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Abstract: BACKGROUND AND OBJECTIVES: 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.
Fuente: Semin Arthritis Rheum. 2015 Apr;44(5):514-7
Editorial: W.B. Saunders
Fecha de publicación: 01/04/2015
Nº de páginas: 4
Tipo de publicación: Artículo de Revista
LEYRE RIANCHO ZARRABEITIA
FELIPE ZURBANO GOÑI
JOSE JAVIER GOMEZ ROMAN
MARTÍNEZ MEÑACA, AMAYA
MARTA LOPEZ SANCHEZ
MIGUEL ANGEL HERNANDEZ HERNANDEZ
PINA MURCIA, TRINITARIO
MIGUEL ANGEL GONZALEZ-GAY MANTECON