Objective To investigate the diagnosis and treatment of pulmonary arterial hypertension ( PAH) due to rare causes. Methods The clinical presentation, laboratory testing, diagnosis and treatment of 4 patients with PAH associated with rare causes in Beijing Anzhen Hospital from January 2001 to March 2008 were analysed retrospectively. Results Primary biliary cirrhosis, hyperthyroidism, antiphospholipid syndrome and pulmonary artery sarcoma may cause PAH, which were improved after corresponding diagnosis and management. Conclusion PAH can result from rare causes. The enhancement of its recognition will help earlier diagnosis and treatment and improve the prognosis.
ObjectiveTo analyze the clinical and immunological characteristics of 26 patients with antiphospholipid syndrome (APS). MethodsThe clinical and immunological features of APS in 26 patients treated between January 2009 and January 2012 were analyzed retrospectively. ResultsThe study consisted of 8 male and 18 female patients with a mean age of 38 years at diagnosis. Seven patients had primary APS. Nineteen patients had APS associated with autoimmune diseases, including 11 cases of systemic lupus erythematosus. Seventy-five percent of the patients had thrombosis, 16 had venous thrombosis, 7 had arterial thrombosis, and 2 had both arterial and venous thrombosis. The deep veins of the lower limbs and the cerebral arterial circulation were the most common sites of venous and arterial thrombosis. Fetal morbidity rate of 16 married women in our study was 43.8%. Thrombocytopenia and anemia were present in 57.7% and 69.2% of the patients respectively. ConclusionAPS is characterized by recurrent thrombosis, pregnancy morbidity and positive antiphospholipid antibodies tests. Early diagnosis and management is essential for APS.