Hughes–Stovin syndrome with pulmonary aneurysms and renal dysfunction mimicking polyarteritis nodosa. Issue 1 (2nd January 2018)

• Record Type: Journal Article
• Authors: Watanabe, Eri; Nishina, Kumiko; Yabe, Hiroki; Gono, Takahisa; Terai, Chihiro
• Is Part Of: Modern rheumatology case reports. Volume 2:Issue 1(2018)
• Page Start: 107
• Page End: 112
• Publication Date: 2018-01-02
• Subjects: Aneurysm -- Behçet's disease -- deep vein thrombosis -- Hughes–Stovin syndrome -- polyarteritis nodosa; Rheumatology -- Periodicals; 616.723005
• DOI: 10.1080/24725625.2017.1379168
• Languages: English
• ISSNs: 2472-5625

Abstract

Abstract: Hughes–Stovin syndrome (HSS) is an extremely rare condition characterised by pulmonary artery aneurysms and deep vein thrombosis (DVT). We report the case of a 34-year-old Senegalese man who initially presented with fever, hemoptysis, and cough. Radiological and laboratory evaluation revealed right pulmonary artery aneurysms, intense inflammatory reaction, and renal dysfunction with protein and occult blood in urine. Vasculitis-like polyarteritis nodosa was suspected, and the patient was treated with oral high-dose prednisolone (PSL) followed by intravenous cyclophosphamide (IVCY). In the course of treatment, he developed bilateral leg edema, and DVT was revealed; he was finally diagnosed with HSS. As the findings of thrombi in the inferior vena cava extend to the level of the renal vein, the cause of renal dysfunction was suspected to be renal vein thrombosis. Continuing treatment with oral PSL and IVCY successfully improved his condition, resulting in remission.