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59 Cards in this Set
- Front
- Back
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congenital cystic adenomatoid malformation CCAM
what is it? |
Hamartomatous mass of lung tissue: disorganized with varying degrees of cystic changes
Increase in terminal bronchiole like structures Polypoid growth of cuboidal epithelium and increased underlying stromal elastic and smooth muscle Mass lesion Some alveoli between cysts |
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clinical presentation of congenital cystic adenomatoid malformation (CCAM)
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Clinical:
Occurs in stillborns or newborns in respiratory distress Stillborn (Anasarca) Mass obstruction of vena cava blocks venous return to heart Newborn Related to mass expansion in one hemithorax M>F Unilateral Segmental bronchial atresia or absence |
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Treatment of congenital cystic adenomatoid malformation CCAM
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Tx.
Resection |
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cystic fibrosis pathology findings
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Pathology:
Nonspecific Wide spread bronchiecasis particularly in upper lobes Chronic inflammation, fibrosis, and irregular thickening of alveolar wall Pulmonary complications: Atelectasis Pneumothorax Hemoptysis Acute respiratory failure Chronic respiratory failure Cor pulmonale |
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primary ciliary dyskinesia
pathology findings |
immotile cilia syndrome
Pathology: Bronchiectasis Bronchitis Bronchiolitis Abnormal cilia Absent/shortened dynein arms Absence of radial spokes Microtubular disarrangement or absence Ciliary disorientation |
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kartagener's syndrome
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subset of primary cilliary dyskinesia
Kartagener’s Syndrome – subset that includes: Bronchiectasis Sinusitis Situs inversus |
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COPD
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chronic bronchitis and emphysema
primary cause is cigarette smoking |
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types of emphysema
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(1) Centrilobular emphysema – dilation primarily in respiratory bronchioles
Smoking (2) Panlobular emphysema – enlargement and destruction of airspaces with uniform involvement of the acinus Alpha 1 anti trypsin deficiency |
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Wegener's granulomatosis
clinical triad |
disorder of pulmonary vasculature
Clinical triad: Upper airway Lower airway Kidney |
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pathologic findings of wegener's granulomatosis
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Remission:
40% cytoplasmic anti-neutrophilic antibody positive C-ANCA Pathologic Triad Parenchymal necrosis - center Vasculitis Granulomatous inflammation - border Pathologic variants Eosinophil-rich Bronchiolocentric Solitary Capillaritis Diffuse pulmonary hemorrhage |
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necrotizing capillaritis
associated with? |
Collagen vascular disease
SLE Wegener’s granulomatosis Henoch-schonlein purpura Cryoglobulinemia Goodpasture’s |
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pathologic findings in necrotizing capillaritis
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Pathologic findings:
Focal necrosis of alveolar septa Neutrophilic infiltration Fragmented neutrophils Capillary fibrin thrombi Interstitial hemorrhage |
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Chrug-strauss syndrome
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Allergic Angiitis and Granulomatosis
Asthma Systemic vasculitis Paranasal sinus abnormalities Eosinophilia Elevated serum IgE p-ANCA |
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pathologic features of Chrug-strauss syndrome
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Pathologic Features:
Eosinophilic infiltrates Granulomatous infiltration Necrotizing vasculitis |
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pulmonary hypertension
risk factors |
Heart disease, chronic thromboembolic disease, underlying pulmonary disorder
Risk factors: F>>M 30s female, 40s male Familial Autosomal dominant Drugs Diseases – HIV |
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pathologic features of pulmonary hypertension
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Pathologic features:
Medial hypertrophy and muscularization of arterioles Cellular proliferation of intima Concentric laminar intimal fibrosis Fibrinoid necrosis Dilatation lesions Plexiform lesions in severe |
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Diffuse alveolar damage (DAD)
clinical presentation |
Acute onset of dyspnea
Rapid respiratory failure Patchy, bilateral with ground glass appearance |
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hamman rich syndrome
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variant of AIP
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Diffuse alveolar damage pathology
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Pathology:
