Pulmonary artery hypertension of adult onset of Still's disease: 3 cases report with literature review / 中华风湿病学杂志

ABSTRACT

Objective To improve the rheumatologists'understanding of pulmonary artery hypertension (PAH) of adult onset Still's disease (AOSD).Methods Three cases of (AOSD) with (PAH) were reported and the related literatures were reviewed.Results All patients were female.The average age was 29 years old.As the cases showed,the clinical manifestations of AOSD were all before the appearance of PAH symptoms and the interval could be 5 months to 9 years.From the ultrasonic cardiogram estimation,the PASP of these patients was (80±21) mm Hg (1 mm Hg=0.133 kPa) on average.The common characteristics included the severity of AOSD,recurrence after high dose of corticosteroid and various immunosuppressive agents aggressively.In addition,PAH was developed rapidly,and we found that patients were die 1 year later after disease onset.Studies showed that all of their death were related to circulation failure caused by right heart failure combined with obstructive shock.And infection was considered as an important predisposing factor of the obstructive shock.Conclusion From the findings above,we can conclude that ASOD complicated with PAH is rare,and it always develops rapidly.Furthermore,the state of disease is very serious and at the same time,the prognosis remains poor.Rheumatologists should aware this rare condition.