Lung Diseases of Vascular Origin PDF

Lung diseases of vascular origin lungs ‫ عملت مشاكل بال‬blood vessels ‫مشاكل بال‬ Khairat Battah, MD Lecturer/ consultant histopathology Faculty of medicine Albalqa Applied University Email: [email protected]....

Lung diseases of vascular origin lungs ‫ عملت مشاكل بال‬blood vessels ‫مشاكل بال‬ Khairat Battah, MD Lecturer/ consultant histopathology Faculty of medicine Albalqa Applied University Email: [email protected] Learning objectives 1. Know definition, pathogenesis, causes and morphologic features of pulmonary hypertention 2. Know pathogenesis and morphologic features of diffuse pulmonary hemorrhage syndromes 3. Know morphologic features of pulmonary embolism and pulmonary infarction -.25/8 ‫ هو‬pulmonary circulation ‫ لل‬blood pressure ‫احنا بنعرف انه ال‬ systole deposit of uremich immunoglobulin along basement membrane ) plasmapheresis 2) Immunodepresent > Lung features, hemoptysis , kidney features: Hematourea (Blood in urine) -> glomerulonephritis - renal failure (MCates of 5.5. 55.15.: 1 8.5Unen Sis uricacids i.. une inMc cause ofdeath - renal Failure Goodpasture syndrome Uncommon autoimmune disease in which lung and kidney injury are caused by circulating autoantibodies against certain domains of type IV collagen that are intrinsic to the basement membranes of renal glomeruli and pulmonary alveoli. The antibodies trigger destruction and inflammation of the basement membranes in pulmonary alveoli and renal glomeruli Necrotizing hemorrhagic Rapidly progressive interstitial pneumonitis glomerulonephritis. &. v Most cases occur in the teens or 20s. vIn contrast to many other autoimmune diseases, there is a male preponderance. W The majority of patients are active smokers. ~ When only renal disease is caused by this antibody, it is called anti– glomerular basement membrane disease. Morphology Grossly: lungs are heavy, with areas of red-brown consolidation. Histologically: Focal necrosis of alveolar walls with intra-alveolar hemorrhages. Hemosiderin-laden macrophages in the alveoli. Immunofluorescence studies → linear deposits of immunoglobulins along the basement membranes of the septal walls. The kidneys have the characteristic findings of focal proliferative glomerulonephritis in early cases or crescentic glomerulonephritis -> infarction 1182;58 cresent shake big in patients with rapidly progressive glomerulonephritis. Clinical features Respiratory symptoms, principally hemoptysis Manifestations of glomerulonephritis leading to rapidly progressive renal failure. The most common cause of death is uremia. Intensive plasmapheresis improves the outcome by removing anti– basement membrane antibodies. Simultaneous immunosuppressive therapy inhibits further antibody production, ameliorating both lung hemorrhage and glomerulonephritis.  rare very diffuse alveolar hemorrhage. young children, but reported in adult insidious onset of productive cough, hemoptysis, and anemia associated with diffuse pulmonary infiltrates. ↳ Unknown immunological therapy (5.81 94 response to long-term immunosuppression indicates that an immunologic mechanism - - 1861 is may be involved in the pulmonary capillary damage underlying alveolar bleeding. Idiopathic Pulmonary Hemosiderosis Rare disorder. a. Characterized by intermittent,lungs diffuse alveolar hemorrhage. Most cases occur in young children, although the disease has been reported in adults as well. Clinical triad of insidious onset of productive cough, hemoptysis, and anemia associated with diffuse pulmonary infiltrates. The cause and pathogenesis are unknown, and anti–basement membrane antibodies (the cause of Goodpasture syndrome) are undetectable. Favorable response to long-term immunosuppression indicates that an immunologic mechanism may be involved in the pulmonary capillary damage underlying alveolar bleeding.  Wegener granulomatosis or granulomatosis with polyangitis angiitis granulomatous + in parenchymal v PR3- ANCA chronic sinusitis if it involve upper respiratory tract: epistaxis, nasal perforation, upper respiratory tract: cough, hemoptysis, pain chest if it lower Vasculitis-associated hemorrhage Granulomatosis and polyangiitis ( formerly called Wegener granulomatosis) The lung lesions are characterized by a combination of necrotizing vasculitis (“angiitis”) and parenchymal necrotizing granulomatous inflammation. The signs and symptoms stem from involvement of the upper- respiratory tract (chronic sinusitis, epistaxis, nasal perforation) and the lungs (cough, hemoptysis, chest pain). Anti-neutrophil cytoplasmic antibodies (PR3-ANCAs) are present in 95% of cases.  embolus 11, From deep veins embolus 918 thromboembolisms - is? Lungs oflower limb deep veins oflower limb 1% in the general population of hospital patients to 30% in patients dying after severe burns, trauma, or fractures. content1).-- 1) immobilization 2) surgeries 3) contraceptive pilswith high estrogen 1) Hypercoagulability 2) vascular damage 3) Blood stasis depends on: 1) size of the occluded vessels 2) number of emboli 3) cardiovascular health of the patient. (pulmonary Hypertension) 4) extent to which pulmonary artery blood flow is obstructed Slungtissues 55- > - 4(cause:emboli 1) Bronchial arteries Ischemic necrosis (infarction) is the Blood from exception rather than the rule, occurring in deoxygenated 2) a small percentage of patients with pulmonary A. thromboemboli. 3) Alveoli NextSlide ⑧ sis? smaller Saddle embolus:3 5 BV. emboli -$5. - BV /155;-5-,embolus sis " a S. PH s'S. S Sudden. suddent in p. pressure BV 58 sudden in workloads very - side 12.151 S. - very right - death "815:5 side heart ; 15 & 85. Cluts1 + on right cor-pulmonales's TXAz,serotonin -ssBV & 99s. sudden deaths rightside H7/ &Ss. Hypoxia. flow I1 BU (1305850 D arteries)? SI 2 Blood ~ alveolis, 88: 5-d 5555588502385d - I :10291 5, & 9,59: -. Mismatch v/Q ⑧ - -> P collapse d5.=S5, 455. 8:11 2 alredlar (1 ds. 5) (Es ] Surfactant Pulmonary thromboembolism A clot that adheres to a vessel wall is called a thrombus, whereas an intravascular clot that floats in the blood is termed an embolus. Pulmonary thromboembolism Pulmonary embolism is an important cause of morbidity and mortality, particularly in patients who are bedridden Occurs in a wide range of conditions that are associated with hypercoagulability. Blood clots that occlude the large pulmonary arteries are almost always embolic in origin. The usual source is thrombi in the deep veins of the leg (>95% of cases) Its incidence at autopsy has varied from 1% in the general population of hospital patients to 30% in patients dying after severe burns, trauma, or fractures. Risk factors for PE (1) prolonged bed rest (particularly with immobilization of the legs). (2) surgery, especially orthopedic surgery on the knee or hip (3) severe trauma (including burns or multiple fractures) (4) congestive heart failure (5) in women, the period around parturition or the use of oral contraception pills with high estrogen content (6) disseminated cancer (7) primary disorders of hypercoagulability (e.g., factor V Leiden) Rarely, pulmonary embolism may consist of fat, air, or tumor. Virchow's triad 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 The cardiovascular health of the patient. Consequences of pulmonary arterial occlusion: 1. Increase in pulmonary artery pressure from blockage of flow and, possibly, vasospasm caused by neurogenic mechanisms and/or release of mediators (e.g., thromboxane A2, serotonin) Occlusion of a major vessel results in an abrupt increase in pulmonary artery pressure, diminished cardiac output, right-sided heart failure (acute cor pulmonale), and sometimes sudden death. If smaller vessels are occluded, the result is less catastrophic and may even be clinically silent. 