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OBJECTIVE: To assess the diagnostic value for GCA in adding the axillary arteries (AX) to the temporal artery (TA) ultrasound, particularly in patients with a cranial phenotype of the disease; and to investigate the utility of facial (FA), occipital (OC), subclavian (SC), and common carotid (CC) ultrasound in patients with suspected GCA. METHODS: Patients with new-onset GCA and a positive ultrasound of the TA, AX, FA, OC, SC or CC, followed at the rheumatology departments of two academic centres, were retrospectively included. RESULTS: 230 patients were assessed. TA halo sign was identified in 206/230 (89.6%) cases, FA in 40/82 (48.8%), OC in 17/69 (24.6%), AX in 56/230 (24.3%), SC in 31/57 (54.4%), and CC in 14/68 (20.6%). Negative TA ultrasound was found in 24/230 (10.4%) patients: 22 had AX involvement, 1 exclusive OC involvement and 1 exclusive SC involvement. Adding AX evaluation to the TA ultrasound increased the diagnostic yield for GCA in 9.6%, whereas adding OC or SCs to the TA and AX ultrasound increased it in 1.4% and 1.8%, respectively. No value was found in adding the FA or CCs. Notably, 13 patients with cranial symptoms and 4 with exclusively cranial symptoms showed negative TA ultrasound but positive AX ultrasound. CONCLUSION: Adding the evaluation of AXs to the TA ultrasound increased the number of patients diagnosed with GCA, even in cases of predominantly cranial symptoms. In the subset of patients where these arteries were assessed, no substantial benefit was found in adding the FA, OC, SC or CC arteries to the TA and AX ultrasonographic assessment.
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Stress fractures are common in young and active individuals, associated with aggressive or repetitive physical activity and their early detection is fundamental to optimise patient care, decrease complications and avoid unnecessary exams. Currently, magnetic resonance imaging is the standard of care for detecting these lesions. Recently, ultrasound has been getting an increasing interest for the detection of stress fractures. In this article, we describe a clinical case that involved a second metatarsal stress fracture diagnosed by ultrasound and review the literature regarding the use of ultrasound in the diagnosis of stress fractures, particularly of the metatarsals.
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Doenças Ósseas , Fraturas de Estresse , Ossos do Metatarso , Humanos , Fraturas de Estresse/diagnóstico , Ossos do Metatarso/diagnóstico por imagem , Doenças Ósseas/complicações , Imageamento por Ressonância Magnética/efeitos adversos , Diagnóstico PrecoceRESUMO
BACKGROUND: Constrictive pericarditis represents a chronic condition and systemic inflammatory diseases are a known, yet uncommon, cause. Pericardial involvement is seldom reported in primary Sjögren's syndrome, usually occurring in association with pericardial effusion or pericarditis. We report a case of constrictive pericarditis with an insidious course and unusual evolution associated with primary Sjögren's syndrome. Due to the challenging nature of the diagnosis, clinical suspicion and multimodality imaging are essential for early identification and prompt initiation of treatment. Long-term outcomes remain uncertain. To the best of our knowledge, no other cases linking this autoimmune disease to constrictive pericarditis have been reported. CASE PRESENTATION: We present the case of a 48-year-old male patient with moderate alcohol habits and a history of two prior hospitalizations. On the first, the patient was diagnosed with primary Sjögren's syndrome after presenting with pleural effusion and ascites, and empirical corticosteroid regiment was initiated. On the second, two-years later, he was readmitted with complaints of dyspnea and abdominal distension. Thoracic computed tomography revealed a localized pericardial thickening and a thin pericardial effusion, both of which were attributed to his rheumatic disease. A liver biopsy showed hepatic peliosis, which was considered to be a consequence of glucocorticoid therapy. Diuretic therapy was adjusted to symptom-relief, and a tapering corticosteroid regimen was adopted. Four years after the initial diagnosis, the patient was admitted again with recurrent dyspnea, orthopnea and ascites. At this time, constrictive pericarditis was diagnosed and a partial pericardiectomy was performed. Although not completely asymptomatic, the patient reported clinical improvement since the surgery, but still with a need for baseline diuretic therapy. CONCLUSION: Albeit uncommon, connective tissue disorders, such as primary Sjögren's syndrome, should be considered as a potential cause of constrictive pericarditis, especially in young patients with no other classical risk factors for constriction. In this case, after excluding possible infectious, neoplastic and autoimmune conditions, a primary Sjögren´s syndrome in association with constrictive pericarditis was assumed. This case presents an interesting and challenging clinical scenario, highlighting the importance of clinical awareness and the use of multimodal cardiac imaging for early recognition and treatment.
