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In the last decade, nailfold capillaroscopy is finding its way to the daily clinic of (pediatric) rheumatologist. This review will provide the necessary knowledge for the clinician performing this easy and non-invasive examination in children. In the first part, background information on type of capillaroscopy device and standardized (internationally validated) interpretations for the different capillary variables compared to healthy pediatric controls will be provided. The second part focusses on capillary changes that are observed in Raynaud's phenomenon with follow-up recommendations. This part will also cover capillaroscopy findings in juvenile systemic sclerosis, childhood-onset systemic lupus erythematosus, juvenile dermatomyositis and -mixed connective tissue disease, as well as correlations with disease severity. Lastly, a research agenda shows the current gaps we have in knowledge in this niche of nailfold capillaroscopy in pediatric connective tissue diseases.
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The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present consensus provides an update to the 2017 European Dermatology Forum Guidelines, focusing on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, updated strategies for the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 1 of this consensus provides clinicians with an overview of the diagnosis and treatment of localized scleroderma (morphea), and systemic sclerosis including overlap syndromes.
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Consenso , Esclerodermia Localizada , Escleroderma Sistêmico , Humanos , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/terapia , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/terapia , Diagnóstico DiferencialRESUMO
The engineered Hand Test System (HTS) glove has shown high reliability in assessing the baseline functional status of rheumatoid arthritis (RA) hand. Starting from this achievement, the aim of the present observational prospective study was to assess the functionality of the single fingers of rheumatoid hand at follow-up. Eighty RA patients performed HTS glove tests at baseline and among these fifty-six patients were re-tested after 7 months. The HTS glove parameters [Touch Duration (TD), Movement Rate (MR), Inter Tapping Interval (ITI)] were correlated with disease activity and disability clinimetric indexes [Disease Activity Score 28 joint count-C-reactive protein (DAS28-CRP), Clinical Disease Activity Index (CDAI), Simplified Disease Activity Index (SDAI), Health Assessment Questionnaire-Disability Index (HAQ-DI), grip strength, visual analogue scale of pain (VAS), patient global assessment (PGA)], and with laboratory values. HTS glove parameters (TD, ITI, and MR) showed statistically significant correlations with clinimetric and clinical indexes at both time points (p < 0.05). During follow-up, a statistically significant variation of all HTS glove parameters for the fingers that have performed both the worst or best HTS test at baseline was detected (p < 0.05), while the mean HTS glove parameter values by considering all fingers did not show a statistically significant variation over time, as well as the traditional clinimetric indexes. Besides the objective role in assessing the RA hand function by integrating the traditional clinimetric indexes, the HTS glove seems a useful tool for evaluating worst or best finger function during time by measuring the movement speed.
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Artrite Reumatoide , Humanos , Estudos Prospectivos , Reprodutibilidade dos Testes , Artrite Reumatoide/diagnóstico , Proteína C-Reativa , Dor , Índice de Gravidade de DoençaRESUMO
The SARS-CoV-2 infection causing the Coronavirus disease-19 (COVID-19) is characterized by a broad range of clinical manifestations, implicating microvascular damage with endothelial dysfunction and different organ involvement.
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COVID-19 , Unhas , Humanos , Unhas/irrigação sanguínea , Capilares , SARS-CoV-2 , SobreviventesRESUMO
This cross-sectional online study was designed by the study group on Capillaroscopy and Microcirculation in Rheumatic Diseases (CAP) of the Italian Society of Rheumatology (SIR) to provide an overview of the management of nailfold capillaroscopy in Italian rheumatology centers. Therefore, SIR distributed the survey to its members in July 2021, and each center's physician with the most expertise in capillaroscopy completed the questionnaire. The survey was completed by 102 centers, with at least one representative from each Italian region. Ninety-three centers perform capillaroscopy, and 52 (56) conduct more than 200 investigations annually. Seventy-eight (84%) of respondents have more than five years of experience with the technique, and 75 centers (80.6%) have received certification from specific national or international training courses. In 85 centers, a videocapillaroscope with 200x magnification is employed (91.4%). The average waiting period for the examination is 2.4 months, and less than 3 months in 64 of the locations (68.8%). The study demonstrates that capillaroscopy is an integral part of both the diagnostic phase of Raynaud's phenomenon and the monitoring of autoimmune connective tissue diseases (CTDs). However, the reporting methods and timing of patient followup are heterogeneous.
