Respiratory System Past Paper PDF

THE LUNG CHAPTER 1 EMBRIOLOGY The lungs develop from the ventral wall of the foregut (endoderm) Los pulmones se desarrollan a partir de la pared ventral del intestino anterior (endodermo) ramificacion...

THE LUNG CHAPTER 1 EMBRIOLOGY The lungs develop from the ventral wall of the foregut (endoderm) Los pulmones se desarrollan a partir de la pared ventral del intestino anterior (endodermo) ramificacion surfactante empieza 26 - 28 semanas ANATOMY 2 lobulos broncios 3 lobulos no son symetricos tenia el corazon  HISTOLOGY Clara cells: increase towards terminal bronchiole, have secretory function, main progenitor cell after bronchiolar injury Type I (squamoid) pneumocytes: 95%, flattened Type II (cuboidal) pneumocytes: 5%, produce surfactant (lamellar bodies on EM); during repair, type II pneumocytes are intercambie el able to give rise to type I cells oxygeno 1.Ciliated epithelium alveolo = bulbuja 2. Goblet cell 3. Gland 4. Cartilage 5. Smooth muscle cell 6. Clara cell 7. Capillary 8. Basal membrane 9. Surfactant 10. Type I pneumocyte 11. Alveolar septum 12. Type II pneumocyte siempre una arteria y una veina  CONGENITAL ANOMALIES  Pulmonary hypoplasia: is the defective development of both lungs, resulting in decreased weight , volumen and acini for body weight and gestational age. Caused by anormalities that compress the lung or impede normal lung expansion (diaphragmatic hernia, oligohydramnios). hypoplasia : defecto genetico o algo que ha hecho que el pulmon no se formé CONGENITAL ANOMALIES: CYSTIC FIBROSIS autosomica ; falta una proteina ; transporte de cloro mucovisidosis fibrosis cystica Autosomal recessive disorder ATELECTASIS (COLLAPSE) una parte del pulmon se colapse ❑ Resorption atelectasis stems from complete obstruction of an airway. Over ▪ Atelectasis refers either to incomplete expansion of time, air is resorbed from the dependent the lungs (neonatal atelectasis) or to the collapse of alveoli, which collapse. previously inflated lung, producing areas of relatively ❑ Compression atelectasis results whenever airless pulmonary parenchyma significant volumes of fluid (transudate, exudate or blood), tumor, or air (pneumothorax) accumulate within the pleural cavity ❑ Contraction atelectasis occurs when focal or generalized pulmonary or pleural fibrosis prevents full lung expansion. RESPIRATORY DISTRESS OF NEWBORN deficit de surfactante no surfactante = no puede respirar no esteroides a una madre embarazada al final asthma = corticoides los alveoles colapsados  no puede respirar ; no hay el epithelio plano para respirar Hyaline membranes of newborn EPOC : fumores (mas los varones) el aire no puede salir = deficit de la expiracion CHRONIC OBSTRUCTIVE PULMONARY DISEASE (COPD/COLD): GENERAL ❑ Obstructive airway disease: increase in resistance to airflow due to obstruction at any level; includes emphysema, chronic bronchitis, bronchiectasis, asthma, tumor, foreign body; have reduced maximal airflow rates (FEV1); ❑ Two major forms are chronic bronchitis (chronic large airway disease) and emphysema; they may coexist ❑ Major symptom is dyspnea (shortness of breath) ❑ Usually due to cigarette smoking EPOC bronchitico o emphysemo empiezan con oxygeno CHRONIC BRONCHITIS ▪ Diagnosis: persistent cough with sputum for 3 months in 2 consecutive years without other apparent explanation ▪ Chronic disease of large airways ▪ Causes: 4× - 10× more common in smokers, chronic irritation and infections may contribute ▪ More infections, purulent sputum, hypercapnia, hypoxia than emphysema; clinically called "blue bloaters“ ▪ May cause secondary pulmonary vascular hypertension, cor pulmonale, congestive heart failure, death due to respiratory acidosis and coma ▪ Reid index: ratio of thickness of mucus gland layer to thickness of wall between epithelium and cartilage; normal is 0.4, increased in chronic bronchitis