RESUMO
Pulmonary arterial hypertension is a lethal complication of different connective tissue diseases such as systemic sclerosis, mixed connective tissue disease and systemic lupus erythematosus. Although the treatment possibilities for patients with pulmonary arterial hypertension have increased in the last two decades and survival of patients with idiopathic pulmonary arterial hypertension has improved, the latter is not the case for patients with pulmonary arterial hypertension associated with connective tissue disease. In this narrative review, we review recent literature and describe the improvement of early diagnostic possibilities, screening modalities and treatment options. We also point out the pitfalls in diagnosis in this patient category and describe the unmet needs and what the focus of future research should be.
Assuntos
Doenças do Tecido Conjuntivo/terapia , Hipertensão Pulmonar/terapia , Doenças do Tecido Conjuntivo/complicações , Dermatomiosite/complicações , Dermatomiosite/terapia , Gerenciamento Clínico , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologia , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/terapia , Programas de Rastreamento , Doença Mista do Tecido Conjuntivo/complicações , Doença Mista do Tecido Conjuntivo/terapia , Prognóstico , Hipertensão Arterial Pulmonar/diagnóstico , Hipertensão Arterial Pulmonar/etiologia , Hipertensão Arterial Pulmonar/fisiopatologia , Hipertensão Arterial Pulmonar/terapia , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/terapia , Síndrome de Sjogren/complicações , Síndrome de Sjogren/terapiaRESUMO
OBJECTIVES: Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are the leading causes of death in systemic sclerosis (SSc). Although the six-minute walk test (6MWT) is used for evaluating ILD and PAH, no data are available on the evolution of the six-minute walk distance (6MWD) in SSc patients without ILD and PAH and whether the baseline 6MWD could serve as individual reference value for the management of those who will develop PAH or ILD. METHODS: Prospectively collected data of the first 6MWT (at baseline or 6-month follow-up) and the 6MWTs at 18-, 30-, 42-, 54-, and 66-month visit of 165 consecutive SSc patients without ILD and PAH, included in the Ghent University SSc Cohort between May 2006 and December 2016 were analysed. RESULTS: 96-100% of the included patients performed a 6MWT during the follow-up visits. The mean 6MWD during the baseline 6MWT of 165 SSc patients without ILD and PAH (35% limited, 56% limited cutaneous, 9% diffuse cutaneous SSc) was 484.20+/-92.65m with no significant difference in the 6MWD at different follow-up visits as compared to baseline. In 46 SSc patients without ILD and PAH who performed a 6MWT at baseline and at 66-month visit, the 6MWD walked at 66-month visit correlated with the baseline 6MWD (r=0.564, p<0.001). CONCLUSIONS: In SSc without ILD and PAH, the 6MWT is feasible and the 6MWD is clinically stable over a 66 months period. Hence, the individual 6MWD might be used as individual reference value in management of those who will develop PAH or ILD.