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Doenças Autoimunes , Derrame Pericárdico , Pericardite Constritiva , Síndrome de Sjogren , Masculino , Humanos , Pessoa de Meia-Idade , Pericardite Constritiva/diagnóstico por imagem , Pericardite Constritiva/etiologia , Pericardite Constritiva/cirurgia , Ascite , Derrame Pericárdico/diagnóstico por imagem , Derrame Pericárdico/etiologia , Derrame Pericárdico/terapia , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnóstico , Pericárdio , DiuréticosRESUMO
OBJECTIVE: To compare the 2-year retention rate between a second tumor necrosis factor alpha inhibitor (TNFi) and secukinumab (SEK) or ustekinumab (UST), in Psoriatic Arthritis (PsA) patients with previous inadequate response to their first TNFi. METHODS: Prospective longitudinal cohort study with a follow-up period of 2 years using the Nationwide Portuguese Reuma.pt database. Patients with a clinical diagnosis of PsA who also fulfill the CASPAR classification criteria, with previous treatment failure to a first-line TNFi and having started a second biotechnological drug (TNFi, SEK or UST) were included. The Cycling group was defined as switching from a first TNFi to a second TNFi, and the Swapping group as switching from a first TNFi to SEK or UST. Sociodemographic data, disease characteristics, disease activity scores and physical function at baseline and after 6, 12 and 24 months were recorded. Cox-proportional hazards regression was used to compare retention rates between Cycling and Swapping groups. To obtain a predictor model of 2-year discontinuation, a multivariable Cox regression model was performed. RESULTS: In total, 439 patients were included, 58% were female, with a mean age (standard deviation) of 49 (12) years. Globally, 75.6% initiated a second TNFi (Cycling group), and 24.4% started SEK/UST (Swapping group). The retention rates after 6, 12 and 24 months were 72%/66%/59% in the Cycling group; and 77%/66%/59% in the Swapping group. There were no significant differences in retention rates between both strategies (HR: 1.06, 95% CI 0.72-1.16). After 2 years of follow-up, 34.4% of patients discontinued their second biologic, mainly due to inefficacy (72.8%), with no differences found between groups. Baseline treatment with glucocorticoids was the only predictor of discontinuation after 2 years of follow-up (HR:1.668, 95% CI 1.154-2.409). CONCLUSIONS: After failure of a first TNF inhibitor, Cycling and Swapping strategies result in similar retention rates suggesting that both are acceptable in the management of patients with psoriatic arthritis.
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Ruptures of the annular pulleys of the finger flexor tendons are not common in the general population. In sport climbing, these structures can be abnormally stressed, mainly because of the so-called crimped position, an extreme flexion of the proximal interphalangeal joint, levering an abnormal tension by flexor tendons. Complete pulley tears manifest with explicit pain and an inability, but strains or minor tears might only be perceived by individuals like professional climbers, since they can bring total disability to crucial grip positions. Complete tears of one or more pulleys have already been characterized by ultrasound and magnetic resonance, but no imaging features were described for strains or smaller partial tears. We describe the case of a climber with symptoms of an A2-pulley injury, in whom ultrasound imaging revealed reversible features of fusiform thickening and hypoechogenicity, which resemble the strains that we find in similar structures like tendons and other ligaments.
