| Pulmonary complications are the leading cause of death in scleroderma, a multi-system, connective tissue disease. Pulmonary hypertension (PHT) and interstitial lung disease (ILD) are the two pulmonary complications. A retrospective chart review and prospective data collection on the Johns Hopkins and University of Maryland Scleroderma Center cohort was conducted to create a database for epidemiologic studies. This database was used to address the following hypotheses: (1) Individuals with combined pulmonary disease are a unique subpopulation of scleroderma patients. (2) Individuals with combined PHT and RVD have a poorer prognosis than other individuals with scleroderma. (3) Clinical features such as age, race, severity of Raynaud's phenomenon, disease subtype, and decreased DLCO predict the development of severe PHT.; To address the first two hypotheses, patients who had concurrent pulmonary function tests and echocardiograms were included. With regard to demographics, extent of skin disease, and lung function, individuals with combined pulmonary disease resemble those with restrictive ventilatory defect as a marker of ILD; however, they are 7–8 years older at disease onset and diagnosis, similar to individuals with isolated PHT. Additionally, they have PHT as severe as those with isolated PHT and a similar mortality, 2.40 times greater than those with no pulmonary disease. To answer the second question, we examined patients who had echocardiograms performed, excluding echocardiograms performed greater than a year before diagnosis of scleroderma. Severe PHT developed in 13.6% of patients who initially had no PHT and 17.7% of patients who initially had mild moderate PHT. Severe PHT was a relatively rare complication, even in the highest risk group (individuals with limited disease, older at diagnosis, who have mild to moderate PHT), the probability of developing severe PHT was only 27.3%. Furthermore, a DLCO <50% of predicted was associated with the development of severe PHT within the next five years in those initially without evidence of PHT. With these results and those of future studies, researchers may determine how best to treat combined pulmonary disease and to intervene for early therapy in PHT, thereby making a significant impact on the lives of individuals with scleroderma. |