Assuntos
Tolerância ao Exercício , Hipertensão Pulmonar/diagnóstico , Doenças Pulmonares Intersticiais/diagnóstico , Escleroderma Sistêmico/diagnóstico , Teste de Caminhada/normas , Caminhada , Adulto , Progressão da Doença , Feminino , Nível de Saúde , Humanos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologia , Estudos Longitudinais , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Prospectivos , Valores de Referência , Reprodutibilidade dos Testes , Fatores de Risco , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/fisiopatologia , Fatores de TempoRESUMO
Screening for pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) improves outcomes. The DETECT screening algorithm is recommended in a high-risk SSc subgroup. This study aims to compare prospectively the positive predictive value of screening using the DETECT algorithm and the 2009 European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines, and to compare their cost-effectiveness in an unselected, day-to-day SSc population. Post hoc, screening according to the 2015 ESC/ERS guidelines using echocardiographic parameters alone ("2015 echo screening") or combined with the DETECT algorithm ("2015 combined screening") in high-risk subjects was analysed.195 consecutive SSc patients included in the Ghent University Hospital SSc cohort were screened using different algorithms.The referral rate for right heart catheterisation was 32% (63 out of 195 patients) (46/4/13/34/40 patients using the DETECT algorithm/2009 guidelines/both/2015 echo screening/2015 combined screening). Right heart catheterisation was performed in 53 patients (84%) (36 (78%)/four (100%)/13 (100%)/28 (82%)/32 (80%) patients recommended by the DETECT algorithm/2009 guidelines/both/2015 echo screening/2015 combined screening). PAH was diagnosed in three patients (incidence 1.5%·year-1, 95% CI 0.5-4.4), in whom all algorithms recommended a right heart catheterisation. The positive predictive value was 6% (95% CI 2-17%; three out of 49 patients) for the DETECT algorithm, 18% (95% CI 6-41%; three out of 17 patients) for the 2009 guidelines, 23% (95% CI 8-50%; three out of 13 patients) for both, 11% (95% CI 4-27%; three out of 28 patients) for the 2015 echo screening and 9% (95% CI 3-24%; three out of 32 patients) for the 2015 combined screening. The cost was EUR224/80/90/112 per patient using the DETECT algorithm/2009 guidelines/2015 echo screening/2015 combined screening.Echocardiography may remain a candidate first step for PAH screening in SSc.
Assuntos
Hipertensão Pulmonar/diagnóstico , Programas de Rastreamento/métodos , Adulto , Algoritmos , Cateterismo Cardíaco , Análise Custo-Benefício , Ecocardiografia , Europa (Continente) , Feminino , Humanos , Hipertensão Pulmonar/complicações , Incidência , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prevalência , Estudos Prospectivos , Escleroderma Sistêmico/complicaçõesRESUMO
OBJECTIVES: Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are the leading causes of death in systemic sclerosis (SSc) patients. Although the six-minute walk test (6MWT) is used for evaluating ILD and PAH in clinical practice, no data are available on six-minute walk distance (6MWD) and oxygen desaturation in SSc patients without ILD and PAH. METHODS: Prospectively collected data of the 6MWTs at baseline and 6-month follow-up of 300 consecutive SSc patients, included in the Ghent University Hospital Systemic Sclerosis Unit between May 2006 and April 2015 were analysed. RESULTS: The mean 6MWD of 165 SSc patients without ILD and PAH who performed a 6MWT at baseline or at the 6-month visit was 484±93m. Patients in the diffuse cutaneous (DcSSc) subgroup (435±94m) walked less than in the limited (LSSc) subgroup (499±91m, p=0.04) and tended to walk less than in the limited cutaneous (LcSSc) subgroup (483±92m, p=0.15). In 115 SSc patients without ILD and PAH who walked at both moments, there was no significant difference between the 6MWDs (mean difference -7.60m 95%CI [-19.93m; 4.73m], p=0.23) and the oxygen desaturation was not statistically different in 102 of them (mean difference 0.41% 95%CI [-0.49%; 1.31%], p=0.37). CONCLUSIONS: In SSc without ILD and PAH, the 6MWD and oxygen desaturation is clinically stable over a 6 months period. The DcSSc subgroup walks less than the LSSc and the LcSSc subgroup.
Assuntos
Escleroderma Sistêmico/fisiopatologia , Teste de Caminhada , Adulto , Idoso , Feminino , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/fisiopatologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Oxigênio/sangue , Estudos Prospectivos , Escleroderma Sistêmico/complicaçõesRESUMO
OBJECTIVES: Reviewing disease activity indices (DAI) in systemic sclerosis (SSc) and reporting their validation status. METHODS: Literature was systematically reviewed on studies documenting the development of DAI, assessing the validation status of DAI and studies using a DAI in their analysis. The qualitative and quantitative validation status of existing DAI was assessed based on OMERACT and on definitions of the American College of Rheumatology (ACR) committee on quality measures. RESULTS: Three DAI in SSc have been proposed in literature: the European Scleroderma Study Group (EScSG) activity index, the 12-point DAI and the Combined Response Index for Systemic Sclerosis (CRISS). The EScSG activity index is yet applied as an outcome measure in 48 different studies. The EScSG activity index and the CRISS are provisional partially validated DAI. CONCLUSIONS: Future studies are needed to fully validate the EScSG activity index and the CRISS and to assess the validation status of the 12-point DAI.