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Ultrasound is a strong diagnostic and therapeutic tool for the elbow joint. Existing guidelines and protocols list relevant structures to be scanned but lack some logical connection and intermediate exploration manoeuvres to link each step, which we consider crucial from an operator's perspective that aims to be efficient in regular clinical practice. We present thirteen steps that are described in detail and accompanied by forty-seven ultrasound images, logically linked in what we believe is the best balance between detail and a real-world applicable protocol to perform an ultrasound of the elbow joint.
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Articulação do Cotovelo , Cotovelo , Humanos , Cotovelo/diagnóstico por imagem , Articulação do Cotovelo/diagnóstico por imagem , UltrassonografiaRESUMO
INTRODUCTION/OBJECTIVES: The study aims to define the clinical and subclinical calcinosis prevalence, the sensitivity of radiographed site and clinical method for its diagnosis, and the phenotype of Portuguese systemic sclerosis (SSc) patients with calcinosis. METHOD: A cross-sectional multicenter study was conducted with SSc patients fulfilling Leroy/Medsger 2001 or ACR/EULAR 2013 classification criteria, registered in the Reuma.pt. Calcinosis was assessed through clinical examination and radiographs of hands, elbows, knees, and feet. Independent parametric or non-parametric tests, multivariate logistic regression, and sensitivity calculation of radiographed site and clinical method for calcinosis detection were performed. RESULTS: We included 226 patients. Clinical calcinosis was described in 63 (28.1%) and radiological calcinosis in 91 (40.3%) patients, of which 37 (40.7%) were subclinical. The most sensitive location to detect calcinosis was the hand (74.7%). Sensitivity of the clinical method was 58.2%. Calcinosis patients were more often female (p = 0.008) and older (p < 0.001) and had more frequently longer disease duration (p < 0.001), limited SSc (p = 0.017), telangiectasia (p = 0.039), digital ulcers (p = 0.001), esophageal (p < 0.001) and intestinal (p = 0.003) involvements, osteoporosis (p = 0.028), and late capillaroscopic pattern (p < 0.001). In multivariate analysis, digital ulcers (OR 2.63, 95% CI 1.02-6.78, p = 0.045) predicted overall calcinosis, esophageal involvement (OR 3.52, 95% CI 1.28-9.67, p = 0.015) and osteoporosis (OR 4.1, 95% CI 1.2-14.2, p = 0.027) predicted hand calcinosis, and late capillaroscopic pattern (OR 7.6, 95% CI 1.7-34.9, p = 0.009) predicted knee calcinosis. Anti-nuclear antibody positivity was associated with less knee calcinosis (OR 0.021, 95% CI 0.001-0477, p = 0.015). CONCLUSIONS: Subclinical calcinosis high prevalence suggests that calcinosis is underdiagnosed and radiographic screening might be relevant. Multifactorial pathogenesis may explain calcinosis predictors' variability. Key Points ⢠Prevalence of subclinical calcinosis in SSc patients is substantial. ⢠Hand radiographs are more sensitive to detect calcinosis than other locations or clinical method. ⢠Digital ulcers were associated with overall calcinosis, esophageal involvement and osteoporosis were associated with hand calcinosis, and late sclerodermic pattern in nailfold capillaroscopy was associated with knee calcinosis. ⢠Anti-nuclear antibody positivity may be a protective factor for knee calcinosis.
