Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 28-year-old woman with a eight-year history of rheumatoid arthritis presented with a three-day history of dyspnea. Physical and electrocardiographic findings were consistent with pulmonary hypertension. Arterial blood gases revealed a ventilation-perfusion mismatch. Chest roentgenogram was normal. After transient improvement, she suddenly deteriorated and died. At autopsy, a necrotizing pulmonary panarteritis was found without parenchymal involvement by rheumatoid disease. The pulmonary arteries were the only vessels affected. Immunofluorescent staining revealed immunoproteins scattered throughout the vessel walls without localization to the basement membrane. The unique features of the case are discussed in relation to pulmonary hypertension and rheumatoid lung disease in which vascular lesions are usually associated with honeycomb lung. The association between the rheumatoid arthritis and pulmonary vasculitis was probably coincidental.
 ...
PMID:Acute respiratory failure and pulmonary arteritis without parenchymal involvement: demonstration in a patient with rheumatoid arthritis. 44 48

In the pathogenesis of hypersensitivity pneumonitis several immune mechanisms are in play. The initial phase appears to be an immune complex reaction occurring 4 to 8 hours after antigen inhalation, and is characterized by an early increase in neutrophils in the alveolar washing and histopathologically there are oedema, neutrophil infiltration in the alveolar walls, and vasculitis. After 12 hours and for several days there is a cell mediated immune response and the alveolitis consists of cytotoxic cells and suppressor cells whose target is perhaps to modulate the diminution of the B cell response in the production of antibodies by plasma cells. During this phase the lymphocytes of CD8 positive phenotype, the NK cells and occasionally some plasma cells are increased in the alveolar lavage. The characteristic histological appearance is a mononuclear infiltrate predominantly of lymphocytes and some plasma cells and foamy macrophages. After several weeks and for several months a delayed hypersensitivity reaction may lead to a discrete increase in the CD4 cells in the broncho-alveolar lavage and the formation of granulomas. Finally after several months and for several years the repeated immunological activity and damage to the alveolar wall leads to the secretion of proteolytic enzymes and to growth factors to attract fibroblasts leading to pulmonary fibrosis and to a honeycomb lung with a concomitant increase in alveolar neutrophils as in other diseases characterised by pulmonary fibrosis.
 ...
PMID:[Alveolitis in hypersensitivity pneumopathies]. 265 1

We herein report the case of a 72-year-old woman with diffuse cutaneous systemic sclerosis (SSc) complicated by antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis who exhibited honeycomb lung without volume loss. On admission, chest computed tomography (CT) revealed honeycomb lung without volume loss in addition to increased density of the partition walls. A renal biopsy revealed global sclerosis and cellular crescent formation. Mononeuritis multiple subsequently occurred, and steroid pulse therapy with cyclophosphamide was administered. Repeat chest CT showed that the honeycomb lung was unchanged; however, overall reduced density of the partition walls was observed. It is necessary to recognise that vasculitis may develop in SSc patients who exhibit honeycomb lung without volume loss.
...
PMID:A patient with diffuse cutaneous systemic sclerosis complicated by antineutrophil-cytoplasmic antibody-associated vasculitis exhibiting honeycomb lung without volume loss. 2469 1