2. Ischemia of the downstream pulmonary parenchyma. Ischemic necrosis (infarction) is the exception rather than the rule, occurring in a small percentage of patients with thromboemboli. 3. Hypoxemia develops as a result of multiple mechanisms: Ventilation perfusion mismatch which occurs due to redistribution of blood from occluded pulmonary arteries to the nonoccluded vessels Patient II Perfusion of atelectatic lung zones. Alveolar collapse occurs in ischemic I 0. areas because of a reduction in surfactant production and because pain·51 emboli 8s. associated with embolism leads to reduced movement of the chest wall. I5/5 0. The decrease in cardiac output causes a widening of the difference in alveoli (15.2, 8885 pres. (1 s s arterial-venous oxygen saturation. alveolar collapses. I Right-to-left shunting of blood may occur through a patent foramen ovale, w present in 30% of normal individuals 9; i left). 02 S1's right I s. 3 is left is 02. patient / oxygenation 19 -  Morphology v Saddle embolus →A large embolus may embed in the main pulmonary artery or its major branches or lodge at the bifurcation. Fatal, suddenly from hypoxia or acute right side heart failure. ~ Smaller emboli become impacted in medium-sized and small-sized pulmonary arteries. With adequate circulation and bronchial arterial flow, the vitality of the lung parenchyma is maintained, but alveolar hemorrhage may occur as a result of ischemic damage to the endothelial cells.  of embolism) Pulmonary infarction (consequence pulmonary Pulmonary infarction results from occlusion of the distal pulmonary arteries leading to ischemia, hemorrhage and ultimately necrosis of the lung parenchyma. The lung parenchyma receives its oxygen supply from a total of three sources: V deoxygenated blood from pulmonary arteries oxygenated blood from the bronchial circulation v direct oxygen diffusion from alveoli. ~ - A sufficient impedance from one of these sources can cause infarction and subsequent tissue necrosis. v Inflammatory mediators from ischemic parenchyma can further limit I. &iMediators gas exchange because of the resultant vasoconstriction and & =1 255 I s bronchoconstriction. bronchi 5is bronchi/192,5,9115-8) 855, 819 I 1 - ( vessels v The relatively higher blood pressure in the bronchial circulation causes an increase in the capillary blood flow, leading to extravasation Blood supply is 55-;* of erythrocytes (i.e. alveolar hemorrhage). dual & s dspischemia. s. i elss ~ If this hemorrhage cannot be resorbed, it results in tissue necrosis 38. - and infarction. alveolar hemorrhage $se,) tissues)! & 5 bronchial S. Blood 8. A.  The more peripheral the embolic occlusion, the higher the risk for infarction. Peripheral 51 emboli 118 $X more infarction -$ $  Morphology v Infarct are wedge-shaped, with their base at the pleural surface and the apex pointing toward the hilus of the lung. v The occluded vessel is usually located near the apex of the infarcted area. /Pulmonary infarcts typically are hemorrhagic. vThe adjacent pleural surface often is covered by a fibrinous exudate. wIn time, fibrous replacement begins at the margins and eventually converts the infarct into a scar. 8.I518 Fibrosis. 15: I al gas exchange. Clinical features The clinical consequences of pulmonary thromboembolism are summarized as follows: v Clinically silent (60% to 80%) → small emboli; the bronchial circulation sustains the viability of the affected lung parenchyma, and the embolic mass is rapidly removed by fibrinolytic activity. ~ Acute right-sided heart failure, or cardiovascular collapse (shock) and sudden death (5%) → more than 60% of the total pulmonary vasculature is obstructed by a large embolus or multiple simultaneous small emboli. / Pulmonary infarction (10% -15%) → Obstruction of small to medium pulmonary branches if some element of circulatory insufficiency also is present. ~ Pulmonary hypertension (