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1.
Z Rheumatol ; 2021 Mar 16.
Artigo em Alemão | MEDLINE | ID: mdl-33725179

RESUMO

The SAPHO syndrome is not a single entity but an inhomogeneous, nosologically heterogeneous complex of symptoms with unknown etiology and heterogeneous pathogenesis. Clinically subacute, recurrent or chronic disease processes and a common skin-bone association (skibo disease) can be found. Under the acronym SAPHO, chronically recurrent multifocal osteomyelitis (CRMO) is the most common disease that can occur in youth as well as adolescence. Spondylarthritis hyperostotica pustulo-psoriatica with the triad palmoplantar pustulosis, sternoclavicular hyperostosis and ossifying spinal manifestations is the most common SAPHO form found in adults. Abortive disease forms are the inflammatory anterior chest wall syndrome, extended sternoclavicular hyperostosis syndrome of the clavicle bone, acne CRMO and acne spondylarthritis. The SAPHO disease usually heals with a relatively favorable prognosis but there are also unfavorable courses with functional limitations. The diagnosis should be made based on clinical examination, imaging (x-ray, scintigraphy, magnetic resonance imaging) and/or histological bone biopsy analysis. Treatment should be interdisciplinary. Antibiotic treatment is obsolete. This article provides an overview of the SAPHO syndrome and a clinical-rheumatological imaging differentiation as well as classification of 35 cases at first presentation.

2.
RMD Open ; 7(1)2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33622673

RESUMO

OBJECTIVE: To analyse the impact of the COVID-19 pandemic on rheumatic patients' and rheumatologists' usage, preferences and perception of digital health applications (DHAs). METHODS: A web-based national survey was developed by the Working Group Young Rheumatology of the German Society for Rheumatology and the German League against Rheumatism. The prospective survey was distributed via social media (Twitter, Instagram and Facebook), QR code and email. Descriptive statistics were calculated, and regression analyses were performed to show correlations. RESULTS: We analysed the responses of 299 patients and 129 rheumatologists. Most patients (74%) and rheumatologists (76%) believed that DHAs are useful in the management of rheumatic and musculoskeletal diseases (RMDs) and felt confident in their own usage thereof (90%; 86%). 38% of patients and 71% of rheumatologists reported that their attitude had changed positively towards DHAs and that their usage had increased due to COVID-19 (29%; 48%). The majority in both groups agreed on implementing virtual visits for follow-up appointments in stable disease conditions. The most reported advantages of DHAs were usage independent of time and place (76.6%; 77.5%). The main barriers were a lack of information on suitable, available DHAs (58.5%; 41.9%), poor usability (42.1% of patients) and a lack of evidence supporting the effectiveness of DHAs (23.2% of rheumatologists). Only a minority (<10% in both groups) believed that digitalisation has a negative impact on the patient-doctor relationship. CONCLUSION: The COVID-19 pandemic instigated an increase in patients' and rheumatologists' acceptance and usage of DHAs, possibly introducing a permanent paradigm shift in the management of RMDs.


Assuntos
Doenças Musculoesqueléticas/terapia , Aceitação pelo Paciente de Cuidados de Saúde/estatística & dados numéricos , Reumatologistas/estatística & dados numéricos , Telemedicina , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Conhecimentos, Atitudes e Prática em Saúde , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Inquéritos e Questionários , Adulto Jovem
3.
Adv Rheumatol ; 61(1): 3, 2021 01 13.
Artigo em Inglês | MEDLINE | ID: mdl-33436106