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Doença de Raynaud , Doenças Reumáticas , Reumatologia , Humanos , Angioscopia Microscópica/métodos , Estudos Transversais , Doenças Reumáticas/diagnóstico por imagem , Doença de Raynaud/diagnóstico por imagem , Itália , Unhas/diagnóstico por imagemRESUMO
BACKGROUND: The GLORIA placebo-controlled trial found a favorable balance of benefit and harm for two years of prednisolone (5 mg/day) as add-on treatment for rheumatoid arthritis (RA) patients aged 65+. This study evaluated the cost-effectiveness of low-dose prednisolone in the treatment of RA. METHODS: The economic evaluation had a societal perspective with a time horizon of two years. Cost data were collected with questionnaires and from recorded events, and valued with standard Dutch unit prices of 2017. The primary effectiveness outcome was the disease activity score in 28 joints (DAS28). For cost-utility, quality-adjusted life years (QALYs) were estimated from the EuroQol-5 Dimension (EQ-5D) questionnaire. Bootstrapping assessed the uncertainty around the average differences in costs and health outcomes. RESULTS: In total, 444 of 451 randomized patients were included in the modified intention-to-treat analysis. Patients had median four active comorbidities at baseline. Mean total costs over two years were k10.8 in the prednisolone group, k0.5 (95% CI -4.0; 1.8) lower than in the placebo group. Total direct medical costs were k0.5 (95% CI -4.0; 1.5) lower in the prednisolone group. The mean number of QALYs was similar in both groups (difference 0.02 [-0.03; 0.06] in favor of prednisolone). The DAS28 was 0.38 lower in the prednisolone group than in the placebo group (0.19; 0.56). CONCLUSION: With greater effectiveness (DAS28) at non-significantly lower costs, low-dose, add-on prednisolone is cost-effective for RA compared to placebo over two years. QALYs were equal in both groups, most likely due to the impact of multiple comorbidities.
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Artrite Reumatoide , Prednisolona , Humanos , Prednisolona/uso terapêutico , Análise Custo-Benefício , Artrite Reumatoide/tratamento farmacológico , Anos de Vida Ajustados por Qualidade de Vida , EtnicidadeRESUMO
Giant cell arteritis (GCA) is the most common form of vasculitis of the adult. General practitioners (GPs) are usually the first physicians who take care of GCA patients. In this study, the awareness of GPs from Genoa, Italy, regarding GCA was investigated by a web-based survey. A web-based questionnaire was sent by mail to 775 Italian GPs. It included 12 multiple choice questions regarding practice seniority, practice population size, number of GCA patients followed, and GPs' diagnostic and therapeutic approach. Of the 775 GPs involved, 76 (9.8%) answered. Thirty-three/75 (44%) declared that they did not see patients with GCA and the remaining 42 (56%) that they diagnose between one and two patients per year. New headache onset was the presenting feature of GCA for the majority of GPs (78.3%). GCA was diagnosed on the basis of clinical presentation alone by 35.2% of them, of temporal artery biopsy by 49.3%, and by imaging, including ultrasound and magnetic resonance imaging, by 15.5%. The referral pattern was mainly toward rheumatologists (68.5%). Only 27.8% GPs declared they start treatment at the first clinical suspicion, with the others waiting for laboratory and imaging examinations or specialist consultation. The doses of glucocorticoids used were in keeping with current guidelines. The management of GCA by GPs from Genoa is in general correct, with the exceptions of excessive confidence in headaches for diagnosis and of the timing of GC initiation. These points suggest that a program of information and education for GPs is warranted.
