Graham Hughes1 and Shirish Sangle2
(1)
The London Lupus Centre, London Bridge Hospital, London, UK
(2)
Louise Coote Lupus Unit, St Thomas’ Hospital, London, UK
Abstract
Pulmonary embolism, following a peripheral venous thrombosis, and in situ pulmonary thrombosis are well-recognised features of Hughes Syndrome (Fig. 15.1).
Pulmonary embolism, following a peripheral venous thrombosis, and in situ pulmonary thrombosis are well-recognised features of Hughes Syndrome (Fig. 15.1).
Figure 15.1
A CT pulmonary angiogram showing thrombosis in the right pulmonary artery in Hughes syndrome
A second important complication of Hughes Syndrome is pulmonary hypertension (PHT). Although recognised in the original 1983 descriptions, the extent of the association is not yet known.
15.1 Pulmonary Hypertension
PHT is defined as a mean pulmonary artery pressure greater than 25 mmHg. Although there are a number of recognised causes of PHT, (Fig. 15.2a, b) including genetic factors, chronic hypoxia, left ventricular failure and repeated pulmonary embolism, in the majority of cases of PHT, the cause is unknown.
Figure 15.2
(a) and (b) A CT scan and chest X-ray showing dilated pulmonary vessels in a case of pulmonary hypertension in Hughes syndrome
Whether the process is predominantly of recurrent pulmonary embolism or of in situ pulmonary thrombosis is also unknown.
The assessment of patients thought to have PHT has become the job of specialised cardiopulmonary centres.
Medical treatment includes anticoagulation, oxygen support, prostacyclin analogues and newer drugs including Bosentan (endothelial B receptor inhibitor).
Surgical treatment in severe cases can include thrombo-endarterectomy, septectomy and, in some cases, heart–lung transplantation.