Pattern of pulmonary vasculitis and major vascular involvement in Hughes-Stovin syndrome (HSS): brief report of eight cases

Abstract

To describe the pattern of pulmonary artery vasculitis and the characteristic computed tomographic pulmonary angiography (CTPA) signs in patients with Hughes-Stovin syndrome (HSS). In a retrospective study, the medical records of eight HSS patients (six men), seen between February 2008 and January 2018, were reviewed regarding history, disease characteristics, laboratory investigations, imaging, and treatments. The mean (SD) age was 37.375 ± 8.65 years (range 30–55) and mean (SD) follow-up 30 ± 41.60 months (range 9–132). In all patients, routine laboratory investigations and complete coagulation profile were done. In all, CTPA studies were performed as well as and Doppler ultrasound for suspected deep vein thrombosis (DVT). Four patients had a history of thrombophlebitis, and DVT was observed in all, in two cases bilateral. Arterial thromboses involving popliteal, tibial, common iliac, and femoral arteries were observed in one patient. All patients had mild to moderate hemoptysis, and one had massive hemoptysis. None of the patients had a history of recurrent mouth and/or genital ulcers, uveitis, or arthritis. In all patients, CTPA identified bilateral pulmonary artery aneurysms (PAAs) with adherent in situ thrombosis and mural enhancement in all patients. Lobar PA branches were involved in all patients, segmental in six and main PA in five patients. Proper immunomodulators were initiated early, with favorable outcome; none was treated with TNF-α antagonists. HSS is a systemic vasculitis that may affect virtually all major veins and arteries in patients with normal coagulation profile. PAAs, adherent in situ thrombosis, and mural wall enhancement are characteristic CTPA signs. Early treatment with immunomodulators is essential.Key Points• Hughes Stevin syndrome (HSS) is a systemic vasculitis that may affect virtually all major veins and arteries in patients. It has a normal coagulation profile.• Computed tomography (CT) pulmonary angiography is considered to be the most important diagnostic tool to assess the degree and the extent of the characteristic pulmonary artery aneurysms, and in situ thrombosis, and mural wall enhancement.• It is likely that HSS syndrome is often not recognized and misdiagnosed as deep venous thrombosis (DVT) with pulmonary thromboembolism.• Early treatment with combined immunomodulators is essential to ensure favorable outcome.