CREST syndrome

CREST syndrome has been described as a form of progressive systemic sclerosis in which there is relatively limited involvement of the skin, prominence of calcinosis, Raynaud's phenomenon, esophageal dysfunction and telangiectasia. The acronym CREST was coined in 1964 by Winterbauer in the USA but the very first case report was by French physicians Thibierge and Weissenbach in 1910. Antinuclear antibodies recognizing chromosomal centromere proteins are characteristic of CREST syndrome and are present in more than 50% of the cases. The prognosis of CREST syndrome is relatively good with a long lasting disease duration (>10 years). Two complications are seldom associated with CREST syndrome: digital gangrene with finger losses and pulmonary hypertension (3 to 14% of CREST syndrome). Pulmonary hypertension is a very late event and the prognosis is very severe (mortality rate of 50% after 2 years).     

CREST综合征

系统性硬化征存在许多分类标准，在其他分类标准未获得广泛确认之前，CREST综合征仍被认为是系统性硬化征的亚型。其由瞰chard首先命名，CREST即皮下钙化、雷诺现象、食管功能障碍、指端硬化、毛细血管扩张五个英文单词字头的缩写。这些特征性的改变均与小血管病变及纤维化有关。目前该病病因不明，可能与遗传、感染、环境、雌激素等原因造成的体液免疫和细胞免疫异常有关。     

Circulating anticoagulant in CREST syndrome

We report the discovery of a circulating anticoagulant in a patient suffering from CREST syndrome. The patient was first seen with a microangiopathic haemolytic anaemia which led to the diagnosis of the CREST syndrome. Several months later, prior to a cataract operation, a routine coagulation screen (prothrombin time and partial thromboplastin time) was abnormal. Investigation showed the presence of a circulating anticoagulant as well as a decrease in several clotting factors, principally factor VIII C.     

CIRCULATING ANTICOAGULANT IN CREST SYNDROME

We report the discovery of a circulating anticoagulant in apatient suffering from CREST syndrome. The patient was firstseen with a microangiopathic haemolytic anaemia which led tothe diagnosis of the CREST syndrome. Several months later, priorto a cataract operation, a routine coagulation screen (prothrombintime and partial thromboplastin time) was abnormal. Investigationshowed the presence of a circulating anticoagulant as well asa decrease in several clotting factors, principally factor VIIIC. KEY WORDS: CREST syndrome, Circulating anticoagulant     

CREST syndrome presenting as pulmonary hypertension

The authors report the case of a 62-year-old patient complaining of recent onset of disabling breathlessness on exertion, and presenting clinical signs of previously undiagnosed scleroderma. Echocardiography revealed a diagnosis of precapillary pulmonary hypertension (74/14 mmHg) (PHT), with no pulmonary cause revealed by pulmonary ventilation-perfusion scintigraphy or by thoracic fine section computed tomography. The diagnosis of PHT in the context of circumscribed scleroderma was confirmed by x-rays of the hands, capillaroscopy, oesophageal investigations and positive anticentromere antinuclear antibodies. The clinical course was marked by rapid deterioration of the symptoms, requiring treatment with prostacyclin by continuous intravenous infusion. The appearance of PHT in a context of circumscribed scleroderma, usually a relatively benign disease, is a rare, late event, exceptionally revealing the disease, as in this case, and indicating a very unfavourable prognosis.     

从CREST研究看颈动脉狭窄的血管内治疗

研究表明,16%的缺血性卒中归因于大血管动脉粥样硬化,颈动脉是主要的好发部位[1].这部分病变治疗对预防卒中有着重要的意义.     

Barrett’s Esophageal Adenocarcinoma with CREST Syndrome

Scleroderma is a systemic and connective tissue disease. Gastrointestinal manifestations including esophageal dysmotility are common in scleroderma patients. The relationship between esophageal reflux and scleroderma has been well described; however, the complication of Barrett’s esophagus and adenocarcinoma due to chronic esophagitis is rare. In addition, there are no detailed reports on the treatment of esophageal adenocarcinoma or the pathological findings in patients with scleroderma. We report the case of a 55-year-old woman who presented with chronic cough and a dilated esophagus. The patient was diagnosed with Barrett’s adenocarcinoma and CREST syndrome, which is a form of scleroderma. Subtotal esophagectomy was performed, and the patient’s chronic cough was relieved. A surgical specimen revealed histological changes at the dilated parts of the esophagus that showed the thinning of the muscular layer with fibrotic changes.     

Pulmonary veno-occlusive disease and the CREST variant of scleroderma.

Pulmonary veno-occlusive disease is a rare cause of pulmonary hypertension. The authors describe a 48-year-old female with the Calcinosis-Raynaud's phenomenon-Esophageal dysmotility-Sclerodactyly-Telangiectasia (CREST) variant of scleroderma who developed acute pulmonary hypertension with pulmonary infiltrates and a normal pulmonary capillary wedge pressure. At post mortem examination typical changes of pulmonary veno-occlusive disease were found. Similarities between this and other cases in the literature suggest a possible association between the CREST variant of scleroderma and pulmonary veno-occlusive disease.     

Calcific constrictive pericarditis. A rare complication of CREST syndrome

This report describes a patient with limited cutaneous scleroderma in whom calcific constrictive pericarditis developed. This complication of limited cutaneous scleroderma has not been reported previously.     

Carotid Artery Stenting Versus Carotid Endarterectomy: Post CREST

The risk of stroke associated with carotid artery stenosis can be reduced with carotid endarterectomy or carotid artery stenting in carefully selected patients. While surgery has been the traditional treatment there have been many trials comparing carotid endarterectomy with carotid artery stenting, and stenting has shown promising results for certain patient populations. This article reviews the natural history of carotid artery stenosis, the data supporting carotid endarterectomy, trials directly comparing surgery to stenting, and current recommendations for each modality.