RESUMO

INTRODUCTION: Whole-body cryotherapy (WBC) has shown to be beneficial in the treatment of fibromyalgia (FM). There is cumulative evidence that cytokines play a crucial role in FM. It's unknown whether clinical effects of WBC can be demonstrated at the molecular level and how long the effects last. METHODS: We compared effects of serial WBC (6 sessions (- 130 °C in 6 weeks) in FM patients and healthy controls (HC). Primary outcome was the change in pain level (visual analogue scale 0-100 mm) after 6 sessions. Secondary outcomes were a change in disease activity (revised Fibromyalgia Impact Questionnaire) and pain after 3 sessions and 3 months after discontinued therapy and in cytokine levels (interleukin (IL-)1, IL-6, tumor necrosis factor α (TNF-α) and IL-10). The patients' opinions on the satisfaction, effectiveness and significance of WBC were evaluated. RESULTS: Twenty-three FM patients and 30 HC were enrolled. WBC resulted in a significant reduction in pain and disease activity after 3 and 6 sessions. No clinical benefit could be measured 3 months after discontinued treatment. Overall, probands were satisfied with WBC and considered WBC to be important and effective. FM patients had significantly different levels of IL-1, IL-6, TNF-α and IL-10 at each reading point compared to HC. Levels of IL-1, IL-6 and IL-10 were significantly altered over time in FM patients. Compared to HC FM patients showed a significantly different response of IL1, - 6 and - 10 to WBC. CONCLUSION: Serial WBC is a fast acting and effective treatment for FM. Proven effects of WBC may be explained by changes in cytokines.

5.
Adv Rheumatol ; 61: 3, 2021. tab, graf
Artigo em Inglês | LILACS-Express | LILACS | ID: biblio-1152737

RESUMO

Abstract Introduction: Whole-body cryotherapy (WBC) has shown to be beneficial in the treatment of fibromyalgia (FM). There is cumulative evidence that cytokines play a crucial role in FM. It's unknown whether clinical effects of WBC can be demonstrated at the molecular level and how long the effects last. Methods: We compared effects of serial WBC (6 sessions (- 130 °C in 6 weeks) in FM patients and healthy controls (HC). Primary outcome was the change in pain level (visual analogue scale 0-100 mm) after 6 sessions. Secondary outcomes were a change in disease activity (revised Fibromyalgia Impact Questionnaire) and pain after 3 sessions and 3 months after discontinued therapy and in cytokine levels (interleukin (IL-)1, IL-6, tumor necrosis factor α (TNF-α) and IL-10). The patients' opinions on the satisfaction, effectiveness and significance of WBC were evaluated. Results: Twenty-three FM patients and 30 HC were enrolled. WBC resulted in a significant reduction in pain and disease activity after 3 and 6 sessions. No clinical benefit could be measured 3 months after discontinued treatment. Overall, probands were satisfied with WBC and considered WBC to be important and effective. FM patients had significantly different levels of IL-1, IL-6, TNF-α and IL-10 at each reading point compared to HC. Levels of IL-1, IL-6 and IL-10 were significantly altered over time in FM patients. Compared to HC FM patients showed a significantly different response of IL1, - 6 and - 10 to WBC. Conclusion: Serial WBC is a fast acting and effective treatment for FM. Proven effects of WBC may be explained by changes in cytokines.

8.
Int J Hyperthermia ; 37(1): 965-970, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32781869

RESUMO

INTRODUCTION: Aim of this randomized controlled trial was to evaluate the effects of serial locally applied water-filtered infrared A radiation (sl-wIRAR) in patients with axial spondyloarthritis (axSpA). METHODS: axSpA patients with active disease undergoing a 7-day multimodal rheumatologic complex treatment under non-steroidal anti-inflammatory drug (NSAID) therapy were eligible. Patients were randomly assigned in a 1:1 ratio. The intervention group (IG) received additional sl-wIRAR treatment of the back (2 treatments for 30 min per day for 6 days) to assess whether locally applied hyperthermia can i) reduce pain levels, ii) reduce disease activity and improve functionality and iii) whether an effect on tumor necrosis factor α (TNFα) levels is detectable. Additionally, it was examined whether a reduction in NSAID therapy could be achieved after trial completion. RESULTS: 71 patients completed the trial (IG: 36 patients, control group (CG) 35 patients). sl-wIRAR led to a significant pain reduction measured by a numeric rating scale (p < .0005) and in comparison, to the CG (p = .006). sl-wIRAR treatment resulted in a significant reduction in the Bath Anyklosing Spondylitis Disease Activity Index (BASDAI) (p = .004) and Bath Ankylosing Spondylitis Functional Index (p = .004) with no significant difference to the CG. TNF-α levels were significantly decreased (p = .001) only in the IG with a significant difference to the CG (p = .01). 26 (76%) of patients in the IC reduced their NSAID therapy after trial completion. CONCLUSION: sl-wIRAR treatment in axSpA leads to a rapid reduction in pain allowing NSAID dosage reduction. A reason for these desirable effects could be a change in TNFα levels.