bronchitis : obeso, retiene carbonico no lo saca; no deporte, tos necesita oxygeno para respirar Gross description: Microscopic (histologic) description Boggy mucosa with excessive mucinous secretions, pus, Early: hypersecretion of mucus in large airways with hypertrophy of prominence of bronchial mucosal pits overlying the orifices submucosal glands in tracheobronchial tree of bronchial mucous glands Later: increase in goblet cells in small airways contributes to excessive mucus production and airway obstruction Increased percentage of bronchial wall is occupied by submucosal mucous glands, as measured by Reid index; this directly correlates with sputum production, variable dysplasia and squamous metaplasia Chronic inflammatory infiltrates range from absent to prominent  EPOC EMPHYSEMA  Pulmonary emphysema is defined as permanent abnormal enlargement of air spaces distal to the terminal bronchioles with destruction of the alveolar septa with little or no fibrosis  Destruction of acinar structure and airspace enlargement, especially due to cigarette smoking  Dyspnea; chronic, progressive and usually irreversible (“pink puffer”)  Cachexia  Chest inflation no tiene tratamiento se rompe todo  alveolos que no functionan se ve con radiografia Panacinar Centriacinar Paraseptal Pathogenesis. ▪ Inflammatory mediators and leukocytes ▪ Protease-antiprotease imbalance. ▪ Oxidative stress. ▪ Infection. ASTHMA mas alergicos ▪ Atopic or Extrinsic: Type I hypersensitivity, generally due to allergens; se sieran los broncios y no pueden respirar begins in childhood, triggered by environmental allergens (dander, dust, ❑ Defined by the National Asthma Education and pollen, food), often positive family history; more common in African Prevention Program as a "chronic inflammatory American children; evidence of allergen sensitization; skin test causes disorder of the airways" in which many cells and wheel and flare reaction ▪ Noneosinophilic ("neutrophilic") asthma: a subgroup of atopic asthma not cellular elements play a role - in particular, mast associated with eosinophilia; IL8 recruiting neutrophils are an important cells, eosinophils, T lymphocytes,macrophages, mechanism; patients tend to be less responsive to corticosteroids neutrophils and epithelial cells ▪ Nonatopic or Intrinsic: nonimmune; due to aspirin ingestion, pneumonia, ❑ In susceptible individuals, causes episodes of cold, stress, exercise; follows respiratory infection (rhinovirus, wheezing, breathlessness, chest tightness and parainfluenza virus); usually not familial; no evidence of allergen coughing, particularly at night or early morning sensitization; normal serum IgE, negative skin tests; viral induced ❑ Episodes are usually associated with inflammation may lower threshold of subepithelial vagal receptors to irritants widespread but variable airflow obstruction that ▪ Occupational asthma: due to repeated exposure to fumes, dusts, gases, is often reversible, either spontaneously or with chemicals, often in minute quantities; varying mechanisms of disease treatment depending upon the stimulus ❑ Inflammation also causes an associated ▪ Drug induced asthma: associated with several drugs, but most noteworthy increase in the existing bronchial is aspirin use; rare, aspirin related cases are associated with recurrent hyperresponsiveness to a variety of stimuli rhinitis, nasal polyps and urticaria; patients are sensitive to small doses of aspirin; may be due to direct effects of aspirin on cyclooxygenase pathway ▪ Status asthmaticus: unremitting attacks due to exposure to previously sensitized antigen; may be fatal, usually in patients with a long history of asthma Atopic or Extrinsic asthma. Pathogenesis. demaciado mucus en el bronchio Microscopic description: inflammacion ; reactiones inflamatorias Curschmann spirals (extrusion of mucus plugs), eosinophils, extracellular Charcot-Leyden crystals (crystalloids composed of galectin-10, an eosinophil lysophopholipase), increased mucosal goblet cells and submucosal glands, thickened basement membrane, bronchial smooth muscle hypertrophy, airway wall edema inflamacion BRONCHIECTASIS pathologia no del alveolo pero de los bronchos tratamiento antibioticos herediatio o infecion Definition: ▪ Congenital or hereditary conditions: cystic fibrosis Permanent dilatation of bronchi and bronchioles caused ▪ Infections, including necrotizing pneumonia caused by bacteria, viruses, or by destruction of mucosal and elastic tissues, caused by fungi; this may be a single severe episode or recurrent infections or associated with chronic necrotizing infection of bronchi ▪ Bronchial obstruction, due to tumor, foreign bodies, or mucus impaction; in and bronchioles each instance the bronchiectasis is localized to the obstructed lung segment se ve a la radiografia (ACUTE RESPIRATORY DISTRESS SYNDROME) ARDS (DIFFUSE ALVEOLAR DAMAGE) DAD no puedee respirar Definition: Acute and rapidly progressive hypoxia with bilateral pulmonary edema due to alveolar injury caused by pulmonary or systemic insults Causes of pulmonary and extrapulmonary origin Direct lung injury Indirect lung injury pierdan el conocimiento Common causes una sespsis Common causes Sepsis Infectious pneumonia Severe trauma or burn, especially Aspiration of gastric contents bronchoaspiracion with shock and multiple transfusions Less common causes traumatismos Less common causes Pulmonary contusion Cardiopulmonary bypass Fat emboli Drug Near drowning Acute pancreatitis Inhalational injury (particle, gas) Autoimmune disease Reperfusion pulmonary edema, after Transfusion related acute lung injury lung transplantation or pulmonary embolectomy The normal alveolus (Left-Hand Side) and the injured alveolus in the acute phase of acute lung injury and the acute respiratory distress syndrome (Right-Hand Mechanisms important in the resolution of acute lung injury and the acute respiratory Side) distress syndrome. Microscopic description: Exudative phase Alveolar change Hyaline membranes on alveolar duct or sacs Interstitial and intra-alveolar edema Collapsed alveoli Epithelial change Denudation and necrosis of type I pneumocytes Vascular change Necrosis of endothelial cells Neutrophil aggregation Micro thromboembolism Hyaline membranes Type II pneumocyte hyperplasia Hemorrhage Proliferative / organizing phase Alveolar change Organizing or remnants of hyaline membrane Interstitial and intra-alveolar proliferation of fibroblasts / myofibroblasts Lymphocytic infiltration Epithelial change Proliferation of type II pneumocyte Vascular change Endothelial injury and thromboembolism in arteries / arterioles Fibrosis phase Alveolar change Diffuse collagenous fibrosis Microscopic honeycomb-like change Traction bronchiolectasis Epithelial change alveolos llenos de inflamacionProliferative / organizing phase Squamous cell hyperplasia Vascular change Remodeling of arteries Fibrosis in intima Thickening of media PNEUMOCONIOSIS sustensias extranas ANTHRACOSIS ▪ Presence of carbon particles in lung ▪ Not a pathologic condition necessarily, often due to urban living ▪ Carbon particles are relatively inert and usually don't elicit reactive fibrosis ▪ When extensive, may cause coal workers' pneumoconiosis (below) Anthracotic pigment ordinarily is not fibrogenic, but in massive amounts (as in "black lung disease" in coal miners) a fibrogenic response can be elicited to produce the "coal worker's carbon = negro pneumoconiosis" seen here. no puede respirar un pigmento extrano = fibrosis Anthracotic lymph node ASBESTOS sustencia toxica !!! los cristales !!!!  