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

INTRODUCTION: Macrophage activation syndrome (MAS) and pulmonary arterial hypertension (PAH) are rare and life-threatening complications of adult-onset Still disease (AOSD). PATIENT CONCERNS: 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. DIAGNOSIS: MAS was diagnosed as a complication of AOSD. PAH was contributed probably by right anomalous pulmonary venous connection. INTERVENTIONS: The patient dramatically improved with methylprednisolone (80 mg I.V. daily) plus supportive treatments, without interleukin (IL) inhibitors or ciclosporin A given. OUTCOMES: The patient's serum hepatic enzyme levels dropped and hemocytes rose within 1 week. CONCLUSION: 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.