11.
JMIR Mhealth Uhealth ; 8(7): e18117, 2020 07 20.
Artigo em Inglês | MEDLINE | ID: mdl-32390592

RESUMO

BACKGROUND: The use of patient-reported outcomes (PROs) allows for patient-centered, measurable, and transparent care. Electronic PROs (ePROs) have many benefits and hold great potential to improve current usage of PROs, yet limited evidence exists regarding their acceptance, usage, and barriers among rheumatologists. OBJECTIVE: This study aims to evaluate the current level of acceptance, usage, and barriers among German rheumatologists regarding the use of ePROs. The importance of different ePRO features for rheumatologists was investigated. Additionally, the most frequently used PROs for patients with rheumatoid arthritis (RA) were identified. METHODS: Data were collected via an online survey consisting of 18 questions. The survey was completed by members of the Working Group Young Rheumatology of the German Society for Rheumatology (Arbeitsgemeinschaft Junge Rheumatologie der Deutschen Gesellschaft für Rheumatologie [DGRh]) at the 2019 annual DGRh conference. Only members currently working in clinical adult rheumatology were eligible to complete the survey. RESULTS: A total of 119 rheumatologists completed the survey, of which 107 (89.9%) reported collecting PROs in routine practice and 28 (25.5%) already used ePROs. Additionally, 44% (43/97) were planning to switch to ePROs in the near future. The most commonly cited reason for not switching was the unawareness of suitable software solutions. Respondents were asked to rate the features of ePROs on a scale of 0 to 100 (0=unimportant, 100=important). The most important features were automatic score calculation and display (mean 77.50) and simple data transfer to medical reports (mean 76.90). When asked about PROs in RA, the respondents listed pain, morning stiffness, and patient global assessment as the most frequently used PROs. CONCLUSIONS: The potential of ePROs is widely seen and there is great interest in them. Despite this, only a minority of physicians use ePROs, and the main reason for not implementing them was cited as the unawareness of suitable software solutions. Developers, patients, and rheumatologists should work closely together to help realize the full potential of ePROs and ensure a seamless integration into clinical practice.

14.
Clin Rheumatol ; 39(5): 1513-1520, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-31916106

RESUMO

OBJECTIVES: Multimodal rheumatologic complex treatment (MRCT) is a specific concept of German inpatient care focusing on physical therapy for patients with rheumatic diseases suffering from exacerbated pain and functional impairment. As physical therapy is a key concept in the treatment of spondyloarthritis (SpA), we conducted a monocentric retrospective analysis of the effects of MRCT on pain and functional status in patients with SpA including patients with axial spondyloarthritis (axSpA), non-radiographic axial spondyloarthritis (nr-axSpA) and psoriatic arthritis with axial involvement (axPsA). METHODS: 134 treatment episodes provided to 100 patients with SpA between 2014 and 2017 were analysed. We evaluated changes in pain intensity, in functional status and in disease activity before and after a treatment episode. In addition, we assessed potential influences of various patient characteristics, the course of the disease and comorbidities. RESULTS: Overall, MRCT resulted in significant amelioration of pain (NRS: p < 0.001), significant improvement of functional capacity (FFbH: p = 0.03; HAQ: p = 0.02; BASFI: p < 0.001) and significant reduction of disease activity (BASDAI p < 0.001; DAS28: p = 0.009). In general, treatment effects on axSpA, nr-axSpA and axPsA were comparable. Different aspects of the disease and its previous course did not have a significant effect on the outcome parameters. Comorbidities (e.g. fibromyalgia) did not significantly influence treatment response. CONCLUSION: MRCT not only decreases pain and improves function but also reduces disease activity in patients with axSpA, nr-axSpA and axPsA irrespective of the course of disease and comorbidities (e.g. fibromyalgia), thus underlining the importance of non-pharmacological and physical treatment in the treatment of SpA.Key Points• Physical treatment is a key component in treating SpA.• Multimodal rheumatologic complex treatment (MRCT) is a specific concept of German inpatient care focusing on physical therapy for patients with rheumatic diseases suffering from exacerbated pain and functional impairment.• MRCT not only decreases pain and improves function but also reduces disease activity in patients with axSpA, nr-axSpA and axPsA irrespective of the course of disease and comorbidities (e.g. fibromyalgia).• MRCT could be a role model of treating SpA by means of physical therapy as its effects are not influenced by therapy, disease duration or comorbidities and as it has no side effects.