Assuntos
Técnicas de Apoio para a Decisão , Avaliação da Deficiência , Indicadores Básicos de Saúde , Avaliação de Resultados em Cuidados de Saúde/métodos , Escleroderma Sistêmico/diagnóstico , Inquéritos e Questionários , Nível de Saúde , Humanos , Valor Preditivo dos Testes , Prognóstico , Qualidade de Vida , Reprodutibilidade dos Testes , Escleroderma Sistêmico/fisiopatologia , Escleroderma Sistêmico/psicologia , Escleroderma Sistêmico/terapia , Índice de Gravidade de DoençaRESUMO
OBJECTIVES: The European Scleroderma Study Group (EScSG) activity index meets nearly all the OMERACT-standards of truth, discrimination and feasibility. The sensitivity to change remains to be attested. This study assesses sensitivity to change of the EScSG activity index in patients with early and severe diffuse cutaneous Systemic Sclerosis (dcSSc) treated with rituximab. METHODS: 12-month follow-up (open-label study) of 14 consecutive patients with early dcSSc. Patients received an infusion of two times 1000 mg rituximab at month 0 and 6, together with 100 mg methylprednisolone. Clinical read outs (modified Rodnan skin score [mRSS], lung function and echocardiography) and EScSG activity index were performed at month 0, 3, 6 and 12. Mixed models analyses (MMA) were used to evaluate changes in parameters over time. RESULTS: There was a clinically significant change in skin score with a mean (SD) mRSS of 24.8 (4.44) at baseline and 10.4 (3.12) at month 12 (MMA p<0.001). Also the EScSG activity index decreased significantly, with a mean (SD) of 4.3 (1.79) at baseline and 0.7 (0.83) at month 12 (MMA p<0.001). The estimated mean change of the EScSG activity index was -3.6 (95%CI -4.9; -2.4) over 12 months. Indices of internal organ involvement remained stable throughout the study. CONCLUSIONS: A significant improvement of the EScSG activity index was observed, in line with the significant improvement of the mRSS and the stabilisation of internal organ involvement. To our knowledge, this is the first study to attest sensitivity to change of the EScSG activity index in the subset of 'early' dcSSc. TRIAL REGISTRATION: ClinicalTrials.gov Registration, http://clinicaltrials.gov, number NCT00379431.
Assuntos
Imunossupressores/administração & dosagem , Rituximab/administração & dosagem , Esclerodermia Difusa/tratamento farmacológico , Administração Intravenosa , Adulto , Idoso , Bélgica , Esquema de Medicação , Quimioterapia Combinada , Ecocardiografia , Feminino , Cardiopatias/diagnóstico , Cardiopatias/tratamento farmacológico , Cardiopatias/etiologia , Humanos , Imunossupressores/efeitos adversos , Pneumopatias/diagnóstico , Pneumopatias/tratamento farmacológico , Pneumopatias/etiologia , Masculino , Metilprednisolona/administração & dosagem , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Indução de Remissão , Reprodutibilidade dos Testes , Testes de Função Respiratória , Fatores de Risco , Rituximab/efeitos adversos , Esclerodermia Difusa/complicações , Esclerodermia Difusa/diagnóstico , Índice de Gravidade de Doença , Fatores de Tempo , Resultado do TratamentoAssuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Lesão de Contragolpe , Ecocardiografia Transesofagiana/métodos , Aneurisma Cardíaco , Traumatismos Cardíacos , Traumatismo Múltiplo , Septo Interventricular , Adolescente , Lesão de Contragolpe/diagnóstico , Lesão de Contragolpe/fisiopatologia , Lesão de Contragolpe/terapia , Aneurisma Cardíaco/diagnóstico por imagem , Aneurisma Cardíaco/etiologia , Aneurisma Cardíaco/cirurgia , Traumatismos Cardíacos/sangue , Traumatismos Cardíacos/diagnóstico , Traumatismos Cardíacos/fisiopatologia , Hemopneumotórax/diagnóstico , Hemopneumotórax/etiologia , Hemopneumotórax/cirurgia , Humanos , Hemorragia Intracraniana Traumática/diagnóstico , Hemorragia Intracraniana Traumática/etiologia , Hemorragia Intracraniana Traumática/cirurgia , Imagem Cinética por Ressonância Magnética/métodos , Masculino , Traumatismo Múltiplo/diagnóstico , Traumatismo Múltiplo/fisiopatologia , Traumatismo Múltiplo/terapia , Administração dos Cuidados ao Paciente/métodos , Resultado do Tratamento , Troponina T/sangue , Septo Interventricular/diagnóstico por imagem , Septo Interventricular/lesões , Septo Interventricular/patologiaRESUMO