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Clínicos Gerais , Arterite de Células Gigantes , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/tratamento farmacológico , Arterite de Células Gigantes/epidemiologia , Humanos , Internet , Itália/epidemiologia , Inquéritos e Questionários , Artérias TemporaisRESUMO
OBJECTIVE: To evaluate the current perspectives of patients and health professionals regarding the efficacy and safety of low-dose glucocorticoids (GCs) in RA. METHODS: Two online surveys were disseminated to patients and health professionals, in their native language, through national patient organizations and national rheumatology medical societies, respectively. SurveyMonkey®, MediGuard.org and the Glucocorticoid Low-dose Outcome in RA Study (GLORIA) website were used to offer and deliver these surveys. RESULTS: A total of 1221 RA patients with exposure to GCs, and 414 rheumatologists completed the surveys. Patients and rheumatologists reported high levels of agreement regarding the efficacy of low-dose GCs: at least 70% considered that they are very rapid and effective in the control of signs and symptoms of RA. However, half of the patients also reported having suffered serious adverse events with GCs, and 83% described concerns about safety. The majority of rheumatologists estimated that endocrine, ophthalmologic and cutaneous adverse events affect >4% of all patients treated with low-dose GCs for 2 years, based on a heat map. CONCLUSIONS: RA patients with self-reported exposure to GCs express high levels of satisfaction with low-dose GCs efficacy, as do rheumatologists. However, both expressed excessive concerns regarding the safety of GCs (greatly exceeding the published evidence data), which may compromise the optimal use of this medication. This study indicates that there is an unmet need for appropriately designed prospective trials that shed light on the real risk associated with low-dose GCs, as well as a need for renovated educational programs on the real benefits and harms of low-dose GCs, for both patients and physicians.
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Artrite Reumatoide/tratamento farmacológico , Atitude do Pessoal de Saúde , Atitude Frente a Saúde , Glucocorticoides/administração & dosagem , Adulto , Idoso , Artrite Reumatoide/fisiopatologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Inquéritos e QuestionáriosRESUMO
AIM: To evaluate, in Systemic sclerosis (SSc) patients, the body composition and the bone status according to the peripheral microcirculatory condition, assessed and scored by nailfold videocapillaroscopy (NVC, "Early", "Active", "Late" patterns). METHODS: Body composition and bone mineral density (BMD) were assessed by Dual X-ray absorptiometry and dedicated software (GE Lunar USA) in 37 female SSc patients classified according to the 2013 EULAR/ACR criteria and 40 sex-matched healthy subjects. Clinical, laboratory, body composition and bone parameters were analyzed according to the different NVC patterns. Means were compared by the Student's t test or one-way analysis of variance; medians were compared by the Kruskal-Wallis test; and frequencies by the chi-square test. RESULTS: Higher prevalence of vertebral (21% vs 7%) and femoral (35% vs 7%) osteoporosis (OP) was found in SSc. Particularly SSc patients with "Late" NVC pattern showed a significantly higher prevalence of vertebral (p = 0.018) and femoral OP (p = 0.016). Regional assessment of bone mass (BM) in seven different body areas showed a significantly lower BMD only at the total spine (p = 0.008) and femoral neck (p = 0.027) in advanced microvascular damage. Patients with "Late" NVC pattern showed a lower whole-body lean mass (LM) compared to "Early" and "Active" NVC patterns, particularly at upper limbs. To note, in all body sites, BMD correlates with LM and BMC according to NVC pattern severity. CONCLUSIONS: SSc patients with most severe microvascular damage show a significantly altered body composition and bone status suggesting a strong link between microvascular failure and associated muscle/bone sufferance.
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Doenças Ósseas Metabólicas/patologia , Microcirculação , Osteoporose/patologia , Escleroderma Sistêmico/complicações , Idoso , Composição Corporal , Doenças Ósseas Metabólicas/epidemiologia , Doenças Ósseas Metabólicas/etiologia , Estudos de Casos e Controles , Feminino , Seguimentos , Humanos , Itália/epidemiologia , Masculino , Pessoa de Meia-Idade , Osteoporose/epidemiologia , Osteoporose/etiologia , Prognóstico , Estudos RetrospectivosRESUMO
OBJECTIVES: Studies on the seasonality of onset of polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) have shown conflicting results. The aim of this systematic literature review and meta-analysis is to determine from aggregated data whether there is a seasonal distribution for these diseases. METHODS: A literature search was performed using Pubmed Central and Embase scientific databases. The incidences per 6-month periods, season or month of onset, that were reported in the studies were summarised in tables considering the two diseases as separate conditions or together. The Incidence Rate Ratio (IRR) for the cold period versus the warm period was pooled across studies by random effects meta-analysis weighed by inverse variance. Funnel plots and Egger test were used to explore possible publication biases. A sensitivity analysis was performed to weigh articles with a disproportionate number of patients compared to the rest. RESULTS: In the scientific literature 22 suitable papers were found: 6 on PMR with 803 patients, 11 on GCA with 2,807 patients, and 5 studies considering both diseases with 19,613 patients. There was considerable heterogeneity amongst studies regarding their quality, the classification criteria used, and the definition of onset of symptoms. No seasonal aggregation was found for GCA and PMR. The pooled IRR estimate of the meta-analysis (1.13[0.89,1.36]) showed a non-significant, higher frequency of diseases onset in the warm season. CONCLUSIONS: Our meta-analysis did not confirm a seasonal onset for PMR and GCA.