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Calcinose , Osteoporose , Escleroderma Sistêmico , Feminino , Humanos , Estudos Transversais , Portugal , Calcinose/complicações , Calcinose/diagnóstico por imagem , Osteoporose/complicaçõesRESUMO
(1) Background: Patients with systemic lupus erythematous (SLE) experience profound effects on health-related quality of life (HRQoL) that cannot be explained by objective indicators of mortality and morbidity. This study aimed to adapt the SLE Quality of Life (SLEQoL) questionnaire to the European Portuguese population and to assess its reliability and validity for patients with SLE. (2) Methods: Two independent translators translated the original version of the SLEQoL questionnaire into Portuguese. A back-translated version was produced. The Portuguese version of the questionnaire was reviewed and tested for validity and reliability. Cronbach's alpha and the internal validity index were calculated to verify the internal reliability and validity of the content. Rheumatologists filled out the SLE Disease Activity Score (SLE-DAS) and Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index SLICC/ACR-DI questionnaires. (3) Results: This study involved 180 patients, of which 93.8% were females. The results indicated very high internal consistency reliability (α = 0.949), low correlations between the SLEQoL and the SLE-DAS, a correlation between all SLEQoL dimensions and all SF-36 dimensions (except for "response to treatment" and "self-image"), and good correlation scores with both the EQ-5D-5L index and VAS. (4) Conclusion: The Portuguese version of the SLEQoL questionnaire is valid and reliable for the measurement of HRQoL in SLE patients.
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Lúpus Eritematoso Sistêmico , Qualidade de Vida , Feminino , Humanos , Masculino , Reprodutibilidade dos Testes , Portugal , Inquéritos e Questionários , Índice de Gravidade de DoençaRESUMO
OBJECTIVE: To compare the effectiveness and safety of original (Enbrel®) and biosimilar (Benepali®) etanercept in Biologic Disease-modifying Antirheumatic Drug (bDMARD)-naïve patients, measured by persistence rates over 36 months of follow-up. METHODS: A retrospective multicentre observational study using data collected prospectively from The Rheumatic Diseases Portuguese Registry (Reuma.pt) was performed, including patients with: age ≥ 18 years old; diagnosis of Rheumatoid Arthritis (RA), Psoriatic Arthritis (PsA) or Spondyloarthritis (SpA) (axial or peripheral) with active disease and biologic-naïve who initiated treatment with etanercept as the first line biological treatment after 2010. Kaplan-Meyer and Cox regression were used to calculate the persistence rate in treatment. Disease activity at baseline and follow-up data at 6, 12, 18 and 24 months of treatment were compared. Causes for discontinuing therapy were summarized using descriptive statistics. Statistical significance was assumed for 2-sided p-values <0.05. RESULTS: We included 1693 patients (413 on Benepali® and 1280 on Enbrel®): 864 diagnosed with RA, 335 with PsA and 494 with SpA. The 3-year persistence rates were not significantly different between both treatment groups in RA, PsA and SpA patients. In the adjusted Cox model, hazard ratios of discontinuation were not statistically different (p>0.05). The proportion of subjects in remission or low disease activity in each disease was similar in both groups. Overall, 535 (31.6%) patients discontinued etanercept (428 patients on Enbrel® and 107 patients on Benepali®). The major cause of discontinuation was inefficacy (57.8%). No differences for the occurrence of inefficacy or adverse effects were found between treatment groups. CONCLUSIONS: Benepali® and Enbrel® demonstrated similar effectiveness and safety in RA, PsA and SpA in our cohort of patients. These data corroborate that the original and biosimilar drugs have similar quality characteristics and biological activity.
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Antirreumáticos , Artrite Psoriásica , Artrite Reumatoide , Medicamentos Biossimilares , Espondilartrite , Adolescente , Antirreumáticos/efeitos adversos , Artrite Psoriásica/tratamento farmacológico , Artrite Reumatoide/tratamento farmacológico , Medicamentos Biossimilares/efeitos adversos , Etanercepte/efeitos adversos , Humanos , Portugal/epidemiologia , Espondilartrite/tratamento farmacológico , Resultado do TratamentoRESUMO
Antiviral therapies targeting SARS-CoV-2 replication change the course of COVID-19. The European Medicines Agency (EMA) has approved a nirmatrelvir/ritonavir combination that inhibits the main protease of the virus. Molnupiravir, an RNA polymerase misdirector, is proposed by EMA in selected cases, despite still without marketing authorisation. Both are for use in mild disease with a high risk of progression to severe COVID. Patients with inflammatory rheumatic diseases under immunosuppression, mainly high-dose glucocorticoids, are at higher risk of developing severe COVID. We report two clinical cases in which nirmatrelvir/ritonavir and molnupiravir were successfully used to treat COVID-19 in immunosuppressed patients during severe flares of connective tissue diseases, namely systemic lupus erythematosus and dermatomyositis. No significant adverse events attributable to these drugs were noted.