Pulmonary hypertension (PH) is present in an important proportion of patients with interstitial lung diseases (ILDs), encompassing a large, heterogeneous group of diffuse parenchymal lung diseases. Development of ILD-related PH is associated with reduced exercise capacity, increased need for supplemental oxygen, decreased quality of life and earlier death. Diagnosis of ILD-related PH is important and requires a high index of suspicion. Noninvasive diagnostic assessment can suggest the presence of PH, although right heart catheterisation remains the gold standard to confirm the diagnosis and to assess its severity. A comprehensive assessment is needed to make sure reversible causes of PH have been ruled out, including thromboembolic events, untreated hypoxaemia and sleep disordered breathing. The results of trials concerning pulmonary vasodilators in this particular patient group have been disappointing and, in some cases, were even associated with an increased risk of harm. Newer strategies such as medications administered through inhalation and combinations with antifibrotic drugs show encouraging results. Moreover, unravelling the role of the vasculature in the pathophysiology of pulmonary fibrosis and ILD-related PH may potentially unlock new therapeutic opportunities.
RESUMO
In this article, we report a patient with new-onset dyspnea and symptoms suggestive of heart failure, who had an unexpected diagnosis of a large left atrial myxoma with diastolic protrusion into the left ventricle. We further underline the role of cardiac Point-of-Care Ultrasound (POCUS) in the initial evaluation of patients with cardiac complaints in the emergency room setting. It can help to differentiate the patients' symptoms in order to achieve a more accurate diagnosis and thus increase the efficacy of the established therapy. In some cases, as with this patient, it can help to establish a diagnosis which needs prompt therapy.
Assuntos
Ecocardiografia , Sistemas Automatizados de Assistência Junto ao Leito , Dispneia/diagnóstico por imagem , Dispneia/etiologia , Serviço Hospitalar de Emergência , Humanos , Testes Imediatos , UltrassonografiaRESUMO
Background: Constrictive pericarditis is characterized by the encasement of the heart by a stiff pericardium leading to impaired diastolic function, which ultimately leads to congestive heart failure. Case summary: We report a case of a young woman, who first presented to the ophthalmologist with the sudden appearance of floaters and vision reduction. Eventually, invasive haemodynamic assessment led to the diagnosis of constrictive pericarditis leading to venous congestion. Conclusion: Understanding the pathophysiology and integrating the results of invasive and non-invasive diagnostic work up is important in making this challenging diagnosis.
RESUMO
BACKGROUND: Malignant cardiac tamponade is a life-threatening condition that requires prompt treatment and effective management to prevent recurrence. This paper describes safety and efficacy outcomes after intrapericardial instillation of bleomycin as well as possible predictors of survival. METHODS: We performed a 10-year retrospective, single-center study to evaluate the safety and efficacy of intrapericardial instillation of bleomycin in patients with suspected malignant cardiac tamponade. RESULTS: Intrapericardial instillation of bleomycin was performed in 31 cancer patients (9 men, 22 women) presenting with cardiac tamponade. Non-fatal complications occurred in 3 patients and relapse occurred in 1 patient. Overall survival was less than 10% at the end of the study. Median survival was 104 days (95% CI, 0-251 days). Survival was compared between different groups (defined by primary tumor, type of tumor, TNM stage and results of cytological analysis) with median survival being considerably higher when oncologic therapy was altered afterwards. CONCLUSIONS: The use of intrapericardial bleomycin instillation following pericardiocentesis for malignant cardiac tamponade is a safe procedure with a high success rate. Survival rates depend on further oncological treatment options available.
