title: Isolated pulmonary vasculitis: case report and literature review.
abstract: OBJECTIVE
  Single-organ vasculitis has been reported to affect the skin, kidneys, central nervous system, peripheral nerves, genitourinary tract, calf muscles, aorta, coronary arteries, retina, or gastrointestinal tract. However, isolated pulmonary vasculitis is a very rare entity. Our aims were to describe a case of localized pulmonary vasculitis affecting medium-sized vessels and review the literature.
  
  
  METHODS
  A patient with localized pulmonary vasculitis affecting medium-sized vessels that presented as pulmonary arterial hypertension is described. A MEDLINE database search of cases with localized pulmonary vasculitis was also conducted.
  
  
  RESULTS
  A 30-year-old man presented with pulmonary hypertension due to isolated pulmonary medium-sized vessel vasculitis that was confirmed histologically. Initially he responded to corticosteroids and vasodilator treatment, but therapy eventually lost efficacy. Treatment with rituximab was not effective, and as the clinical situation worsened, lung transplant was performed. Isolated large pulmonary vessel disease, often related to Takayasu disease or giant cell arteritis, may present as pulmonary artery hypertension, thus mimicking chronic thromboembolic disease. Medium- and small-vessel pulmonary vasculitis usually develops in the context of a systemic disease. Some cases of isolated small-vessel vasculitis have been reported presenting as diffuse alveolar hemorrhage. In contrast, our case developed pulmonary artery hypertension secondary to medium-sized vessels vasculitis. To our knowledge, this is the first case of lung transplantation in isolated pulmonary vasculitis.
  
  
  CONCLUSIONS
  Pulmonary isolated vasculitis is a rare cause of pulmonary hypertension but it must be taken into consideration after more common disorders are excluded.
authors.affiliation: Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain.
authors.forename: Leyre
authors.initials: L
authors.lastname: Riancho-Zarrabeitia
authors.affiliation: Division of Pneumology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain.
authors.forename: Felipe
authors.initials: F
authors.lastname: Zurbano
authors.affiliation: Division of Pathology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain.
authors.forename: Javier
authors.initials: J
authors.lastname: Gómez-Román
authors.affiliation: Division of Pneumology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain.
authors.forename: Amaya
authors.initials: A
authors.lastname: Martínez-Meñaca
authors.affiliation: Division of Intensive Care, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain.
authors.forename: Marta
authors.initials: M
authors.lastname: López
authors.affiliation: Division of Intensive Care, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain.
authors.forename: Miguel A
authors.initials: MA
authors.lastname: Hernández
authors.affiliation: Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain.
authors.forename: Trinitario
authors.initials: T
authors.lastname: Pina
authors.affiliation: Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain; Division of Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa. Electronic address: miguelaggay@hotmail.com.
authors.forename: Miguel A
authors.initials: MA
authors.lastname: González-Gay
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D006801:Humans; D008171:Lung Diseases / Q000188:drug therapy / Q000473:pathology / Q000601:surgery*; D016040:Lung Transplantation; D008297:Male; D019233:Retreatment; D017211:Treatment Failure; D016896:Treatment Outcome; D014657:Vasculitis / Q000188:drug therapy / Q000473:pathology / Q000601:surgery*
chemical_list: D000305:Adrenal Cortex Hormones
keywords: Isolated vasculitis; Lung; Pulmonary; Vasculitis