Pulmonary artery pseudoaneurysms (PAPs) in Hughes-Stovin syndrome (HSS) as an emerging concept for a potentially fatal course

Abstract

Introduction

Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by pulmonary artery aneurysms (PAA) and widespread venous and arterial thrombosis. Fatalities in HSS resulted from unforeseeable fatal suffocating hemoptysis. It is critical to early detect pulmonary involvement to take timely measures against inevitable serious life-threatening complications.

Case presentation

A 27 year old Filipino male patient presented to the emergency department with an acute attack of massive hemoptysis. The markers of inflammation were elevated with anemia and a normal coagulation profile. The patient had bilateral lower limb edema with tender calf muscles. Color Doppler ultrasound revealed bilateral deep vein thrombosis. Importantly, an urgent computerized tomography pulmonary angiography (CTPA) revealed bilateral large PAAs which matched the pattern of pseudoaneurysms described by the HSS international study group (HSSISG). There was no history of iridocyclitis, recurrent oral or genital ulcers. Accordingly, the patient was diagnosed with HSS. The patient received intravenous pulse methylprednisolone (1 g/3 days), then oral prednisone (1 mg/kg/d/3 months), and monthly pulse cyclophosphamide (1 g) for three months. The patient remained symptom-free, yet on the third pulse of cyclophosphamide; he patient contracted corona virus disease-2019 (COVID-19) infection and died three weeks later from a fatal episode of massive hemoptysis. The case was discussed and the recent literature was reviewed relative to fatal hemoptysis associated with pulmonary artery pseudoaneurysms (PAPs) pattern in HSS

Conclusion

HSS presenting with massive hemoptysis could be lifethreatening and the PAPs are considered a double edged sword. Detailed description of such rare cases is warranted for optimum future management.