Temporally homogenous – all damage occurs at the same time Stages: (1)Exudative Stage – Interstitial edema Type I pneumocyte sloughing – undergo extensive necrosis and slough off alveolar surface Denudation of underlying basement membrane becomes surface for hyaline membrane attachment Hyaline membranes Edema and exudation of plasma proteins into alveolar interstitium and spaces Eosinophilic membranes line alveolar surfaces consists of precipitated plasma proteins, cytoplasmic, and nuclear debris from sloughed epithelial cells (2)Organizing Stage Proliferating type II pneumocytes - hyperplastic Interstitial fibroblasts – loose early fibrosis in the alveolar spetal walls Focal airspace organization |
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cryptogenic organizing pneumonia (COP) also called (BOOP)
clinical presentation |
Clinical Presentation:
Subacute onset of cough, dyspnea, fever Multiple patchy airspace opacities bilaterally |
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BOOP timing
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homogenous
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DAD timing
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heterogenous
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BOOP histology
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Histology:
Patchy – adjacent to normal lung zones Intraluminal loose/immature connective tissue (like organizing fibrosis) in distal airspace Particularly in alveolar ducts Preservation of lung architecture Minimal or lack of interstitial connective tissue/fibroblasts No hyaline membranes Intraluminal plug of immature connective tissue filling alveolar duct Fibroblasts lack mature collagen fibers |
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BOOP tx
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steroids
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Diffuse Alveolar Hemorrhage (DAH)
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Goodpastures
Idiopathic pulmonary hemosiderosis |
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Goodpasture's syndrome
clinical presentation |
M>>>F
Young adults Smokers HLA: DRw15, DQw6 Anti-body mediated immune reaction to basement membrane IgG in both lung and kidney |
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Goodpasture's findings
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Hemoptysis with:
Acute and or chronic hemorrhage Anemia Renal insufficiency Diffuse lung infiltrates Linear IgG staining of basement membrane |
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Idiopathic pulmonary hemosiderosis
clinical |
M:F is equal
Adolescents and children Hemoptysis and chronic hemorrhage – sometimes acute No renal involvement Etiology unknown |
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idiopathic pulmonary hemosiderosis pathologic finding
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hemosiderin-laden macrophages in alveolar spaces
coarse granules of hemosiderin fill alveolar spaces |
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Pulmonary Alveolar Proteinosis (PAP
clinical |
Predominantly affects adults 30-50
Associated with autoimmunity, dust, drugs, infection, idiopathic Clinical presentation: Cough – non productive or productive with expectoration of gelatinous material Impaired clearance of alveolar surfactant by alveolar macrophages GM-CSF implicated – defective alveolar macrophage function IgG to GM-CSF |
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Non-caseating granulomatous lung disease
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sarcoidosis
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sarcodiosis clinical presentation
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Clinical Presentation:
Multiorgan disease: lungs, lymphatics, liver, spleen, eyes Wide variety of symptoms – asymptomatic the most common Symptomatic – dyspnea with or without exertion, nonproductive cough, nonspecific chest pain Young adults: 20-40 F>M African Americans most common in US Irish and Scandinavians outside US |
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sarcoidosis dx
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Noncaseating granulomas in lymphatic and bronchovascular distribution
Exclusion of other disorders |
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etiology pathology of sarcoidosis
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Etio/Path:
Unknown Hieightened/exaggerated helper Tcell type I immune response Depression of cutaneous delayed type hypersensitivity Elevated circulating immune complexes and signs of B cell hyperactivity |
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sarcoidosis findings
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Findings:
Symmetric, bilateral hilar and mediastinal lymphadenopathy Mild reticulonodular interstitial infiltrate in perihilar regions Nodules with irregular borders along pulmonary vessels and bronchi Later stages: interstitial fibrosis Cavitary lesions Honeycomb change Subpleural cysts in apex |
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sarcoidosis histology
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Histology:
Non caseating granulomas Well formed or tightly packed, may become hyalinized Cluster of epitheliod cells and giant cells Distributed along lymphatic routes All cultures are negative Vasculitis Punctuate necrosis Inclusions: Schaumann bodies, Asteroid bodies, birefringent calcium carbonate, calcium oxalate crystals, microcalcifications, hamazaki-wesenberg bodies Infiltrating pleura and the interlobular septa surrounded by concentric fibrosis |
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sarcoidosis complications and tx
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Complications: interstitial fibrosis with honeycombing in advanced stages
Cavitation/cystic changes Aspergillomas Tx. Usually remits, but can give steroids At risk for aspergilloma |
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Hypersensitivity pneumonitis
clinical |
Diffuse
Interstitial granulomatous lung disease Immunologic reaction to inhaled organize antigens Immune complex mediate – Type III and T cell mediated, delayed Type IV hypersensitivity IgG antibodies in serum common Symptoms can develop hours, weeks, or months after exposure Mild or subclinical – is the first reaction to exposure (need multiple exposure to be severe) Farmers lung, bird fancier lung |
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hypersensitivity pneumonitis pathology
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Pathology:
Temporally homogenous Acute HP Neutrophilic infiltrate in alveoli Sometimes pattern of DAD Subacute HP Lymphocytic interstitial pneumonitis, granulomas, organizing pneumonia, fibrosis Chronic HP Most biopsies performed at this stage Foamy macrophages Progression Pleural fibrosis |
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timing of hypersensitivity pneumonitis
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homogenous
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Cryptostroma corticale
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– Maple Bark Strippers Disease
Nodules of interstitial inflammation centered on bronchioles |
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dx of hypersensitivity pneumonitis
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Dx. Traid of chronic…by exclusion of others first
Temporally uniform chronic interstitial inflammation with peribronchiolar accentuation Bronchiolocentric cellular interstitial pneumonia Poorly formed granulomas Foci of BOOP/COP |
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tx of hypersensitivity pneuomonitis
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Removal of causative antigen
Steroids as last resort |
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usual interstitial pneumonia UIP
clinical presentation |
Insidious onset of dyspnea with chronic progressive downhill course
M>>Females 50-70 years |
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pathogenesis of UIP
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Pathogenesis:
Intrinsic abnormality of tissue repair TGF Beta1 and caveolin 1 suggested role |
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physical findings of UIP
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Physical Findings:
Tachypneic – with rapid shallow breaths Crackles bibasilar, late inspiratory, fine crackles Clubbing, pulmonary hypertension and cyanosis late |
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histology of UIP
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Arterial gas – hypoxemia at rest
Histology: Patchy lung involvement Temporally heterogeneous Most prominent in subpleural and peripheral lobular regions Dense fibrosis with remodeling of lung architecture with honeycombing No granulomas Usually lower lobes |
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dx of UIP
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Dx.
Requires open lung biopsy Cannot sue transbronchial biopsy Can have lung carcinoma |
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tx of UIP
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Tx.
Steroids But in general bad prognosis |
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non-specific interstitial pneumonia
NSIP clinical presentation |
40-50 year olds
F>M Correlated with smoking Subacute for weeks to months Exertional dyspnea Cough Fever Crackles Clubbing |
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radiographic findings in NSIP
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Radiographic:
Variable and nonspecific Ground glass opacities of lower lobes Mimics DIP and HP |
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NSIP pathologic findings
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Pathologic Findings:
Temporally homogeneous Inflammation and fibrosis Diffuse lung involvement Architecture of lung is preserved Alveolar walls are uniformly thickened by fibrosis |
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NSIP histology
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Histology:
Dense or loose interstitial fibrosis lacking temporal heterogeneity Lung architecture preserved Mild inflammation No fibroblastic foci Lack of airway centricity Granulomas and eosinophils Lack of viral inclusions and organisms |
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NSIP tx
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steroids
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dx of NSIP
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by exclusion
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desquamative interstitial pneumonia DIP
clinical |
90% smokers
Clinical presentation: Subacute for weeks or months Insidious dyspnea and cough M>F 40s/50s |
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DIP physical findings
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Physical exam:
Crackles Clubbing Lung Function: Restrictive Bilateral ground glass in lower Irregular linear in subpleural regions |
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DIP histology
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Histology:
Diffuse, marked, intra-alveolar macrophage accumulation Minimal fibrosis No scaring fibrosis – get remodeling of lung architecture Temporally homogeneous Thickened alveolar wall Focal DIP reactions around mass lesions – infarcts and tumors |
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DIP Tx
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Tx.
Stop smoking Corticosteroids Lung transplant Prognosis: Good |