15.
J Bone Miner Metab ; 38(3): 378-384, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-31802224

RESUMO

INTRODUCTION: Adult lactose intolerance (ALI) significantly alters calcium intake and absorption, and thus may promote osteoporosis. ALI is a recessive condition with a geographical north-south gradient characterised by decreased levels of intestinal lactase. PCR-based genotyping of lactase (LCT) gene polymorphisms is a safe and easy way to diagnose ALI and may complement diagnostic procedures to identify individuals at risk for reduced calcium intake and subsequently osteoporosis due to lactose malabsorption. Therefore, we investigated the frequency of ALI and its influence on calcium intake, markers of bone metabolism and bone mineral density (BMD) in a cohort of Turkish immigrants living in Germany. MATERIALS AND METHODS: We investigated single nucleotide polymorphisms of the LCT gene, calcium intake, markers of bone metabolism and BMD in 183 Turkish immigrants. RESULTS: ALI was diagnosed in 154 out of 183 (81%) probands. ALI was significantly associated with self-reported lactose intolerance (p < 0.001) and dislike for dairy products (p < 0.01). Osteopenia was diagnosed in 59 out of 183 (32%) and osteoporosis in 15 out of 183 (8%) probands. Probands with reduced BMD had ALI in 86%. All probands had a decreased calcium intake [mg/week]. There was no significant association between ALI, calcium intake, markers of bone metabolism or BMD. CONCLUSION: Turkish immigrants mostly have ALI and overall show a reduced calcium intake per week. However, ALI did not significantly influence calcium intake, markers of bone metabolism or BMD in this cohort. Therefore, ALI in Turkish immigrants does not seem to be a risk factor for osteoporosis.


Assuntos
Densidade Óssea , Osso e Ossos/metabolismo , Cálcio na Dieta/farmacologia , Emigrantes e Imigrantes , Intolerância à Lactose/epidemiologia , Adulto , Biomarcadores/metabolismo , Índice de Massa Corporal , Densidade Óssea/genética , Osso e Ossos/efeitos dos fármacos , Estudos de Coortes , Feminino , Genótipo , Alemanha , Humanos , Intolerância à Lactose/genética , Masculino , Fosfatase Ácida Resistente a Tartarato/metabolismo , Turquia
17.
Immun Inflamm Dis ; 7(4): 271-275, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31502765

RESUMO

OBJECTIVE: The present study aimed to investigate progranulin autoantibodies in systemic sclerosis and autoimmune connective tissue disorders. Progranulin is a physiologic tumor necrosis factor (TNF) antagonist. Progranulin antibodies decrease progranulin levels. METHODS: Serum samples of 123 patients with systemic sclerosis and various autoimmune connective tissue disorders (Sjoegren's syndrome [SjS], mixed connective tissue disorder, polymyositis [PM] and dermatomyositis [DM], antiphospholipid syndrome [APLS], and undifferentiated connective tissue disease [UCTD]) were tested for progranulin antibodies using enzyme-linked immunosorbent assay. RESULTS: Progranulin antibodies were found in 34 of 123 (27.6%) patients at least once during their disease. In detail, 2 of 8 (25%) patients with limited cutaneous systemic sclerosis, 10 of 31 (32.3%) patients with diffuse cutaneous systemic sclerosis, 9 of 22 (40.9%) patients with SjS, 1 of 3 (33.33%) patients with mixed connective tissue disease, 4 of 33 (12.1%) patients with DM or PM, 6 of 15 (40%) patients with APLS, and 2 of 11 (18.2%) patients with UCTD were positive for progranulin antibodies during the course of disease. CONCLUSIONS: Progranulin antibodies are frequently present in patients with systemic sclerosis and other autoimmune connective tissue disorders. Despite the lack of specificity for a given autoimmune disease, progranulin antibodies might not only indicate a potential subtype but also play a pathogenic role in patients with autoimmune connective disorders. Given the important role of TNF-α in inflammatory processes in autoimmune connective tissue disorders, progranulin antibodies might support the proinflammatory environment by neutralizing the TNF blocker progranulin.


