SESSION TITLE: Pulmonary Vascular Disease Student/Resident Case Report Posters II
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM
INTRODUCTION: Hughes-Stovin syndrome(HSS) is an exceedingly rare and widely under-recognized phenomenon despite its high mortality rate1. The underlying pathophysiology and treatment is not clearly understood owing to the lack of quality case studies. We describe a case of HSS requiring anticoagulation at presentation and later successfully suppressed with steroids, to add to the paucity of the current knowledge.
CASE PRESENTATION: 27 year old male with a history of Behcet's syndrome presented with shortness of breath on exertion for 2 days associated with hemoptysis. He gave no history of chest pain, fever, immobilization or calf swelling. He had a past history of uveitis and apthous oral ulcers but no DVT/PE. CTA thorax revealed multiple filling defects in pulmonary arteries(PA) consistent with extensive pulmonary emboli(PE) and pulmonary infarcts. Also seen was a single small aneurysmal dilatation of PA. Doppler of lower extremities confirmed venous thrombosis but echocardiography showed no evidence of right heart strain. Patient was started on heparin drip due to extensive (PE) and pulse dose methylprednisolone. Rheumatology workup showed elevated complements and ESR while ANCA was negative. Patient noted improvement in breathing status and he was discharged home in medically stable condition with prednisone taper and warfarin bridged by enoxaparin. Patient has not had any further episodes of hemoptysis or dyspnea in 3 months of follow up.
DISCUSSION: Behcet’s syndrome(BS) is a multisystem vascular disease occasionally affecting the pulmonary arteries via destruction of the vessel wall and perivascular infiltration. Hughes-Stovin syndrome is considered a variant of BS characterized by pulmonary artery aneurysms and venous thromboembolism. Due to its rarity, the pathogenesis of the disease is not clearly understood but some theories include direct endothelial damage from the autoantibodies, concomitant presence of antiphospholipid antibodies or hyperhomocysteinemia 2 Management entails steroids and cytotoxic agents like cyclophosphamide. Anticoagulants are usually avoided due to risk of bleeding from aneurysms except in cases with significant thromboembolism 3, but there is lack of clinical data to prove mortality benefit.
CONCLUSIONS: 1. The actual incidence of HSS may be higher than expected as the majority of the cases remain undiagnosed or under-reported. 2. Early diagnosis and proper treatment with immunosuppressive therapy can improve survival 3 3. There is lack of clinical trials to prove the efficacy of anticoagulants in patients with significant embolism.
Reference #1: Kanchinadham S, Potikuri D. Multiple pulmonary arterial aneurysms in a young male patient with incomplete behcet's syndrome. Lung India. 2013;30(1):76-77.
Reference #2: Hammami S, Mahjoub S, Ben-Hamda K, Brahem R, Gamra H, Ben Farhat M. Intracardiac thrombus in behcet's disease: Two case reports. Thromb J. 2005;3:9.
Reference #3: Khalid U, Saleem T. Hughes-stovin syndrome. Orphanet J Rare Dis. 2011;6:15-1172-6-15.
DISCLOSURE: The following authors have nothing to disclose: Gaurang Vaidya, Wajihuddin Syed, Shalin Kothari, Omair Chaudhary
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