Multimodality imaging of Hughes–Stovin syndrome

Extract

A 21-year-old man presented with fever, haemoptysis, and weight loss. Chest radiograph showed a rounded right parahilar opacity (Panel A, arrow). His serum inflammatory markers were significantly elevated: C-reactive protein of 38 (normal 0–6 mg/L); erythrocyte sedimentation rate of 25 (normal 0–20 mm/h). Computed tomography pulmonary angiography revealed multiple focal contrast opacified saccular outpouchings, arising from the segmental branches of the right and left pulmonary arteries, limited peripherally by non-enhancing hyperdense areas, suggestive of partially thrombosed aneurysms (Panel B, arrow). A three-dimensional image reconstruction confirmed multiple saccular pulmonary aneurysms (Panel D, arrow). ¹⁸F-fluorodeoxyglucose (FDG) positron emission tomography showed abnormal FDG uptake [maximum standardized uptake value (SUVmax) of 2.9] in the aneurysms (Panel C, arrow). An abdominal colour Doppler showed a partially-occluding thrombus in the infrahepatic portion of the inferior vena cava (Panel E, arrow). The diagnosis of Hughes–Stovin syndrome was made and the patient was treated with steroids and cyclophosphamide. The patient was asymptomatic on follow-up. Hughes–Stovin syndrome is characterized by multiple pulmonary and or bronchial artery aneurysms and thrombophlebitis. Immunosuppressive therapy has the potential to stabilize aneurysms, or in certain cases may even make them regress. Corroborating multimodality imaging with clinical and laboratory findings can aid in the prompt diagnosis and treatment of this rare and often fatal syndrome.