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COVID-19 , Doenças do Tecido Conjuntivo , Humanos , Antivirais/uso terapêutico , Ritonavir/uso terapêutico , SARS-CoV-2Assuntos
Bursite , Calcinose , Escleroderma Sistêmico , Articulação do Ombro , Calcinose/complicações , Calcinose/diagnóstico por imagem , Feminino , Humanos , Injeções Intra-Articulares/efeitos adversos , Escleroderma Sistêmico/complicações , Articulação do Ombro/diagnóstico por imagem , Dor de Ombro/complicaçõesRESUMO
OBJECTIVES: To evaluate belimumab effectiveness and safety in real-life Portuguese patients with Systemic Lupus Erythematosus (SLE). MATERIALS AND METHODS: Multicenter cohort study including all SLE patients treated with belimumab in seven Portuguese rheumatology centers. Demographic, clinical and serological data were collected at baseline, 6, 12 and 24 months of treatment with belimumab. To evaluate effectiveness we used SLE Responder Index (SRI) rates and changes in SELENA-SLEDAI. Safety was evaluated by the number of adverse events. RESULTS: Thirty-eight patients were included: 37 (97.4%) female, with a mean age of 46.2±13.9 years. Mean SELENA-SLEDAI was 8.2±3.9, 78.8% had elevated anti-double-stranded DNA (anti-dsDNA) antibodies and 72.7% had complement consumption at baseline. Multiorgan involvement was the leading cause for the use of belimumab. SRI response was achieved in 51.9%, 60% and 91.7% at 6, 12 and 24 months of belimumab treatment, respectively. LUNDEX adjusted SRI response rates were 45.4%, 45.0% and 45.8% at 6, 12 and 24 months of belimumab, respectively. Mean SELENA-SLEDAI, anti-dsDNA antibodies and daily prednisolone dosage decreased significantly from baseline to 6, 12 and 24 months and C3 levels increased significantly at 12 months of belimumab treatment. Five patients presented adverse events (infections in three cases) and eleven patients discontinued belimumab (four due to inefficacy, three due to adverse events and four were lost to follow-up). CONCLUSIONS: Our study confirmed, in real-life Portuguese patients with active SLE, the effectiveness of belimumab in reduction of disease activity, immunological response and steroid-sparing, with a good safety profile.
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Anticorpos Monoclonais Humanizados/uso terapêutico , Lúpus Eritematoso Sistêmico , Adulto , Estudos de Coortes , Feminino , Humanos , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Portugal , Índice de Gravidade de Doença , Resultado do TratamentoRESUMO
Autologous fat grafting is widely used for soft-tissue augmentation and replacement in reconstructive and aesthetic surgery providing a biocompatible, natural and inexpensive method. Multiple approaches have been developed in the past years, varying in the location of adipose tissue donor-sites, use of wetting solutions, harvesting, processing and placing techniques. Despite many advances in this subject, the lack of standardization in the protocols and the unpredictability of the resorption of the grafted tissue pose a significant limitation for graft retention and subsequent filling. In this review, we discuss several approaches and methods described over the last years concerning the harvesting of autologous fat grafts. We focus on contents such as the best donor-site, differences between existing harvesting techniques (namely tissue resection, hand aspiration or liposuction techniques), recommended harvesting cannula diameters, pressure application and volume of wetting solution injected prior aspiration. Results and comparisons between methods tend to vary according to the outcome measured, thus posing a limitation to pinpoint the most efficient methods to apply in fat grafting. Additionally, the lack of a standard assay to determine viability or volume augmentation of fat grafting remains another limitation to obtain universally accepted grafting procedures and protocols.