Macrophage activation syndrome and pulmonary arterial hypertension in a patient with adult-onset Still disease: A case report

Macrophage activation syndrome (MAS) and pulmonary arterial hypertension (PAH) are rare and life-threatening complications of adult-onset Still disease (AOSD). We reported an interesting case of a 25-year-old AOSD patient with MAS and PAH, and the patient was found to have right anomalous pulmonary venous connection accompanied by an atrial septal defect. MAS was diagnosed as a complication of AOSD. PAH was contributed probably by right anomalous pulmonary venous connection. The patient dramatically improved with methylprednisolone (80 mg I.V. daily) plus supportive treatments, without interleukin (IL) inhibitors or ciclosporin A given. The patient's serum hepatic enzyme levels dropped and hemocytes rose within 1 week. Other causes need to be excluded carefully before giving a diagnosis of PAH with AOSD. Early diagnosis and aggressive treatments are pivotal to improve the quality of life and the survival of patients.