Cell Nevus Syndrome manifestations in nonCaucasian 在非白种人的基底细胞痣综合征表现课件.pptVIP

Morning Report Anne Lachiewicz September 22, 2009 Broncholitis obliterans organizing pneumonia (BOOP) Cryptogenic organizing pneumonia Inflammatory lung disease Intraluminal organizing fibrosis in distal airspaces (bronchioles, alveolar ducts, and alveoli) Reversible fibrosis Dx by lung bx. VAT preferred to transbronchial bx to obtain sufficient tissue to exclude other diagnoses with overlapping features Classification of BOOP Idiopathic (most common) Rapidly progressive Focal nodular Postinfection Drug-related (case reports) Rheumatologic or connective tissue related Immunologic disorder Organ transplantation (lung, bone marrow) Radiotherapy (breast cancer) Environmental exposures Miscellaneous Histologic features Intra-alveolar stages Fibrinoid inflammatory cell clusters, fibrin bands with cells (esp. lymphocytes) Fibroinflammatory buds, fibroblasts migrate, proliferate develop Mature fibrotic buds: concentric rings of fibroblasts alternating with layers of connective tissue (mainly collagen bundles, few inflammatory cells (usually in the center of the buds) Mild interstitial chronic inflammation in alveolar walls with reactive type II cells Increased foamy macrophages in the alveoli without buds Features Preservation of lung architecture Patchy distribution, rarely unilateral Uniform temporal appearance Absence of interstitial fibrosis granulomas neutrophils or abscesses necrosis hyaline membranes eosinophilic infiltration vasculitis Clinical presentation Males = Females Mean age 50-60 years 2x more in non-smokers/ex-smokers Flu-like illness, malaise, fever Persistent nonproductive cough Progressive mild dyspnea Weight loss of greater than 10 pounds (57%) Inspiratory rales or focal sparse crackles 25% normal pulmonary exam No clubbing Studies Labs: 50% leukocytosis, elevated ESR, CRP, no eosinophilia PFTs: mild-mod restrictive defect, only 20% obstructive (all former smokers) BAL: Mixed pattern (mild increase in lymphocytes, neutrophils, eosinophils) Imaging: