Successful cyclophosphamide therapy with complete resolution of pulmonary artery aneurysm in Hughes–Stovin syndrome patient

Abstract

Hughes-Stovin syndrome is an exceedingly rare disorder of unknown etiology, and it is characterized by multiple pulmonary artery aneurysms and peripheral venous thrombosis. We report a case of Hughes-Stovin syndrome with complete resolution of pulmonary artery aneurysm after treatment with cyclophosphamide. A 48-year-old man presented with massive hemoptysis, fever, and weight loss of 4-month duration. Computed tomography angiography revealed multiple pulmonary arterial aneurysms of variable sizes in both lung fields. Inferior vena caval thrombosis was also noted. Despite multiple surgical procedures including embolectomy and lobectomy combined with steroid therapy, new aneurysms continued to appear with persistent hemoptysis. Thorough history and physical examination did not suggest any evidence of Behcet's disease. Hughes-Stovin syndrome was subsequently diagnosed. High-dose prednisolone combined with oral cyclophosphamide therapy was initiated. Chest radiography follow-up in 3 months revealed complete resolution of pulmonary aneurysm. No new aneurysm was detected at 15 months of follow-up.

Hughes-Stovin Syndrome and Massive Hemoptysis: A Management Challenge

“…10,13 No standardized guidelines exist for the treatment of HSS and immunosuppressant drugs remain the predominant first-line therapy. 11,14 This usually involves a combination of steroids and cytotoxic agents, the most commonly used being cyclophosphamide. Anticoagulants and thrombolytic agents are generally contraindicated in patients with HSS due to the increased risk of fatal hemorrhage from pulmonary aneurysms.…”

Fibrosarcoma de arteria pulmonar simulando un síndrome de Hughes Stovin

“…El tratamiento del SHS se basa en corticoides a altas dosis y ciclofosfamida, terapia que en algunos casos puede llevar a la resolución completa de los aneurismas. El uso de anticoagulación no se recomienda por el riesgo de sangrado con hemoptisis masiva, principal causa de muerte 3,7 . El rol de la cirugía es controversial, dado por la alta morbimortalidad y la recurrencia los sitios de la anastomosis, la cual puede llegar a 25% 3,8 .…”

Síndrome Hughes-Stovin: Caso clínico

“…Dependiendo de la respuesta clínica se puede discontinuar el rev Med Chile 2013; 141: 922-926 esteroide y mantener ciclofosfamida, por lo menos, durante 1 año 22 . Se han descrito casos, con la terapia señalada, de estabilización y hasta de regresión de los aneurismas pulmonares 23 .…”