Esclerodermia y paniculitis localizadas / Localized scleroderma and panniculitis

La esclerodermia es una enfermedad del tejido conectivo, autoinmunitaria y caracterizada por fibrosis de la piel1-3. Literalmente significa 'piel dura'. La afectación puede ser mínima (solo en dedos y cara, muy lentamente progresiva) o generalizada (afectando de forma rápida a uno o más órganos internos). La paniculitis neutrofílica4,6 es una respuesta inmunitaria localizada en forma de placa o nódulo, en el seno generalmente de una enfermedad sistémica (AU)

Scleroderma is a disease that affects the connective tissue. It is an autoimmune disease and it is characterized by skin fibrosis. It literally means 'hard skin'. The involvement can be minimal (only fingers and face, progressing slowly) or generalized (quickly affecting one or two internal organs). Neutrophilic panniculitis is an immune response located in the form of a plate or nodule, generally related to a systemic disease (AU)

A systematic overview on the use and relevance of capillaroscopy in systemic sclerosis.

Capillaroscopy is a non-invasive and safe technique that allows the detection and quantification of the early microvascular abnormalities that characterize secondary Raynaud's phenomenon. The well-established role of capillaroscopy for the early diagnosis of systemic sclerosis, its inclusion in the classification criteria, combined with its predictive value for clinical complications of the disease and its potential for monitoring disease progression and treatment response, makes nailfold capillaroscopy an important assessment in clinical practice and research. Capillaroscopy provides a unique window into the microcirculation and its application in diseases in which a microvascular component is suspected; it also may provide new insights into their pathophysiology and natural history.

State of the art on nailfold capillaroscopy: a reliable diagnostic tool and putative biomarker in rheumatology?

Capillaroscopy is a non-invasive and safe tool to morphologically study the microcirculation. In rheumatology it has a dual use. First, it has a role in differential diagnosis of patients with RP. Second, it may have a role in the prediction of clinical complications in CTDs. In SSc, pilot studies have shown predictive associations with peripheral vascular and lung involvement hinting at a role of capillaroscopy as putative biomarker. Also and logically, in SSc, microangiopathy, as assessed by capillaroscopy, has been associated with markers of the disease such as angiogenic/static factors and SSc-specific antibodies. Moreover, morphological assessments of the microcirculation (capillaroscopy) seem to correlate with functional assessments (such as laser Doppler). Because of its clinical and research role, eyes are geared in Europe to expand the knowledge of this tool. Both the European League Against Rheumatism (EULAR) and the ACR are stepping forward to this need.

¿Podemos predecir la gravedad de la hipertensión arterial pulmonar en pacientes con esclerodermia? / Can we predict the severity of pulmonary hypertension in patients with scleroderma?

Objetivos. Describir las características clínico-biológicas de pacientes con esclerodermia (ES) e hipertensión arterial pulmonar (HTAP). Establecer la relación entre las pruebas funcionales respiratorias (PFR), la ecocardiografía Doppler (eco-Doppler) y la gravedad de la HTAP. Material y métodos. Estudio retrospectivo de pacientes con diagnóstico de ES seguidos en un centro de tercer nivel. Se les realizó un estudio protocolizado con analítica completa y pruebas complementarias; se estimó la presión arterial pulmonar sistólica (PAPs), la velocidad de reflujo de la válvula tricúspide (VRT), la difusión de monóxido de carbono (DLCO) y la capacidad vital forzada (CVF), por medio de la eco-Doppler y la PFR. Resultados. Se incluyó a 331 pacientes, de los cuales 68 (20,5%) tenían HTAP. El subtipo de ES más prevalente fue la limitada. Se calculó el coeficiente de correlación de Pearson a las siguientes variables: CVF-PAPs CVF/DLCO-PAPs, DLCO-PAP y VRT-PAPs, observándose una asociación lineal moderada significativa en la relación DLCO-PAPs y con VRT-PAP. Se constataron 29 fallecimientos, 12 relacionados a la HTAP. El tiempo medio entre el diagnóstico de HTAP y la muerte fue de 1,8 años. Conclusiones. La disminución de la DLCO y el aumento de la VRT son factores predictores de HTAP, que al mismo tiempo condiciona un peor pronóstico en los pacientes con ES (AU)

Objectives: To describe the clinical-biological characteristics of patients with scleroderma (SSc) and pulmonary artery hypertension (PAH). To establish the relationship between pulmonary functional tests (PFT), Doppler echocardiography (ECHO) and the severity of the PAH. Material and methods: Retrospective study of patients with scleroderma treated at a tertiary center. All participants received a protocol study, which included a complete analysis and additional tests: Doppler Echocardiography and pulmonary functional tests (PFT) with carbon monoxide diffusing capacity (DLCO). Results: Overall, 331 patients were treated, including 68 (20.5%) with PAH. The limited subtype of Scleroderma was the most prevalent. The Person‘s correlation coefficient was used for the following variables: FVC-sPAP, FVC/DLCO-sPAP, DLCO-sPAP and TRV-sPAP, showed a significant moderate linear association in the relationship DLCO-sPAP and TRV-sPAP. 29 deaths occurred, with 12 of them related to PAH. The median time between the PAH diagnosis and death was 1.8 years. Conclusions: The decrease in DLCO and the increase in TRV are negative predictor factor of PAH which, at the same time, means a worsening prognosis for patients with Scleroderma (AU)

Human microvascular imaging: a review of skin and tongue videomicroscopy techniques and analysing variables.

Imaging techniques are increasingly important in clinical diagnostic work. Microcirculation is essential for function and survival of all tissues, but imaging techniques for microvascular monitoring of patients have only been used to a limited extent in clinical practice. The microcirculation is complex with its static and dynamic feature, and only skin and oral mucosa are readily accessible for microscopic examination. In addition, there is a no general agreement on which microvascular parameters and analysing techniques that could be useful. The aim of this article is to give an overview of techniques for human intra-vital microscopy and to describe software and variables used to analyse pictures and film sequences of human microcirculation. We also present clinical examples where microvascular examinations have been shown to be of diagnostic value. Improved techniques for assessing microvascular morphology and physiology will probably become important tools in clinical work in many disciplines in the near future.