Pathology 2 - Block 2 - Part 4

davidwurbel7's version from 2016-06-27 16:21

Obstructive Lung Disease 2

Question Answer
Permanent and irreversible dilatation of medium sized airways (bronchi and bronchioles due to destruction of cartilage, muscle and supporting elastic tissue in the walls of airways because of inflammatory reaction resulting from chronic persistent necrotizing infectionsBronchiectasis
The most common cause of Bronchiectasis in the western world is thisCystic Fibrosis
The most common cause of Bronchiectasis in the rest of the world is thisTB
Bronchiectasis on chest x-ray appear as thisRing Shadows
Ring shadows on chest x-ray of Bronchiectasis are due to thisFibrosis
Gross findings show dilated bronchi and bronchioles filled with pus. Dilated airways extend to the lung periphery. Dilations are tube like or saccularBronchiectasis
Clinical findings include fever, night sweats and copious foul smelling sputum, Hemoptysis, Digital clubbing, Hypoxemia, hypercapnia, pul HT, and cor pulmonaleBronchiectasis
A chronic inflammatory disorder of the airways characterized by hyper-reactivity of the respiratory tree to various stimuli leading to reversible airway obstruction causing airflow obstructionAsthma
Obstruction produced by combination of constriction of bronchial musculature (bronchospasm), mucosal inflammation and excessive secretion of mucusAsthma
Clinically Manifested by recurrent episodes of Wheezing, Breathlessness, Chest tightness and Cough. Patients also experience difficulty in getting the air out of the lung. These occur particularly at night and/or in the early morningAsthma
Initiated by IgE mediated (type I) hypersensitivity reaction. Triggered by exposure to an allergen. Typically begins in childhood. A positive family history of asthmaAllergic (Extrinsic) Asthma
Associated with Increased serum IgE levels. Increase in blood Eosinophil Count . Positive skin test with offending antigenAllergic (Extrinsic) Asthma
Also known as non allergic asthma. Family history of asthma/allergies – less common. Skin test results- usually negative. Typically develops later in life. Initiated by diverse non-immune mechanisms. Normal serum levels of IgE and eosinophils. Triggers include - Viral respiratory infections ( rhino, parainfluenza and RSV), Inhaled air pollutants (ozone*, SO2, smoke), Stress ,exercise , Smoke & ColdIntrinsic Asthma
Some pharmacologic agents provoke asthma. Patients present with recurrent rhinitis and nasal polyps, urticaria, and bronchospasmDrug-Induced Asthma
Drug-Induced Asthma is most commonly triggered by this drugAspirin
Drug-Induced Asthma is also called thisAspirin-Exacerbated Respiratory Disease (AERD)
Aspirin induced asthma is a pseudo-allergic reaction resulting from thisProstaglandin/Leukotriene Misbalance
Any nocturnal, newly diagnosed asthma in a middle-aged patient should raise a suspicion for thisGastroesophageal Reflux Disease (GERD)
Any nocturnal asthma should be administered this drug as both therapeutic and diagnosticOmeprazole
This is triggered by environmental allergens by dust, pollen and animal danderAllergic (Extrinsic) Asthma
In the initial sensitization to an inhaled allergen, IL-4 produces this effectIgE Production
In the initial sensitization to an inhaled allergen, IL-5 produces this effectEosinophil Activation
In the initial sensitization to an inhaled allergen, IL-13 produces this effectMucus Production
Cross-linking of this on mast cells causes the release of histamine and other mediatorsIgE Antibodies
This type of cells is the most important cell in the late phase of asthma which cause the continuation of inflammation and epitheium damage in asthmaEosinophil
These chemicals mediators are the most important for late phase asthma by prolonged bronchoconstriction, increased vascular permeability and increased mucin secretionLeukotrines C4, D4 and E4
Formation of spiral shaped mucus plugs contain shed epithelial cells called thisCurschmann Spirals
Crystalline granules in eosinophils fuse to formCharcot-Leyden Crystals
This is characterized by severe dyspnea with wheezing. Difficulty in expiration – prolonged expiration. Patient labors to get air in, then cannot get it out. Diminished breath sounds, hyper- resonance and increased AP diameterAsthmatic Attack
Severe attack which persists for days and even weeks and does not respond to therapyStatus Asthmatics