Crystalline hydrated silicates that form fibers  Causes localized fibrous plaques, pleural effusions, parenchymal interstitial fibrosis (asbestosis), bronchogenic carcinoma, mesothelioma, laryngeal carcinoma and possibly colon carcinoma  Exists in serpentine / chrysotile (curly, flexible) and amphibole (straight, stiff, brittle) forms; most asbestos in industry are serpentine, but amphiboles are more pathogenic; link with mesothelioma is almost always with amphibole form  Chrysotiles usually are caught in upper respiratory passages, removed by mucociliary elevator; they are soluble and leached from tissue if they reach alveoli  Amphiboles (straight, stiff) go deeper into lungs; fibers > 8 mm and thinner than 0.5 mm are more injurious  Asbestos related tumors have no special histologic features  ASBESTOSIS: Begins in lower lobes and subpleurally (in contrast to coal workers' pneumoconiosisP and silicosis), progresses to middle and upper lobes  SILICOSIS enfermedad profesional granuloma = una inflamacion ▪ Most prevalent chronic occupational disease in the world, due to foundry work, sandblasting, stone cutting and coal mining ▪ Decades of exposure usually required for symptoms ▪ Causes a progressive, nodular fibrosing pneumoconiosis ▪ Detect on routine chest xray as a fine nodularity in upper lobes, but normal pulmonary function ▪ No symptoms until progressive massive fibrosis ▪ Disease may progress even after exposure to silica ceases ▪ Not associated with lung cancer By polarized light microscopy can be seen the etiology for most pneumoconioses (even those in coal miners)--silica crystals. Here are seen bright white crystals of varying sizes. The silica induces a fibrogenic response by macrophages to produce the nodular foci of collagen deposition. A silicotic nodule within lung parenchyma is seen here. It is composed mainly of bundles of interlacing pink collagen. There is a minimal inflammatory reaction. The greater the degree of exposure to silica and increasing length of exposure determine the amount of silicotic nodule formation and the degree of restrictive lung disease. HYPERSENSITIVITY PNEUMONITIS ▪ Also known as extrinsic allergic alveolitis ▪ An interstitial pneumonia with acute to chronic respiratory failure caused by inhalation exposure to a variety of natural or chemical antigens ▪ Histologically characterized by airway centered inflammation with fibrosis and poorly formed nonnecrotizing granulomas ▪ Predominant in middle to upper lobes of the lung; usually bilateral. SARCOIDOSIS immunologico sarcomiosis Microscopic description: ▪ Noncaseating epithelioid granulomas with tightly packed epithelioid ❑ Multisystemic disease of unknown origin that involves lung or cells, Langhans giant cells and lymphocytes (T cells), usually in bilateral hilar lymph nodes in 90% of cases interstitium adjacent to bronchioles and around and within vessel ❑ May be a manifestation of disordered immune regulations in walls, pleura and connective tissue septa.May also be hyalinization, genetically susceptible individuals after exposure to diffuse interstitial fibrosis. environmental agents ▪ Schaumann bodies: laminated concretions of calcium and protein ❑ Presents as perihilar node involvement, diffuse pulmonary ▪ Asteroid bodies: stellate inclusions within giant cells, in 60% of disease, pulmonary interstitial fibrosis, localized bronchial granulomas stenosis, distal bronchiectasis and atelectasis ❑ Age usually 20 - 40 years, F > M, 90% are black A diagnosis of exclusion, since there are no specific criteria for disease; should culture and use special stains Asteroid bodies Schaumann bodies USUAL INTERSTITIAL PNEUMONIA (UIP) / IDIOPATHIC PULMONARY FIBROSIS (IPF) chronica ▪ Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic interstitial Microscopic description lung disease of unknown cause, associated with histologic and radiologic Dense fibrosis, often destructs alveolar architecture pattern of usual interstitial pneumonia (UIP) Honeycomb: Cystic spaces lined by bronchiolar ▪ Chronic and progressive respiratory failure due to fibrosis in the lung epithelium and fibrotic wall ▪ No treatment has been found to substantially improve its prognosis Fibroblastic foci no tratamiento fibrosis rotos alveolos causa = desconosida tratamiento = no hay CRYPTOGENIC ORGANIZING PNEUMONIA (BOOP) ▪ Organizing pneumonia pattern is one of the most commonly seen lung lesions in a variety of diseases, such as infections and systemic diseases ▪ Cryptogenic organizing pneumonia is a relatively rare disease ▪ Histologically, both cryptogenic organizing pneumonia and secondary organizing pneumonia are characterized by polypoid fibroblastic aggregations which plug alveolar sacs, ducts and bronchioles (Masson body) ▪ There is no interstitial fibrosis or honeycomb lung. Some patients recover spontaneously, but most need treatment with oral steroids for 6 months or longer for complete recovery. PNEUMONIA  Pneumonia is defined as any infection of lung parenchyma  Pneumonia is due to impairment of normal defense mechanisms or lowered host resistance  Normal defense mechanisms are nasal clearance (sneezing, blowing, swallowing), tracheobronchial clearance (mucociliary action) and alveolar clearance (alveolar macrophages) INFECTIONS  Impairment is due to primary or acquired immunosuppression, suppression of cough reflex (drugs, virus, coma, anesthesia), injury to mucociliary apparatus (smoking, virus, Kartegeners syndrome), injury to PNEUMONIA - macrophages (tobacco, alcohol, anoxia), pulmonary congestion / edema or accumulation of secretions (cystic fibrosis) GENERAL  Often divided into community acquired, hospital acquired and aspiration pneumonia Microscopic description: ▪ Bronchopneumonia: neutrophils in bronchi, bronchioles and adjacent alveolar spaces ▪ Lobar pneumonia: initially congestion with bacteria and few neutrophils; then red hepatization (grossly resembles liver) with massive congestion, neutrophils, fibrin; then gray hepatization with fibrinopurulent exudate and organization el pulmon esta duro ( no normal) ▪ Interstitial pneumonia, characterized by inflammation in the interstitium, the soft tissue between the alveoli. inflamatorio ❑ Lobar pneumonia: affects entire lung but now rare due to antibiotics; associated with increased virulence of organism or increased host vulnerability (infants, elderly); may be due to extension of existing bronchiolitis or bronchitis ❑ Bronchopneumonia: patchy consolidation of lung centered on bronchi; may progress to lobar pneumonia; patterns of bronco- and lobar The cut surface of this lung demonstrates the pneumonia may overlap typical appearance of a bronchopneumonia with areas of tan-yellow consolidation. Remaining lung is dark red because of marked pulmonary congestion  virus bactaria  atipica PNEUMOCYSTIS JIROVECII PNEUMONIA ▪ Formerly known (incorrectly) as P. carinii Microscopic (histologic) description: ▪ Opportunistic yeast-like fungus presente in BAL, sputum or biopsy ▪ Most common pneumonia in AIDS patients, Alveolar spaces filled with pink, foamy amorphous material composed of who are at high risk if CD4 < 200 or if protein- proliferating fungi and cell debris calorie malnutrition Fungi are 4 - 6 microns, cup / boat shaped cysts ▪ May also occur in otherwise immunocompetent patients with protein- calorie malnutrition, hematologic malignancies or undergoing chemotherapy or chronic corticosteroid treatment ▪ Radiology: usually atypical interstitial pneumonia Gomori methenamine silver (GMS) CMV PNEUMONIA / PNEUMONITIS ▪ Lung injury caused by infection by ❑ Gross description CMV, a β herpes virus Lung is firm (similar to interstitial lung disease) with hemorrhagic nodules that may ▪ Usually immunocompromised patients become confluent ▪ In AIDS patients, CMV disease generally occurs when the CD4+ cell count < 50/mm3 ❑ Microscopic (histologic) description ▪ CMV is the most common life Mononuclear infiltrates, usually mild edema and pneumocyte hyperplasia