a MAS should be considered as a rare cause of acute liver disease.


Assuntos
Hepatite Autoimune , Distúrbios do Metabolismo do Ferro/complicações , Síndrome de Ativação Macrofágica , Doença Aguda , Adulto , Feminino , Alemanha , Hepatite Autoimune/complicações , Hepatite Autoimune/diagnóstico , Humanos , Síndrome de Ativação Macrofágica/complicações
19.
Autoimmun Rev ; 16(3): 253-257, 2017 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-28147261

RESUMO

OBJECTIVE: Arthritis, myositis and interstitial lung disease (ILD) constitute the classic clinical triad of anti-synthetase syndrome (ASSD). These patients experience other accompanying features, such as Raynaud's phenomenon, fever or mechanic's hands. Most ASSD patients develop the complete triad during the follow-up. In the present study we aimed to determine whether the subsequent appearance of accompanying features may suggest the development of triad findings lacking at the onset in anti-Jo1 positive ASSD patients. METHODS: Anti-Jo1 positive patients presenting with incomplete ASSD (no >2 classic triad features) were assessed. Clinical characteristics and clusters of disease manifestations were retrospectively collected and analyzed in a large international multicenter cohort of ASSD patients. RESULTS: 165 patients (123 women) with incomplete ASSD were identified. Ninety-five patients (57.5%) developed new classic triad manifestations after 15months median (IQR 9-51) and 40 (24%) developed new accompanying features after 19months median (IQR 6-56) from disease onset. During the follow-up, the ex-novo occurrence of triad features was observed in 32 out of 40 patients (80%) with new accompanying findings and in 63 out of 125 patients (50.5%) without new accompanying findings (p=0.002). In patients with at least one new accompanying feature the odds ratio for the occurrence of new triad manifestations was 3.94 with respect to patients not developing ex-novo accompanying findings (95% CI 1.68-9.21, p=0.002). CONCLUSION: Anti-Jo1 ASSD patients with incomplete forms at disease onset are at high risk for the subsequent occurrence of lacking classic triad findings. Although all ASSD patients should be carefully assessed for the occurrence of new triad features, a closer follow-up should be considered in the subgroup of patients developing ex novo accompanying findings. These patients, indeed, have near four-fold increased risk for new classic triad manifestation occurrence with respect to patients not presenting ex novo accompanying findings.


Assuntos
Ligases/antagonistas & inibidores , Doença de Raynaud/metabolismo , Autoanticorpos , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Síndrome
20.
Arthritis Rheumatol ; 69(5): 1016-1027, 2017 05.
Artigo em Inglês | MEDLINE | ID: mdl-28118533

RESUMO

OBJECTIVE: To assess the efficacy and safety of subcutaneous (SC) belimumab in patients with systemic lupus erythematosus (SLE). METHODS: Patients with moderate-to-severe SLE (score of ≥8 on the Safety of Estrogens in Lupus Erythematosus National Assessment [SELENA] version of the SLE Disease Activity Index [SLEDAI]) were randomized 2:1 to receive weekly SC belimumab 200 mg or placebo by prefilled syringe in addition to standard SLE therapy for 52 weeks. The primary end point was the SLE Responder Index (SRI4) at week 52. Secondary end points were reduction in the corticosteroid dosage and time to severe flare. Safety was assessed according to the adverse events (AEs) reported and the laboratory test results. RESULTS: Of 839 patients randomized, 836 (556 in the belimumab group and 280 in the placebo group) received treatment. A total of 159 patients withdrew before the end of the study. At entry, mean SELENA-SLEDAI scores were 10.5 in the belimumab group and 10.3 in the placebo group. More patients who received belimumab were SRI4 responders than those who received placebo (61.4% versus 48.4%; odds ratio [OR] 1.68 [95% confidence interval (95% CI) 1.25-2.25]; P = 0.0006). In the belimumab group, both time to and risk of severe flare were improved (median 171.0 days versus 118.0 days; hazard ratio 0.51 [95% CI 0.35-0.74]; P = 0.0004), and more patients were able to reduce their corticosteroid dosage by ≥25% (to ≤7.5 mg/day) during weeks 40-52 (18.2% versus 11.9%; OR 1.65 [95% CI 0.95-2.84]; P = 0.0732), compared with placebo. AE incidence was comparable between treatment groups; serious AEs were reported by 10.8% of patients taking belimumab and 15.7% of those taking placebo. A worsening of IgG hypoglobulinemia by ≥2 grades occurred in 0.9% of patients taking belimumab and 1.4% of those taking placebo. CONCLUSION: In patients with moderate-to-severe SLE, weekly SC doses of belimumab 200 mg plus standard SLE therapy significantly improved their SRI4 response, decreased severe disease flares as compared with placebo, and had a safety profile similar to placebo plus standard SLE therapy.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Corticosteroides/administração & dosagem , Adulto , Progressão da Doença , Método Duplo-Cego , Feminino , Humanos , Imunoglobulina G/imunologia , Síndromes de Imunodeficiência/induzido quimicamente , Síndromes de Imunodeficiência/imunologia , Infusões Subcutâneas , Masculino , Pessoa de Meia-Idade , Razão de Chances , Modelos de Riscos Proporcionais , Índice de Gravidade de Doença , Terapêutica , Resultado do Tratamento
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA