[Long-term retrospective study of patients with connective tissue diseases accompanied by pulmonary hypertension]

To determine clinical and serological features that are related with the prognosis of connective tissue diseases with pulmonary hypertension (PH), we studied a long-term prognosis of 14 patients (4 SLE, 1 PSS, 3 MCTD, 2 primary Sjögren's syndrome, and 5 OL) accompanied by PH. The patients were divided into three groups; group A (4 cases) that is alive for 5-12 years until present, group B (6 cases) in which PH was the main cause of death, and group C (4 cases) was dead of pulmonary fibrosis and pericardial tamponade. No significant difference was observed in background connective tissue diseases among the three groups. However, the mean ages at the onset of PH was obviously younger in group B (25.7 yrs.) than both in group A (38.3 yrs.) and in group C (42.0 yrs.) (group A vs group B, p < 0.02, and group B vs group C, p < 0.05). Sudden death occurred in 5 of the 6 in group B, whereas it was not observed either in group A or in group C. Also, the interval between onset of PH and death was shorter in group B (1.3 yrs.) than in group C (3.5 yrs.). The incidence of digital necrosis and pericardial effusion was higher in group B (83% and 83%, respectively) than both in group A (0% and 25%, respectively) and in group C (25% and 40%, respectively). A large amount of pericardial effusion was detected in 4 of 5 cases in group B. The incidence of digital necrosis between group A and group B was significantly different (p < 0.05, Fisher's test).(ABSTRACT TRUNCATED AT 250 WORDS)