Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0034067 (emphysema)
 11,506 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) are conditions classified under the general heading of antinuclear cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). Lung lesion is a very common and important clinical feature in AAV. In MPA, diffuse alveolar hemorrhage and pulmonary fibrosis (PF) are the most frequent manifestations. High-resolution computed tomography (HRCT) chest findings associated with MPA in PF patients demonstrate a high frequency of usual interstitial pneumonia (UIP), fibrotic-nonspecific interstitial pneumonia (F-NSIP), and combined PF and emphysema (CPFE) pattern with honeycombing, traction bronchiectasis, ground-glass opacity, and emphysema. In most of these cases, the histologic pattern of PF has been classified as UIP and/or fibrotic NSIP. In addition, a high incidence of histological findings, such as extensive interstitial fibrosis, lymphoid hyperplasia, and bronchiolitis, are characteristics observed in PF associated with collagen vascular diseases and which are not observed in idiopathic PF (IPF). In some cases, PF precedes the development of MPA. Indeed, there are some cases of pulmonary-limited MPA in this group. Therefore, clinicians should be aware of MPA as an underlying feature of PF in order to avoid overlooking and misdiagnosing this condition as IPF. The median survival time (MST) in UIP pattern/MPA is comparable with that of IPF. In GPA, almost all patients have either upper airway or lower respiratory tract lesions. Solitary or multiple nodules (frequently cavitated) and masses are the most common findings on chest images. Asthma is a cardinal symptom of Churg-Straus syndrome, often preceded by allergic rhinitis. To induce remission, a severity-based regimen was given to patients according to the appropriate protocol of the Japanese patients with myeloperoxidase (MPO)-ANCA-associated vasculitis (JMAAV) study group: low-dose corticosteroid and, if necessary, cyclophosphamide or azathioprine in patients with mild form; high-dose corticosteroid and cyclophosphamide in those with severe form; severe-form regimen plus plasmapheresis in those with the most severe form.
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PMID:Pulmonary involvement in ANCA-associated vasculitis from the view of the pulmonologist. 2318 94

Idiopathic pulmonary fibrosis (IPF) is the most frequent and severe idiopathic interstitial pneumonia, with typical high-resolution computed tomography (HRCT) features and histologic pattern of usual interstitial pneumonia (UIP); its main differential diagnosis is fibrotic nonspecific interstitial pneumonia (F-NSIP). Usual interstitial pneumonia was mainly described from lung biopsies, and little is known on explants. Twenty-two UIP/IPF explants were analyzed histologically and compared with previous open lung biopsies (OLBs; n = 11) and HRCT (n = 19), when available. Temporospatial heterogeneity and subpleural and paraseptal fibrosis were similarly found in UIP/IPF explants and OLB (91%-95%). Fibroblastic foci were found in 82% of OLBs and 100% of explants, with a higher mean score in explants (P = .023). Honeycombing was present in 64% of OLBs and 95% of explants, with a higher mean score in explants (P = .005). Almost 60% of UIP/IPF explants showed NSIP areas and 41% peribronchiolar fibrosis; inflammation, bronchiolar metaplasia, and vascular changes were more frequent in UIP/IPF explants; and Desquamative Interstitial Pneumonia (DIP)-like areas were not common (18%-27%). Numerous large airspace enlargements with fibrosis were frequent in UIP/IPF explants (59%). On HRCT, honeycombing was observed in 95% of the cases and ground-glass opacities in 53%, correlating with NSIP areas or acute exacerbation at histology. Six patients had combined IPF and emphysema. Lesions were more severe in UIP/IPF explants, reflecting the worsening of the disease. Usual interstitial pneumonia/IPF explants more frequently presented with confounding lesions such as NSIP areas, peribronchiolar fibrosis, and airspace enlargements with fibrosis sometimes associated with emphysema.
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PMID:Usual interstitial pneumonia end-stage features from explants with radiologic and pathological correlations. 2602 58