cell nevus syndrome manifestations in noncaucasian 在非白种人的基底细胞痣综合征表现课件

Morning Report Anne Lachiewicz September 22, 2009 Broncholitis obliterans organizing pneumonia (BOOP) nCryptogenic organizing pneumonia nInflammatory lung disease nIntraluminal organizing fibrosis in distal airspaces (bronchioles, alveolar ducts, and alveoli) nReversible fibrosis nDx by lung bx. VAT preferred to transbronchial bx to obtain sufficient tissue to exclude other diagnoses with overlapping features Classification of BOOP nIdiopathic (most common) nRapidly progressive nFocal nodular nPostinfection nDrug-related (case reports) nRheumatologic or connective tissue related nImmunologic disorder nOrgan transplantation (lung, bone marrow) nRadiotherapy (breast cancer) nEnvironmental exposures nMiscellaneous nIntra-alveolar stages nFibrinoid inflammatory cell clusters, fibrin bands with cells (esp. lymphocytes) nFibroinflammatory buds, fibroblasts migrate, proliferate 161:158-164. A, Intraluminal organization and polypoid granulation tissue within a small bronchiole nPreservation of lung architecture nPatchy distribution, rarely unilateral nUniform temporal appearance nAbsence of ninterstitial fibrosis ngranulomas nneutrophils or abscesses nnecrosis nhyaline membranes neosinophilic infiltration nvasculitis Features nMales = Females nMean age 50-60 years n2x more in non-smokers/ex-smokers nFlu-like illness, malaise, fever nPersistent nonproductive cough nProgressive mild dyspnea nWeight loss of greater than 10 pounds (57%) nInspiratory rales or focal sparse crackles n25% normal pulmonary exam nNo clubbing Clinical presentation Studies nLabs: 50% leukocytosis, elevated ESR, CRP, no eosinophilia nPFTs: mild-mod restrictive defect, only 20% obstructive (all former smokers) nBAL: Mixed pattern (mild increase in lymphocytes, neutrophils, eosinophils) Imaging: 3 main patterns of COP nTypical multiple alveolar opacities, usually bilateral, peripheral, and often migratory, may see an air bronchogram nFocal solitary opacity, often upper lobes and may be cavitary, not a characteristic pattern, may have false + FDG-PET uptake nInfiltrative infiltrative opacities associated with interstitial 161:158-164. A, Chest radiograph of a 54-year-old man with a flulike illness, bilateral crackles, decreased vital capacity, and a decreased diffusing capacity that shows bilateral patchy infiltrates in the lower lungs Treatment 26:422- 446. nEpler, GR. Bronchiolitis obliterans organizing pneumonia. Arch Inten