08.18 - Obstructive, Restrictive Dz (Nichols) - Questions Flashcards
Bronchiectasis: Obstructive or Restrictive?
Obstructive
Pneumoconiosis: Obstructive or Restrictive?
Restrictive
Sarcoidosis: Obstructive or Restrictive?
Restrictive
4 types of Non-infectious interstitial lung disease
Usual Interstitial Pneumonia; Crytogenic Organizing Pneumonia; Nonspecific Interstitial Pneumonia; Radiation Pneumonitis
3 cytokines involved in pathogenesis of emphysema
IL-8, TNF, and LeukotrieneB4
What causes pulmonary HTN in emphysema
Decr pulmonary vascular capacitance –> Same blood thru smaller vessels –> Incr pulmonary arterial pressure
Pulmonary arterial pressure in Emphysema
Increased –> Cor Pulmonale
Emphysema due to smoking is usually ___-acinar
Centri-
Type of emphysema in young male smokers with spontaneous pneumothorax
Distal Acinar
Most typical symptom of pure emphysema
Insidious onset of progressive dyspnea
Mucus hypersecretion thought to be mediated by what cytokine and incr transcription of what gene
IL-13; MUC5AC
From where do the cytokines originate that cause mucus hypersecretion in CB
T Cells –> IL-13
Symptom of CB
Productive Cough
Distinctive, defining feature of CB
Bronchial hypersecretion of mucus
Bronchiectasis is permanent dilation of bronchi due to ____ by ___
destruction of muscle and elastic tissue by necrotizing infection
3 underlying states leading to infection causing Bronchiectasis
(1) CF; (2) Immunodeficiency; (3) Kartagener Syndrome
What is Kartaganer Syndrome
Ciliary impairment
Localized bronchiectasis can occur
distal to a bronchial tumor or from necrotizing pneumonia
Symptoms of Bronchiectasis
Chronic persistent cough productive of copious purulent sputum
Signs of Bronchiectasis
Expiratory Rhonchi
Early acute phase asthma
IgE, Mast Cells, Histamine, Leukotriene B4, Vagus Nerve
Late Acute Phase Asthma
Eosinophils, Neutrophils, Lymphs, IL-1, TNF, IL-6, Leukotrienes C4, D4, E4, PGD2, PAF
Asthma exhibits ___-type inflammation
TH2-type
Three C’s of Asthma
Curschmann’s Spirals; Charcot-Leyden Crystals; Creola Bodies
Curschmann’s Spirals
Small whorled mucus strands
Creola Bodies
Fragments of degenerated sloughed respiratory epithelium
Presence of goblet cells in bronchioles
Not normally present –> Appear in Asthma
Pneumoconioses
Fibrosing restrictive lung diseases caused by inhalation of particles
Fibrosing restrictive lung diseases caused by inhalation of particles
Pneumoconioses
Coal Worker’s: Activation of __ and __ production leads to fibrosis
Activation of Inflammasome and IL-1 production
What percent of Coal Worker’s results in progressive massive fibrosis
Less than 10%
Most prevalent chronic occupational disease in the world
Silicosis
Ingestion of Silica by alveolar macrophages leads to release of
TNF, IL-1, Fibronectin, ROS, and TGF-B –> Fibrosing chronic inflammation
Gross pathology of Silicosis
Discrete, palpable grey-tan nodules w/ or w/out anthracotic pigment
Microscopic pathology of Silicosis
Nodules of concentrically arranged hyalinized collagen
Tissue response to asbestos
Coated with iron and elicit fibrosis
Signs of Asbestosis
Basal pulmonary crackles
Symptoms of Asbestosis
Dyspnea and persistent dry cough
4 most common sites of sarcoidosis
Lung, Lymph Node, Eye, Skin
Sarcoid: stimuli cause ___ response, mediated by ___
TH1 CD4 response, mediated by IL2, IFN-g, and TNF-A
Role of IL-8 in Sarcoid
attract neutrophils
Sarcoid: IL-4 mediates
later transition to fibrosing inflammation; chemoattractant to fibroblasts
later transition to fibrosing inflammation; chemoattractant to fibroblasts
IL-4
Type of granulomas in Sarcoidosis
Non-caseating; Non-necrotic; Tight naked
Pulmonary signs of Sarcoidosis
Usually none
Classic CXR of Sarcoidosis
Reticulonodular pulmonary infiltrates and bilateral hilar lymphadenopathy
Reticulonodular pulmonary infiltrates and bilateral hilar lymphadenopathy
Sarcoidosis, Histoplasmosis, Tuberculosis
Distribution of granulomas in Sarcoidosis
Lympharangitic distribution along bronchovascular bundles and in bilateral hilar lymph nodes
Symptoms and Signs of UIP/IPF
Insidious onset of dyspnea; Pulmonary crackles
Gross pathology of UIP/IPF
Large discrete scars, making lung firmer and more gray than usual; Visceral pleura has nodularity
Microscopic Hallmark of UIP/IPF
Fibroblast foci of immature fibrosis bulging into alveoli from interstitium
Definitive dx of UIP/IPF is made by
lung biopsy
Treatment of UIP/IPF
Transplantation
Why is it important to differentiate UIP from COP and NSIP
COP and NSIP respond to steroid therapy
Epidemiology of COP
Late middle age non-smokers
Histologic Hallmark of COP
Masson Bodies: Plugs of fibrosing granulation tissue in the alveoli
Masson Bodies
Plugs of fibrosing granulation tissue in the alveoli: COP
Symptoms and Signs of COP
Present with cough, sometimes productive; Dry inspiratory crackles
CXR of COP
Ground Glass Bilateral Opacities
Tx of COP
Steroids
Epidemiology of NSIP
Middle age women non-smokers
Presentation of NSIP
Dyspnea and Cough
CXR of NSIP
Bilateral Ground Glass Opacities
Microscopic features of Radiation Pneumonitis
Atypical Type 2 Pneumocyte Hyperplasia and blood vessel injury
Young male smoker with spontaneous pneumothorax
Distal Acinar Emphysema
Top relevant Interleukin in Asthma vs COPD
IL-5; IL-8
Charcot-Leyden crystals are composed of
Galectin-10
UIP is usually due to
Genetically-determined aberrant healing from aspiration of gastric contents, smoking, exposure to toxins
Temporality of UIP
Heterogenous
Lobar location of UIP
Worse in peripheral lower lobes
UIP genetic predisposition in many patients
TERT or TERC genes
Fibroblast Foci
Histologic Hallmark of UIP
Masson Bodies are found in
COP
Temporality of NSIP vs UIP
Homogenous vs Heterogenous
Number of infections/injuries in NSIP, UIP, COP
1 in NSIP and COP; repeated in UIP
Patients with Silicosis are more prone to __ due to ___
Tuberculosis, paralyzed macrophages
Tight, well-formed, non-caseating granulomas
Sarcoidosis
Caseating Granulomas =
Tuberculosis