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Arterite de Células Gigantes , Polimialgia Reumática , Arterite de Células Gigantes/epidemiologia , Humanos , Incidência , Polimialgia Reumática/epidemiologia , Estações do AnoRESUMO
Osteoarthritis (OA) is the most common joint condition and, with a burgeoning ageing population, is due to increase in prevalence. Beyond conventional medical and surgical interventions, there are an increasing number of 'alternative' therapies. These alternative therapies may have a limited evidence base and, for this reason, are often only afforded brief reference (or completely excluded) from current OA guidelines. Thus, the aim of this review was to synthesize the current evidence regarding autologous chondrocyte implantation (ACI), mesenchymal stem cell (MSC) therapy, platelet-rich plasma (PRP), vitamin D and other alternative therapies. The majority of studies were in knee OA or chondral defects. Matrix-assisted ACI has demonstrated exceedingly limited, symptomatic improvements in the treatment of cartilage defects of the knee and is not supported for the treatment of knee OA. There is some evidence to suggest symptomatic improvement with MSC injection in knee OA, with the suggestion of minimal structural improvement demonstrated on MRI and there are positive signals that PRP may also lead to symptomatic improvement, though variation in preparation makes inter-study comparison difficult. There is variability in findings with vitamin D supplementation in OA, and the only recommendation which can be made, at this time, is for replacement when vitamin D is deplete. Other alternative therapies reviewed have some evidence (though from small, poor-quality studies) to support improvement in symptoms and again there is often a wide variation in dosage and regimens. For all these therapeutic modalities, although controlled studies have been undertaken to evaluate effectiveness in OA, these have often been of small size, limited statistical power, uncertain blindness and using various methodologies. These deficiencies must leave the question as to whether they have been validated as effective therapies in OA (or chondral defects). The conclusions of this review are that all alternative interventions definitely require clinical trials with robust methodology, to assess their efficacy and safety in the treatment of OA beyond contextual and placebo effects.
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Terapias Complementares/métodos , Osteoartrite do Joelho/terapia , Fatores Etários , Condrócitos/transplante , Feminino , Humanos , Masculino , Transplante de Células-Tronco Mesenquimais/métodos , Transplante Autólogo/métodos , Resultado do Tratamento , Vitamina D/uso terapêutico , Vitaminas/uso terapêuticoRESUMO
OBJECTIVE: The aim of this observational study was to investigate the evolution of scleroderma microangiopathy throughout different nailfold videocapillaroscopy (NVC) patterns ('early', 'active', 'late') as well as the prevalence of organ involvement in SSc patients during a 12-year follow-up. METHODS: Thirty-four SSc patients showing at baseline (first capillaroscopic analysis) the 'early' NVC pattern of microangiopathy were enrolled and followed for 12 years (s.d. 2). Complete NVC analysis and clinical and serological findings were collected. Patients were in a standard therapeutic care setting. Statistical analysis was carried out by non-parametric tests. RESULTS: After a 12-year follow-up, the 'early' NVC pattern changed from baseline in 76% of the patients. The NVC pattern was found to be 'active' in 9 patients (26%), 'late' in 13 (38%) and characterized by non-specific capillary abnormalities in 4 (12%). In the subgroup whose microangiopathy progressed from the 'early' to the 'late' NVC pattern, the median time of progression from the 'early' to the 'active' pattern was significantly shorter (11 months) when compared with patients who progressed from the 'early' to the 'active' NVC pattern (55 months) (P = 0.002). The median time of progression between NVC patterns was significantly shorter in SSc patients showing either a nucleolar ANA pattern or Scl70 autoantibodies (P = 0.048). Organ involvement was progressively greater in SSc patients with 'early', 'active' and 'late' NVC patterns, respectively. CONCLUSIONS: This longitudinal study confirms over a 12-year follow-up the evolution of specific NVC patterns associated with the progressive severity of organ involvement in SSc patients in a standard clinical care setting.
