Schreiber, B; (2013) Diagnosis and Prognosis in Connective Tissue Disease Associated Pulmonary Hypertension. Doctoral thesis , UCL (University College London).

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Abstract

The thesis addresses questions of diagnosis and prognosis in connective tissue disease associated pulmonary hypertension, as addressed in analyses of clinical data from our patients over the last 15 years. Chapter 1 explores use of pulmonary function tests to improve the detection of patients at risk of pulmonary hypertension. A novel formula is derived and validated. Chapter 2 evaluates whether the formula described in the previous chapter can be combined with echocardiography and other measures in order to further improve the selection of patients for right heart catheterisation. In chapter 3 we turn to the prognostic value of ‘borderline pulmonary hypertenion’, exploring indicators of future progression to pulmonary hypertension with important implications for patient care. In chapter 4 we scrutinise our data on body size and inspected the relationship between obesity and pulmonary hypertension, finding intriguing associations which have not been reported before in this population. Chapter 5 addresses the very rare diagnosis of lupus associated pulmonary hypertension and compare this small group of patients to scleroderma patients with pulmonary hypertension in order to consider whether their response to treatment and prognosis are distinct. For chapter 6 we review the relationship between cross-sectional imaging and pulmonary haemodynamics, and specifically address CT findings suggestive of the rare pulmonary veno-occlusive disease. In chapter 7 we turn our attention to the unfortunate group of patients with systemic sclerosis who develop interstitial lung disease and pulmonary hypertension. We compare their survival to those patients who have pulmonary hypertension without interstitial lung disease and find an important predictor of survival. In chapter 8 we review our cohort of patients with systemic sclerosis and pulmonary hypertension and consider whether pulmonary function tests can be combined with pulmonary haemodynamic measures to improve prognostication.

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