Assuntos
Antineoplásicos , Tamponamento Cardíaco , Neoplasias Pulmonares , Derrame Pericárdico , Antineoplásicos/uso terapêutico , Bleomicina/efeitos adversos , Tamponamento Cardíaco/tratamento farmacológico , Feminino , Humanos , Neoplasias Pulmonares/tratamento farmacológico , Masculino , Recidiva Local de Neoplasia , Estudos RetrospectivosRESUMO
INTRODUCTION: Peripartum cardiomyopathy (PPCM) patients refractory to medical therapy and intra-aortic balloon pump (IABP) counterpulsation or in whom weaning from these therapies is impossible, are candidates for a left ventricular assist device (LVAD) as a bridge to recovery or transplant. Continuous-flow LVADs are smaller, have a better long-term durability and are associated with better outcomes. Extra corporeal membrane oxygenation (ECMO) can be used as a temporary support in patients with refractory cardiogenic shock. The aim of this study was to evaluate the efficacy and safety of mechanical support in acute and critically ill PPCM patients. METHODS: This was a retrospective search of the patient database of the Ghent University hospital (2000 to 2010). RESULTS: Six PPCM-patients were treated with mechanical support. Three patients presented in the postpartum period and three patients at the end of pregnancy. All were treated with IABP, the duration of IABP support ranged from 1 to 13 days. An ECMO was inserted in one patient who presented with cardiogenic shock, multiple organ dysfunction syndrome and a stillborn baby. Two patients showed partial recovery and could be weaned off the IABP. Four patients were implanted with a continuous-flow LVAD (HeartMate II, Thoratec Inc.), including the ECMO-patient. Three LVAD patients were successfully transplanted 78, 126 and 360 days after LVAD implant; one patient is still on the transplant waiting list. We observed one peripheral thrombotic complication due to IABP and five early bleeding complications in three LVAD patients. One patient died suddenly two years after transplantation. CONCLUSIONS: In PPCM with refractory heart failure IABP was safe and efficient as a bridge to recovery or as a bridge to LVAD. ECMO provided temporary support as a bridge to LVAD, while the newer continuous-flow LVADs offered a safe bridge to transplant.
Assuntos
Cardiomiopatias/terapia , Cuidados Críticos/métodos , Oxigenação por Membrana Extracorpórea , Coração Auxiliar , Balão Intra-Aórtico , Complicações Cardiovasculares na Gravidez/terapia , Doença Aguda , Adulto , Estado Terminal , Oxigenação por Membrana Extracorpórea/efeitos adversos , Feminino , Transplante de Coração , Coração Auxiliar/efeitos adversos , Humanos , Balão Intra-Aórtico/efeitos adversos , Período Periparto , Gravidez , Estudos Retrospectivos , Resultado do TratamentoRESUMO
OBJECTIVES: The epidemiology of interstitial lung disease (ILD) in systemic sclerosis (SSc) in Belgium is unknown. In literature, its prevalence varies between 19% and 52% in limited/diffuse cutaneous SSc (LcSSc/DcSSc). However, its prevalence in "early" SSc (pre-clinically overt SSc without [yet] skin involvement), nor its incidence rate in SSc (LcSSc/DcSSc/"early" SSc) has ever been described. Against this background, we aimed to determine the prevalence/incidence (rate) and progression of ILD in SSc. METHODS: 12-year follow-up data of consecutive SSc patients, included in two Flemish cohorts (University Hospitals Ghent and Leuven), were retrospectively analysed. ILD was classified according to the simplified Goh algorithm. Progression of ILD was defined as a relative decline of FVC ≥10%, a combined relative decline of FVC 5-10% and DLCO ≥15%, or as an increase in HRCT extent. RESULTS: 722 patients (60% LcSSc/ 20% DcSSc/ 20% "early" SSc, median (IQR) follow-up 39 [12-80] months) had baseline HRCT. 243 were rated to have ILD at baseline and 39 during follow-up (prevalence of 34%/ incidence rate of 20.3/1000PY, 95%CI:14.5-27.8). Amongst those with baseline ILD, 60% had lung functional progression at five years of follow-up. In the "early" SSc subgroup, eight patients were rated to have ILD at baseline and three during follow-up (prevalence of 6%/ incidence rate of 5.8/1000 PY, 95%CI:1.2-17.0). CONCLUSION: Both LcSSc and DcSSc patients should be monitored for ILD evolution. The low prevalence and incidence of ILD in the "early" SSc subgroup may instruct future decisions on the construction of uniform patient follow-up pathways in "early" SSc.