threatening infection in hematopoietic Infected cells are generally large with a prominent basophilic nuclear inclusion, often stem cell transplant recipients and is with a clear halo, and smaller basophilic cytoplasmic inclusions are also present the most common opportunistic viral Hemorrhagic necrosis may be present infection in AIDS In lung, usually infects endothelial and epithelial cells and alveolar histiocytes ▪ In addition to lung, CMV causes severe disease in retina, brain, kidneys, GI ASPERGILLUS Aspergilloma ❑ Aspergillus infection is associated with solid organ or bone marrow transplants and antileukemic chemotherapy; relatively uncommon in AIDS patients ❑ Gross description: Invasive disease usually shows targetoid lesions with peripheral consolidation and central thrombosed vessels due to angioinvasive fungi. ❑ Microscopic (histologic) description: Dichotomous (into two nearly equal branches, or 45 degrees) branching, hyphae with frequent septation, diameter ranges from 2.5 to 4.5 μm PAS stain TUBERCULOSIS un bacilo de coc First discovered in 1882 by Robert Koch Other bacteria are commonly identified with a ▪ Due to Mycobacteria tuberculosis microscope by staining them with Gram stain. ▪ Transmission is from person to person via However, the mycolic acid in the cell wall of M. airborn droplets, infections may be dormant tuberculosis does not absorb the stain. Instead, for years acid-fast stains such as Ziehl-Neelsen stain, or fluorescent stains such as auramine are used ▪ Infection does not mean disease; most M. tuberculosis can be grown in the laboratory. infected individuals are asyptomatic Compared to other commonly studied ▪ TB is often a disease of poverty, bacteria, M. tuberculosis has a remarkably slow overcrowding and associated with other growth rate (Löwenstein–Jensen médium) chronic diseases ▪ Lung involvement is the major cause of morbidity / mortality ▪ Multidrug resistant TB and extensive drug resistant TB have recently emerged as clinical and public health challenges that have come about, at least in part from incomplete compliance with drug treatment regiments ▪ AIDS patients are more susceptible to TB and have more severe disease GHON'S COMPLEX: PRIMARY TB tuberculosis ❑ Initial focus of infection is Ghon's complex, consisting of parenchymal subpleural lesion, near upper / lower lobe interlobar fissure (apex has high oxygen tension) with enlarged caseous lymph nodes ❑ Lesions usually undergo fibrosis, calcification and cause no symptoms: Ranke complex (Rx) ❑ Rarely (infants, children, immunocompromised), get progressive spread with cavitation, TB pneumonia and miliary TB granuloma = bola de inflamacion Langhans giant cells: They are formed by the fusion of epithelioid cells (macrophages), and contain nuclei arranged in a horseshoe-shaped pattern in the cell periphery. Although traditionally their presence was associated with tuberculosis, they are not specific for tuberculosis or even for mycobacterial disease. In fact, they are found in nearly every form of granulomatous disease, regardless of etiology. SECONDARY TB nodulos atipicos ▪ About 90 - 95 % of cases with secondary tuberculosis in adults occur by the reactivation of the latent primary infection, the other cases resulting from reinfection with Mycobacterium tuberculosis. ▪ The initial lesion in secondary tuberculosis is the apical nodule, which presents as a uni- or bilateral, small-sized (max. 3 cm), yellow-grey, low consistency (caseous necrosis) solid nodular mass. After treatment, the apical nodule cures by fibrosis and calcium salts impregnation  cavidades en el pulmon Secondary progressive tuberculosis ▪ Apical cavitary fibrocaseous tuberculosis occurs by the draining of the apical nodule through the bronchial wall resulting thin anfractuos multiple cavities, lined with caseous debris (recent cavitation). ▪ Advanced cavitary fibrocaseous