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Progressão da Doença , Angioscopia Microscópica/métodos , Doenças da Unha/diagnóstico por imagem , Unhas/irrigação sanguínea , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/imunologia , Autoanticorpos/imunologia , Feminino , Seguimentos , Humanos , Estudos Longitudinais , Masculino , Doenças da Unha/etiologia , Estudos Retrospectivos , Medição de Risco , Escleroderma Sistêmico/diagnóstico , Índice de Gravidade de Doença , Fatores de TempoRESUMO
Patellar tendinopathy, or Jumper's knee, is a painful knee condition caused by inflammation of the patella tendon. This condition is most frequently observed in subjects who play sports that require repetitive regular jumping. Jumper's knee is frequently misdiagnosed as a minor injury and many athletes, like our patient, keep on training and competing and either tend to ignore the injury or attempt to treat it themselves. However, jumper's knee is a serious condition that requires a correct and timely diagnosis, which often necessitates ultrasound investigation in order to start the most appropriate treatment.
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Traumatismos em Atletas/diagnóstico por imagem , Ligamento Patelar/diagnóstico por imagem , Tendinopatia/diagnóstico por imagem , Voleibol , Adulto , Traumatismos em Atletas/complicações , Humanos , Masculino , Tendinopatia/etiologia , Ultrassonografia/métodosRESUMO
OBJECTIVE: The aim of this study was to identify any correlations between microvascular damage, assessed by nailfold videocapillaroscopy and skin impairment, evaluated by three different methods, the modified Rodnan skin score (mRSS), skin high-frequency ultrasound (US) and the plicometer skin test (PST) in systemic sclerosis (SSc) patients. METHODS: Sixty-three SSc patients and 63 healthy subjects were enrolled. Nailfold videocapillaroscopy (NVC) was used to assess the nailfold capillaroscopy pattern ("Early", "Active" or "Late"), according to the Cutolo classification. All subjects were assessed by mRSS, US and PST to evaluate their dermal thickness (DT) in the seventeen skin areas of the body usually evaluated by mRSS (zygoma, fingers, hands, dorsum of hands, forearms, arms, chest, abdomen, thighs, legs, feet). Statistical evaluation was performed by nonparametric tests. RESULTS: All the three methods demonstrated progressively higher values of skin impairment in patients with "Early", "Active" or "Late" pattern of nailfold microangiopathy (for mRSS pâ¯<â¯0.01, US pâ¯<â¯0.02 and PST pâ¯<â¯0.02). A positive correlation was also observed in SSc patients between the three methods used to evaluate skin involvement (mRSS vs US, mRSS vs PST, PST vs US, pâ¯<â¯0.0001 respectively). CONCLUSIONS: This study demonstrates that there is a correlation between two of the most important aspects to classify and monitor the SSc patients, i.e. microvascular damage progression (evaluated by NVC) and skin damage (assessed by mRss, US and PST).
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Capilares/patologia , Angioscopia Microscópica , Unhas/irrigação sanguínea , Escleroderma Sistêmico/patologia , Pele/irrigação sanguínea , Pele/patologia , Idoso , Idoso de 80 Anos ou mais , Estudos de Casos e Controles , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Variações Dependentes do Observador , Valor Preditivo dos Testes , Prognóstico , Reprodutibilidade dos Testes , Escleroderma Sistêmico/diagnóstico por imagem , Índice de Gravidade de Doença , UltrassonografiaRESUMO
Hashimoto's encephalopathy (HE) is an autoimmune form of encephalopathy, associated with autoimmune thyroiditis. Its prevalence is estimated to be 2:100,000. HE is characterized by behavioral changes, mental confusion, dysarthria, ataxia, psychosis, paranoia, convulsions, hallucinations, headache and hyperthermia. Elevated thyroid antibodies are necessary for diagnosis and the disease responds dramatically to glucocorticoid therapy. We describe a patient with HE and panniculitis, an association reported twice in the literature.
