title: Nonthrombotic proliferative vasculopathy associated with antiphospholipid antibodies: A case report and literature review.
abstract: A 20-year-old man presented with recurrent hemoptysis for seven months. A small subpleural nodule in his right lower lobe was found and excised surgically. Based on the presence of antiphospholipid antibodies (aPL) and vascular wall hypertrophy without vasculitis or an intraluminal thrombus, nonthrombotic proliferative vasculopathy (NTPV) affecting pulmonary arteries was diagnosed. Recently, aPL have been postulated to directly induce the proliferation of vascular cells in the intima and media, leading to NTPV. We review 5 cases of NTPV-associated aPL with critical ischemia in the lower extremities and gastrointestinal infarction. NTPV-associated aPL might be distinct from classic antiphospholipid syndrome and should be considered in aPL-positive patients who present with vascular occlusions of medium-sized vessels in the absence of atherosclerotic risk factors and systemic or local inflammation.
authors.affiliation: a Division of Rheumatology, Department of Internal Medicine , Seoul National University Hospital , Seoul , Korea.|b Department of Pathology , Seoul National University Hospital , Seoul , Korea , and.
authors.forename: Jeong Seok
authors.identifier: http://orcid.org/0000-0001-8261-7044
authors.initials: JS
authors.lastname: Lee
authors.affiliation: b Department of Pathology , Seoul National University Hospital , Seoul , Korea , and.
authors.forename: Hyojin
authors.identifier: http://orcid.org/0000-0001-9201-8328
authors.initials: H
authors.lastname: Kim
authors.affiliation: a Division of Rheumatology, Department of Internal Medicine , Seoul National University Hospital , Seoul , Korea.
authors.forename: Eun Bong
authors.identifier: http://orcid.org/0000-0003-0703-1208
authors.initials: EB
authors.lastname: Lee
authors.affiliation: a Division of Rheumatology, Department of Internal Medicine , Seoul National University Hospital , Seoul , Korea.|c Department of Molecular Medicine and Biopharmaceutical Sciences , BK 21 plus Graduate School of Convergence Science and Technology, College of Medicine, Medical Research Institute, Seoul National University , Seoul , Korea.
authors.forename: Yeong Wook
authors.identifier: http://orcid.org/0000-0002-5384-3437
authors.initials: YW
authors.lastname: Song
authors.affiliation: a Division of Rheumatology, Department of Internal Medicine , Seoul National University Hospital , Seoul , Korea.|c Department of Molecular Medicine and Biopharmaceutical Sciences , BK 21 plus Graduate School of Convergence Science and Technology, College of Medicine, Medical Research Institute, Seoul National University , Seoul , Korea.
authors.forename: Jin Kyun
authors.identifier: http://orcid.org/0000-0003-2167-9393
authors.initials: JK
authors.lastname: Park
mesh_terms: D017152:Antibodies, Antiphospholipid / Q000032:analysis*; D016736:Antiphospholipid Syndrome / Q000150:complications / Q000175:diagnosis / Q000276:immunology; D049109:Cell Proliferation; D003937:Diagnosis, Differential; D006469:Hemoptysis / Q000175:diagnosis / Q000209:etiology; D006801:Humans; D008297:Male; D011651:Pulmonary Artery / Q000276:immunology / Q000473:pathology; D017539:Tunica Intima / Q000473:pathology / Q000503:physiopathology; D017540:Tunica Media / Q000473:pathology / Q000503:physiopathology; D014657:Vasculitis / Q000175:diagnosis / Q000209:etiology / Q000276:immunology / Q000503:physiopathology; D055815:Young Adult
chemical_list: D017152:Antibodies, Antiphospholipid
keywords: Antiphospholipid antibodies; Antiphospholipid syndrome; Medium-sized vessel; Proliferative vasculopathy; Pulmonary artery; Vasculitis