Assuntos
Doenças Pulmonares Intersticiais , Escleroderma Sistêmico , Algoritmos , Humanos , Incidência , Pulmão , Doenças Pulmonares Intersticiais/epidemiologia , Prevalência , Estudos Retrospectivos , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/epidemiologiaRESUMO
SUMMARY: Perioperative infections remain an important problem for patients undergoing liver transplantation (LT). For prevention of these infections, perioperative prophylaxis has become the standard procedure. Yet, either guidelines or data on current practice are lacking. The aim of the study was to gain insight into prophylactic antimicrobial strategies used in Europe. A survey questionnaire was sent out to all LT centers that are member of the European Liver and Intestine Transplant Association. In the survey questionnaire, we asked for details on the prophylactic antimicrobial regimen used in LT recipients. The response rate was 48%. Antibiotic prophylaxis for elective LT was provided by a first-line betalactam antibiotic or co-trimoxazole in 25%. Seventy-three per cent of those centers surveyed gave an extended spectrum, and one center used a 6-month rotation strategy. Antifungal prophylaxis was administered in 35% of centers in all LT recipients, in 53% of centers in patients at risk, and in 12% of centers not at all. Cytomegalovirus prophylaxis was never administered in 10%. In 12% of the centers surveyed, all the patients received cytomegalovirus prophylaxis, and another 78% of the centers gave it only to risk groups. In Europe, there is a considerable variation in the different antibiotic, antifungal and cytomegalovirus prophylactic strategies used for LT. These findings underscore the need for randomized controlled trials to determine the optimal prophylactic antimicrobial regimen.
Assuntos
Anti-Infecciosos/administração & dosagem , Transplante de Fígado/métodos , Complicações Pós-Operatórias/prevenção & controle , Antibacterianos/administração & dosagem , Antifúngicos/administração & dosagem , Antivirais/administração & dosagem , Infecções por Citomegalovirus/prevenção & controle , Europa (Continente) , Humanos , Controle de Infecções/métodos , Falência Hepática Aguda/cirurgia , Transplante de Fígado/efeitos adversos , Transplante de Fígado/estatística & dados numéricos , Sociedades Médicas , Inquéritos e QuestionáriosRESUMO
OBJECTIVE: Pulmonary arterial hypertension (PAH) is one of the leading causes of death in systemic sclerosis (SSc). Current screening algorithms are hampered by low positive predictive values. Outcome measures that could add to performance characteristics would be welcome. We aim to evaluate the role of nailfold videocapillaroscopy (NVC) using standardized definitions, in SSc-related PAH (SSc-PAH). METHODS: A systematic review to identify original research papers documenting an association between NVC and right heart catheterization-defined SSc-PAH was performed according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. Subsequently, NVC characteristics were subdivided into quantitative (capillary density, dimension, morphology, and hemorrhages), semiquantitative, and qualitative assessment (NVC pattern), according to the definitions of the European League Against Rheumatism Study Group on Microcirculation in Rheumatic Diseases. RESULTS: The systematic search identified 316 unique search results, of which 5 were included in the final qualitative analysis. The occurrence of incident SSc-PAH unequivocally associated in 2 longitudinal studies with progressive capillary loss (p = 0.04 and p = 0.033) and the progression to a severe (active/late) NVC pattern (p = 0.05/0.01 and HR = 5.12, 95% CI 1.23-21.27). In 3 cross-sectional studies, SSc-PAH was found to be unequivocally inversely associated with capillary density (p = 0.001 and p < 0.05) and associated with the presence of a severe NVC pattern (p = 0.03 and p < 0.05). CONCLUSION: This is the first systematic literature review investigating the role of NVC in SSc-PAH using standardized description, to our knowledge. Unequivocal associations were found between (incident) SSc-PAH and capillary density and NVC pattern. Integration of NVC into current screening algorithms to boost their performance may be a future step.