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Encefalite/complicações , Doença de Hashimoto/complicações , Paniculite/complicações , Idoso , Humanos , MasculinoRESUMO
To identify nailfold videocapillaroscopy (NVC) changes in patients with dermatomyositis (DM) during a 3-year follow-up and to compare the NVC findings between DM and systemic sclerosis (SSc) patients at their first visit. Retrospective study of 24 DM and 24 SSc patients, matched for age and disease duration at first NVC. Capillaroscopic patterns/scores and clinical parameters had been yearly assessed. Nineteen out of 24 DM patients (79%) showed a NVC "scleroderma-like pattern." No statistically significant variation of all the capillaroscopic scores was observed during the 3-year follow-up. By comparing DM patients with or without anti-Jo-1 positivity, no statistically significant difference of the scores of the main capillary parameters was observed at baseline between the groups. Comparing at baseline DM with SSc patients, the giant capillary and microhemorrhage scores were significantly higher in SSc than those in DM patients (p = 0.04 and p = 0.05, respectively), while capillary density, ramification (abnormally shaped capillaries, expression of angiogenesis), and disorganization scores were higher in DM patients (p = 0.05, p = 0.002, p = 0.004, respectively). The absolute number of ramified capillaries was significantly higher in DM patients (p = 0.002), while the absolute capillary number was significantly higher in SSc patients (p = 0.05) at baseline. This pilot study demonstrates, for the first time, over long-term, that the capillaroscopic manifestations of DM persist in contrast to the progressive changes described in SSc patients, and the anti-Jo-1 positivity does not seem to modify the NVC pattern.
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Capilares/diagnóstico por imagem , Dermatomiosite/diagnóstico por imagem , Unhas/irrigação sanguínea , Escleroderma Sistêmico/diagnóstico por imagem , Adulto , Idoso , Progressão da Doença , Feminino , Seguimentos , Humanos , Masculino , Angioscopia Microscópica , Pessoa de Meia-Idade , Unhas/diagnóstico por imagem , Estudos RetrospectivosRESUMO
OBJECTIVES: Iloprost plays an important role in the treatment of Raynaud's phenomenon (RP), but has transient vasodilatory effects owing to its very short half-time. We aimed to evaluate short- and medium-term haemodynamic effects of iloprost by measuring dorsal finger microvessel blood flow using laser Doppler flowmetry (LDF), in patients with RP associated with systemic sclerosis (SSc). METHOD: In 24 consecutive SSc patients with RP (disease duration 10.5 ± 1.3 years), LDF with heating probes was used to measure blood flow in four fingers by occlusive and heating tests, at baseline, after 3 consecutive days of iloprost infusion, and at 24 h and 7 days after last iloprost infusion. Nailfold videocapillaroscopy (NVC) patterns of microvascular damage were investigated. Sixteen healthy controls were studied to compare baseline flows. RESULTS: Compared to controls, SSc patients showed significantly impaired axon reflex vasoregulation and nitric oxide responses at baseline (p = 0.001 and p = 0.03, respectively). After iloprost, a prompt but transient significant improvement in endothelial-dependent vasodilation (occlusive test) was seen only in SSc patients with an 'active' NVC pattern (p ≤ 0.05). The iloprost effects vanished within 7 days after the last infusion. No significant differences were found, in the whole study, between patients with and without digital ulcers. CONCLUSIONS: Microcirculatory blood flow increases following 3 days of iloprost infusion but fades shortly after treatment. Although iloprost is effective in reducing the severity of RP in SSc, the most suitable regimen and timing to obtain longer lasting vasodilatory benefits remain to be established.