Restrictive Lung Disease

Question Answer
This is associated with reduced lung compliance ( decreased filling of lungs on inspiration) and an increased elasticity (increased recoil of lungs on expiration)Restrictive Lung Disease
The Pulmonary Function Test reveals the following decreased TLC and RV, decreased FEV 1sec and decreased FVCRestrictive Lung Disease
Clinical finding include dyspnea, dry cough, and hypoxemiaRestrictive Lung Disease
Pathologic finding in restrictive lung diseaseAlveolar Wall Fibrosis
An x-ray of patient with restrictive lung disease would show thisGround Glass Shadows
Ground Glass Shadows are usually found in this part of the lung x-rayLung Base
Restrictive Lung disease as a result of inhalation of inorganic mineral dustPneumoconiosis
Restrictive Lung disease as a result of inhalation of known organic organismsHypersensitivity Pneumonitis
Restrictive Lung diseases as a result of autoantibodies attacking the basement membrane of the lungsGoodpasture Syndrome
Restrictive Lung diseases as a result of activated macrophages forming granulomatic lesion in the lungsWegner Granulomatosis
Restrictive Lung diseases as a result of a parasitic infection of the lungSarcoidosis
Restrictive Lung disease as a result of unknown originIdiopathic Pulmonary Fibrosis
Refers to a group of occupational lung diseasesPneumoconiosis
Pneumoconiosis due to the prolonged inhalation of coal dustCoal Worker's Pneumoconiosis (CWP)
Usually asymptomatic. Carbon pigment (anthracotic pigment) accumulates in macrophages along lymphatics and in interstitial lung tissuePulmonary Anthracosis
Characterized by presence of nodular masses in lung composed of collagen fibers and anthracotic pigment. X ray: opacities <1 cm. Little pulmonary dysfunctionSimple Coal Worker's Pneumoconiosis
Characterized by fibrotic opacities >1 cm on X ray. On microscopically - Opacities show dense collagen and anthracotic pigment. Complications include crippling lung disease (black lung disease), Hypoxemia, pulmonary HTN, cor pulmonaleComplicated Coal Worker's Pneumoconiosis
This Pneumoconiosis does not include an increased risk of developing TB and primary lung cancerComplicated Coal Worker's Pneumoconiosis
This condition is associated with Pneumoconiosis in which there is Pneumoconiosis plus Rheumatoid nodule in the lungsCaplan's Syndrome
Most prevalent chronic occupational disease in the worldSilicosis
Due to inhalation of crystalline silicone dioxide resulting in inflammation and fibrosis of the lung tissueSilicosis
Chronic exposure leads to nodular opacities in lungs of concentric layers of collagen. Birefringent can be seen with polarized lightSilicosis
Complications of this Pneumoconiosis include increased risk of developing TB and primary lung cancerSilicosis
Condition found in persons in the occupations - Pipe fitting in shipyards, roofing, insulation and construction industries, demolition of building containing asbestos, auto mechanics (break lining)Asbestos
Curley and flexible asbestos fibers. Tend to be in upper airway. Cause interstitial fibrosis and lung cancer.Serpentine (Chrysotile) Asbestos
Lung cancer associated with serpentine asbestosBronchogenic Carcinoma
Straight, stiff, needle like. Delivered deep. Cause lung cancer and mesotheliomasAmphibole (Crocidolite) Asbestos
Cancer associated with amphibole asbestosMesothelioma
Asbestos fibers coated by iron and protein. Macrophages phagocytose and coat fibers with ferritinFerruginous Bodies
Most common lesion associated with exposure to asbestos. Calcified plaques on pleura and domes of diaphragm. Not a precursor for mesotheliomaBenign Pleural Plaques
Most common cancer associated with exposure to asbestos. Risk further increases if person smokes. Occurs ~20 years after first exposurePrimary Bronchogenic Carcinoma
2nd most common cancer. No etiologic relationship with smoking. Arises from cells lining the pleura. Occurs ~25-40 years after 1st exposureMalignant Mesothelioma
Exposure in nuclear and aerospace industry. Lesion - Diffuse interstitial fibrosis with non-caseating granulomas. Increased incidence of cor pulmonale and primary lung cancerBerylliosis