tuberculosis presents large lesions extended to one or more pulmonary lobes. ▪ Tuberculous bronchopneumonia occurs as an acute complication of secondary tuberculosis, presenting as patchy circumscribed condensated foci. ▪ Milliary tuberculosis appears by lymphatic dissemination exclusively in lung, and by blood dissemination resulting in systemic Milliary tuberculosis, affecting the spine, spleen, liver, adrenal. ▪ Caseous pneumonia, acute complication of tuberculosis, presents as a diffuse yellowish area of consolidation accompanied by multiple small sized cavities with irregular walls and caseous debris, that can be identified also in the bronchioles.  cardiac embolismo en el pulmon ; va por la arteria ; coagulo DISEASES OF VASCULAR ORIGIN: PULMONARY EMBOLISM  Pulmonary embolism is an important cause of morbidity and mortality  Blood clots that occlude the large pulmonary arteries are almost always embolic in origin.  Source—thrombi in the deep veins of the leg (>95% of cases)  Pulmonary embolism usually occurs in patients with a predisposing condition that produces an increased tendency to clot (thrombophilia): cardiac disease, cancer, or have been immobilized for several days or weeks prior to the appearance of a symptomatic embolism. Those with hip fractures are at particularly high risk. Hypercoagulable states, either primary or secondary (e.g., obesity, recent Saddle embolus surgery, cancer, oral contraceptive use, pregnancy), are important risk factors. ▪ Pulmonary embolus can be distinguished from a postmortem clot by the presence of the lines of Zahn in the thrombus  Rarely pulmonary embolism may consist of fat, air, embolismo pulmonal or tumor.  The pathophysiologic response and clinical significance of pulmonary embolism depend on the extent to which pulmonary artery blood flow is obstructed, the size of the occluded vessels, the number of emboli, and the cardiovascular health of the patient  The pulmonary infarct is classically hemorrhagic and appears as a raised, red-blue area in the early stages Pulmonary infarction DISEASES OF VASCULAR ORIGIN: PULMONARY HYPERTENSION ▪ Pulmonary hypertension is defined as a mean pulmonary artery pressure greater than or equal to 25 mm Hg at rest. (usual 1/8) ▪ Pathogenesis: - Chronic obstructive or interstitial lung diseases - Antecedent congenital or acquired heart disease (mitral stenosis) - Recurrent thromboemboli - Idiopatic pulmonary hypertension ▪ Morphology Regardless of their etiology, all forms of pulmonary hypertension are associated with medial hypertrophy of the pulmonary muscular and elastic arteries, pulmonary arterial atherosclerosis, and right ventricular hypertrophy DIFFUSE PULMONARY HEMORRHAGE SYNDROMES imunologicos ; necrosis Goodpasture Syndrome Polyangiitis With Granulomatosis: Wegener  Autoimmune disease in which kidney and lung injury are caused by  Previously called Wegener granulomatosis, granulomatosis with circulating autoantibodies against the noncollagenous domain of the α3 polyangiitis is a necrotizing vasculitis characterized by a triad of: chain of collagen IV. en la - Necrotizing granulomas of the upper respiratory tract (ear, nose,  The antibodies initiate inflammatory destruction of the basement tuberculosis membrane in renal glomeruli and pulmonary alveoli, giving rise to rapidly sinuses, throat) or the lower respiratory tract (lung) or both. sarcodeosis wegener progressive glomerulonephritis and a necrotizing hemorrhagic interstitial - Necrotizing or granulomatous vasculitis affecting small- to medium-sized pneumonitis vessels  Histologically, there is focal necrosis of alveolar walls associated with intra-alveolar hemorrhages. - Focal necrotizing, often crescentic, glomerulonephritis.  In many cases immunofluorescence studies reveal linear deposits of immunoglobulins along the basement membranes of the septal walls (IgG) necrosis e inflamacion