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Dedos/irrigação sanguínea , Iloprosta/farmacologia , Microcirculação/efeitos dos fármacos , Fluxo Sanguíneo Regional/efeitos dos fármacos , Escleroderma Sistêmico/tratamento farmacológico , Vasodilatação/efeitos dos fármacos , Vasodilatadores/farmacologia , Adulto , Idoso , Estudos de Casos e Controles , Feminino , Dedos/diagnóstico por imagem , Humanos , Iloprosta/uso terapêutico , Infusões Intravenosas , Fluxometria por Laser-Doppler , Estudos Longitudinais , Masculino , Angioscopia Microscópica , Pessoa de Meia-Idade , Escleroderma Sistêmico/fisiopatologia , Vasodilatadores/uso terapêuticoRESUMO
OBJECTIVE: To identify possible correlations between skin blood perfusion (BP) and dermal thickness (DT) in different skin areas of systemic sclerosis (SSc) patients. METHODS: Sixty-two SSc patients, according to 2013 EULAR/ACR criteria, and 62 healthy subjects (CNT) were enrolled. Skin BP was analysed by laser speckle contrast analysis (LASCA) at the level of dorsum of the middle phalanx of the third fingers, dorsal aspect of the hands and zygoma. DT was assessed by both skin high frequency ultrasound (US) and modified Rodnan skin score (mRSS) in the same above reported areas. All patients were studied also by nailfold videocapillaroscopy (NVC) to assess the proper pattern of microvascular damage ("Early", "Active", or "Late"). RESULTS: At the level of finger dorsum a statistically significant negative correlation was observed in SSc patients between skin BP and both ultrasound-DT (p=0.0005 r=0.43) and mRSS (p=0.0007 r=0.42), but not at the level of hand dorsum and zygoma. No statistically significant correlation was present between skin BP and ultrasound-DT at any level in CNT. In detail, SSc patients, compared to CNT, showed a statistically significant lower BP only at level of fingers (median PU 72.6 vs 136.1 respectively, p<0.0001) and a statistically significant higher ultrasound-DT at the level of dorsum of 3th finger bilaterally (median mm 0.9 vs 0.7, p<0.0001), dorsum of hands (median mm 0.9 vs 0.7, p<0.0001) and zygoma (median mm 0.8 vs 0.7, p<0.0001). A significant positive correlation between ultrasound-DT and mRSS was observed in SSc patients at level of the three areas (dorsum of fingers p<0.0001 r=0.51; dorsum of hands p=0.03 r=0.27; zygoma p=0.0001 r=0.45). A progressive decrease of skin BP and increase of ultrasound-DT was found correlated with the progression of the severity of NVC patterns. CONCLUSIONS: This study demonstrates for the first time in SSc patients a significant inverse relationship between skin BP, measured by LASCA, and DT, evaluated by both US and mRSS, at the level of dorsum of the middle phalanx of the third fingers.
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Microcirculação , Escleroderma Sistêmico/fisiopatologia , Pele/irrigação sanguínea , Idoso , Velocidade do Fluxo Sanguíneo , Estudos de Casos e Controles , Feminino , Humanos , Fluxometria por Laser-Doppler , Masculino , Angioscopia Microscópica , Pessoa de Meia-Idade , Imagem de Perfusão/métodos , Fluxo Sanguíneo Regional , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/patologia , Índice de Gravidade de Doença , Pele/patologia , Fatores de Tempo , UltrassonografiaRESUMO
OBJECTIVES: Systemic sclerosis (SSc) is heterogenous. The objectives of this study were to evaluate the purpose, strengths and limitations of existing SSc subset criteria, and identify ideas among experts about subsets. METHODS: We conducted semi-structured interviews with randomly sampled international SSc experts. The interview transcripts underwent an iterative process with text deconstructed to single thought units until a saturated conceptual framework with coding was achieved and respondent occurrence tabulated. Serial cross-referential analyses of clusters were developed. RESULTS: Thirty experts from 13 countries were included; 67% were male, 63% were from Europe and 37% from North America; median experience of 22.5 years, with a median of 55 new SSc patients annually. Three thematic clusters regarding subsetting were identified: research and communication; management; and prognosis (prediction of internal organ involvement, survival). The strength of the limited/diffuse system was its ease of use, however 10% stated this system had marginal value. Shortcomings of the diffuse/limited classification were the risk of misclassification, predictions/generalizations did not always hold true, and that the elbow or knee threshold was arbitrary. Eighty-seven percent use more than 2 subsets including: SSc sine scleroderma, overlap conditions, antibody-determined subsets, speed of progression, and age of onset (juvenile, elderly). CONCLUSIONS: We have synthesized an international view of the construct of SSc subsets in the modern era. We found a number of factors underlying the construct of SSc subsets. Considerations for the next phase include rate of change and hierarchal clustering (e.g. limited/diffuse, then by antibodies).