Assuntos
Hipertensão Arterial Pulmonar , Escleroderma Sistêmico , Capilares , Estudos Transversais , Humanos , Angioscopia Microscópica , Unhas , Escleroderma Sistêmico/complicaçõesRESUMO
OBJECTIVE: To investigate whether nailfold videocapillaroscopy (NVC), an increasingly worldwide used non-invasive tool to reliably evaluate the peripheral microcirculation, may be an outcome measure in future screening algorithms for systemic sclerosis related interstitial lung disease (SSc-ILD). METHODS: A systematic review to identify original research papers documenting an association between NVC and SSc-ILD was performed in 3 electronic databases according to the PRISMA guidelines. Subsequently, NVC parameters were subdivided according to the consented standardised capillaroscopic definitions of the EULAR Study Group on Microcirculation in Rheumatic Diseases / Scleroderma Clinical Trials Consortium on Capillaroscopy, into quantitative (capillary density, capillary dimension, capillary morphology and haemorrhages) and qualitative assessment (NVC pattern). RESULTS: The systematic search identified 310 unique search results, of which 2 cross-sectional and 1 longitudinal study were retained. In both cross-sectional studies, the presence of SSc-ILD was found to be inversely associated with capillary density (p = .008 and p = .005). The presence of a severe (active/late) NVC pattern was evaluated and associated with the presence of SSc-ILD in one of the cross-sectional studies. In the longitudinal study, incident SSc-ILD was associated with progressive capillary loss (p = .03) and the conversion to a worse (active/late) NVC pattern (p = .001/p = .003). CONCLUSIONS: This first systematic literature review investigating the role of NVC in SSc-ILD using standardised capillaroscopic definitions uncovered associations between NVC and (incident) SSc-ILD. If large prospective studies further corroborate and elucidate these findings, NVC might possibly be a candidate outcome measure to be integrated in screening algorithms for incident/progressive SSc-ILD.
Assuntos
Algoritmos , Doenças Pulmonares Intersticiais/diagnóstico , Angioscopia Microscópica , Escleroderma Sistêmico/diagnóstico , Capilares , Estudos Transversais , Humanos , Estudos Longitudinais , UnhasRESUMO
Background: Pulmonary arterial hypertension (PAH) is a devastating, life-threatening disease with poor prognosis when left untreated. The long-term prognosis is definitely influenced by the natural progression of PAH but late disease-specific complications may also contribute. Case summary: We present a patient with a long-standing idiopathic PAH in whom progressive dilatation of pulmonary trunk and pulmonary arteries leads to compression of the left main coronary artery and the left atrium with hemodynamic compromise. Conclusion: With the current treatment options, survival in PAH has improved. Guidelines focus on more aggressive treatment with initial combination therapy and earlier referral for transplantation.