Assuntos
Autoanticorpos , Doenças Autoimunes , Progranulinas , Escleroderma Sistêmico , Idoso , Autoanticorpos/sangue , Autoanticorpos/imunologia , Doenças Autoimunes/sangue , Doenças Autoimunes/imunologia , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Progranulinas/sangue , Progranulinas/imunologia , Escleroderma Sistêmico/sangue , Escleroderma Sistêmico/imunologia
20.
J Autoimmun ; 61: 17-28, 2015 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-26005049

RESUMO

Recently we identified in a wide spectrum of autoimmune diseases frequently occurring proinflammatory autoantibodies directed against progranulin, a direct inhibitor of TNFR1 & 2 and of DR3. In the present study we investigated the mechanisms for the breakdown of self-tolerance against progranulin. Isoelectric focusing identified a second, differentially electrically charged progranulin isoform exclusively present in progranulin-antibody-positive patients. Alkaline phosphatase treatment revealed this additional progranulin isoform to be hyperphosphorylated. Subsequently Ser81, which is located within the epitope region of progranulin-antibodies, was identified as hyperphosphorylated serine residue by site directed mutagenesis of candidate phosphorylation sites. Hyperphosphorylated progranulin was detected exclusively in progranulin-antibody-positive patients during the courses of their diseases. The occurrence of hyperphosphorylated progranulin preceded seroconversions of progranulin-antibodies, indicating adaptive immune response. Utilizing panels of kinase and phosphatase inhibitors, PKCß1 was identified as the relevant kinase and PP1 as the relevant phosphatase for phosphorylation and dephosphorylation of Ser81. In contrast to normal progranulin, hyperphosphorylated progranulin interacted exclusively with inactivated (pThr320) PP1, suggesting inactivated PP1 to cause the detectable occurrence of phosphorylated Ser81 PGRN. Investigation of possible functional alterations of PGRN due to Ser81 phosphorylation revealed, that hyperphosphorylation prevents the interaction and thus direct inhibition of TNFR1, TNFR2 and DR3, representing an additional direct proinflammatory effect. Finally phosphorylation of Ser81 PGRN alters the conversion pattern of PGRN. In conclusion, inactivated PP1 induces hyperphosphorylation of progranulin in a wide spectrum of autoimmune diseases. This hyperphosphorylation prevents direct inhibition of TNFR1, TNFR2 and DR3 by PGRN, alters the conversion of PGRN, and is strongly associated with the occurrence of neutralizing, proinflammatory PGRN-antibodies, indicating immunogenicity of this alternative secondary modification.


Assuntos
Autoanticorpos/imunologia , Peptídeos e Proteínas de Sinalização Intercelular/imunologia , Precursores de Proteínas/imunologia , Serina/imunologia , Animais , Autoanticorpos/genética , Autoanticorpos/metabolismo , Sítios de Ligação/genética , Western Blotting , Linhagem Celular , Linhagem Celular Tumoral , Citometria de Fluxo , Células HEK293 , Humanos , Peptídeos e Proteínas de Sinalização Intercelular/genética , Peptídeos e Proteínas de Sinalização Intercelular/metabolismo , Mutagênese Sítio-Dirigida , Fosforilação , Progranulinas , Isoformas de Proteínas/genética , Isoformas de Proteínas/imunologia , Isoformas de Proteínas/metabolismo , Proteína Quinase C beta/genética , Proteína Quinase C beta/imunologia , Proteína Quinase C beta/metabolismo , Precursores de Proteínas/genética , Precursores de Proteínas/metabolismo , Membro 25 de Receptores de Fatores de Necrose Tumoral/imunologia , Membro 25 de Receptores de Fatores de Necrose Tumoral/metabolismo , Receptores Tipo I de Fatores de Necrose Tumoral/imunologia , Receptores Tipo I de Fatores de Necrose Tumoral/metabolismo , Receptores Tipo II do Fator de Necrose Tumoral/imunologia , Receptores Tipo II do Fator de Necrose Tumoral/metabolismo , Serina/genética , Serina/metabolismo
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