Restrictive Lung Diseases 2

Question Answer
Immunologically mediated disorder caused by repetitive exposure to a known inhaled (organic) antigen. Involves type III and IV hypersensitivity reactions to bacterial and fungal infected vegetable matter, or moldy “droppings. Does not involve IgE ab and therefore no eosinophiliaHypersensitivity Pneumonitis
Hypersensitivity Pneumonitis does not show this when doing blood workEosinophilia
Hypersensitivity pneumonitis is also know asExtrinsic Allergic Alveolitis (EAA)
Patient presents with fever, cough, dyspnea ( 4-6 hours after exposure). Resolution after cessation of exposure. Type III HSRAcute Hypersensitivity Pneumonitis
Patients presents with cough, dyspnea, weight loss, granuloma formation, fibrosis, restrictive lung disease and cor pulmonale. Type IV HSRChronic Hypersensitivity Pneumonitis
Chronic Hypersensitivity Pneumonitis on microscopy will show this in the interstitiumNon-Caseating Granulomas
Condition associated with exposure to moldy hay containing thermophilic actinomycetesFarmer’s Lung
Condition associated inhalation of wheat weevil protein (in fumes originating from fermenting corn)Silo Filler’s Disease
Occurs in workers in textile factoriesByssinosis
Associated with exposure to cotton, hemp or linen (“Monday morning blues”)Byssinosis
Lung disease that develops after repeated exposure to moldy sugar caneBagassosis
Proteins from bird feathers or excretaPigeon Breeders Lung
Exposure to thermophilic bacteriaHumidifier or Air Conditioner Lung
Multisystem granulomatous diseases of unknown etiology characterized by non-caseating granulomas in many tissues and organs. Second most common ILD. More common in African American women. Occurs in adults of 20-40 yrs age. Most commonly targets the lungs, eyes (blurry vision), salivary glands (dry mouth) and skin. Blood work showed hypercalcemia and increase in angiotensin converting enzymeSarcoidosis
Noncaseating granulomas in the lung interstitium and hilar lymphnodes (potato nodes - bilateral lymphadenopathy). Granulomas contain multinucleated giant cells with laminated calcium concretions and stellate inclusionsSarcoidosis
Granulomas contain multinucleated giant cells with laminated calcium concretionsSchaumann Bodies
Granulomas contain multinucleated giant cells with stellate inclusionsAsteroid bodies
Clinical and pathological findings include Skin lesions - Erythema nodosum - raised, red, tender nodules (anterior aspect of legs) with no granulomas or Nodular, painless lesions containing granulomas. Eye and lacrimal gland lesions iritis, iridocyclitis and uveitis leading to blurry vision to total loss of vision. Dry eyes. Parotid glands: unilateral or bilateral parotitis with painful enlargement leading to dry mouth = xerostomia. Liver: Granulomatous hepatitis +/- hepatomegaly. Diabetes insipidus - hypothalamic and or posterior pituitary disease. Other organs - granulomas in spleen, CNS , heart and bone marrowSarcoidosis
Clinical findings include Asymptomatic to Shortness of breath, dry cough ,fever, fatigue, weight loss and night sweats. Laboratory findings - Hypercalcemia - due to synthesis of 1 alpha hydroxylase by macrophages in granulomas (increases vit D). Elevated serum angiotensin converting enzyme (ACE). Chest X ray - Enlarged hilar lymphnodes – potato nodes. Prognosis: Majority of patients recover with corticosteroid therapySarcoidosis
The hypercalcemia seen in sarcoidosis is due to over production of this by macrophages1 Alpha Hydroxylase
The elevated serum angiotensin converting enzyme seen in sarcoidosis is due to over production of this by macrophagesAngiotensin Converting Enzyme
Sarcoidosis is treated with thisCorticosteroids
Immune-mediated diseases that manifest as the triad of hemoptysis, anemia, and diffuse pulmonary infiltratesDiffuse Alveolar Hemorrhage Syndromes
Male predominant disease characterized by Glumerulonephritis and Hemorrhagic interstitial pneumonitis. Lesions caused by IgG anti-basement membrane antibodies directed against BM of pulmonary and glomerular capillariesGoodpasture Syndrome
In Goodpasture Syndrome, the antibodies are directed against thisNon-Collagenous Domain of the α3 Chain of Collagen IV in the Basement Membrane
Typically patients first present with hemoptysis, cough, dyspnea and then acute rapidly progressive glomerulonephritis leading to acute renal failureGoodpasture Syndrome
On Immunofluorescence a linear pattern of immunoglobulin (IgG) deposition is seenGoodpasture Syndrome
A disorder of unknown cause. Pathogenesis is repeated cycles of alveolitis triggered by an unknown antigen. Release of cytokines produces interstitial fibrosis. Alveolar fibrosis leads to dilatation of airways. Lung has a honeycomb appearanceIdiopathic Pulmonary Fibrosis (IPF)
Lines that appear on x-ray of patients with restrictive lung disease. These lines are more prominent at the lung baseReticular Pattern
Nitrofurantoin, amiodarone, bleomycin, busulfan and cyclophosphamide can cause thisDrug-Inducted Idiopathic Lung Disease
The most common cause of pulmonary complications in patients with systemic sclerosis is thisInterstitial Fibrosis