Assuntos
Aneurisma , Doença da Artéria Coronariana , Hipertensão Pulmonar Primária Familiar , Intervenção Coronária Percutânea/métodos , Artéria Pulmonar , Enxerto Vascular , Adulto , Aneurisma/diagnóstico , Aneurisma/fisiopatologia , Aneurisma/cirurgia , Síndromes Compartimentais/diagnóstico , Síndromes Compartimentais/etiologia , Síndromes Compartimentais/fisiopatologia , Síndromes Compartimentais/cirurgia , Doença da Artéria Coronariana/diagnóstico , Doença da Artéria Coronariana/etiologia , Doença da Artéria Coronariana/cirurgia , Diagnóstico Diferencial , Técnicas de Diagnóstico Cardiovascular , Progressão da Doença , Hipertensão Pulmonar Primária Familiar/diagnóstico , Hipertensão Pulmonar Primária Familiar/etiologia , Hipertensão Pulmonar Primária Familiar/fisiopatologia , Evolução Fatal , Feminino , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/fisiopatologia , Humanos , Prognóstico , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/patologia , Artéria Pulmonar/cirurgia , Tomografia Computadorizada por Raios X/métodos , Enxerto Vascular/instrumentação , Enxerto Vascular/métodosRESUMO
Introduction Juvenile hemochromatosis is a rare but severe form of hereditary hemochromatosis that typically presents early in life and can be fatal if left untreated. Case presentation We present the case of a 30-year-old man with a clear symptomatology of juvenile hemochromatosis, but in whom the diagnosis was initially mistaken for alcoholic liver disease because of known excessive use of alcohol, with the consequence that an adequate treatment was postponed. Discussion In this report, we discuss the diagnosis and treatment of juvenile hemochromatosis, focusing on the interaction between hemochromatosis and alcohol induced liver disease and how to differentiate both. We conclude that every young patient with suspected alcoholic liver disease and signs of iron overload should have a testing to rule out other iron overloading pathology, since early recognition and treatment with phlebotomy may prevent organ damage and improve life expectancy.
Assuntos
Cardiomiopatias , Hemocromatose/congênito , Cirrose Hepática , Adulto , Cardiomiopatias/diagnóstico , Cardiomiopatias/etiologia , Diagnóstico Diferencial , Hemocromatose/complicações , Hemocromatose/diagnóstico , Hemocromatose/terapia , Humanos , Cirrose Hepática/diagnóstico , Cirrose Hepática/etiologia , Masculino , Tempo para o TratamentoRESUMO
Nailfold capillaroscopy is an easy, non-invasive technique to assess microvascular involvement in rheumatic diseases. Multiple studies describe capillaroscopic changes in systemic lupus erythematosus (SLE), including a wide range of non-specific findings. On behalf of the European League Against Rheumatism (EULAR) study group on microcirculation in rheumatic diseases, a systematic review was done to obtain all original research studies (in English) in which SLE patients had capillaroscopy. Forty such studies are identified. This article firstly provides a résumé of the results of these studies according to capillaroscopic parameters (density, dimensions, morphology, haemorrhages), semi-quantitative assessment and qualitative assessment of capillaroscopy in SLE patients. Secondly, the correlations between capillaroscopic parameters in SLE patients and clinical and laboratory parameters (including auto-immune parameters) are outlined. The following capillaroscopic parameters are found to be significantly more prevalent in SLE patients compared to healthy controls: tortuous capillaries, abnormal morphology and haemorrhages. Hairpin-shaped capillaries are significantly less prevalent than in healthy persons. The semi-quantitatively determined nailfold capillaroscopic score (NFC score) in SLE patients is also higher than in healthy controls. Several correlations between clinical and laboratory parameters and capillaroscopic parameters are identified in the review. Disease activity is correlated with NFC score in seven studies, with abnormal morphology (i.e. "meandering") in one study and with haemorrhages in one study. Frequent attacks of Raynaud's phenomenon (RP) and gangrene are significantly correlated with dilated capillaries. In two studies a possible correlation between anti-SSA antibodies and lower density of capillaries is withheld. About other immune parameters conflicting results are found. In one study a significant negative correlation is found between 24-hour proteinuria and abnormal morphology (i.e. "meandering"). For the first time, an overview of the nailfold capillaroscopic changes that have been described in SLE and their correlations with clinical and laboratory findings is given. Further large-scale research on the identification of capillaroscopic changes in SLE and their correlations with standardised clinical and laboratory parameters, is ongoing at the EULAR study group on microcirculation in rheumatic diseases.