RESPIRATORY SYSTEM - Main Pathologies
Transcription
RESPIRATORY SYSTEM - Main Pathologies
APARELHO RESPIRATÓRIO RESPIRATORY SYSTEM INFO RESPIRATORY SYSTEM - Main Pathologies DESCRIPTION SYMPTOMS DIAGNOSIS TREATMENT Acute Laryngitis(1,2) Acute inflammation of the larynx. Catarrhal laryngitis is more frequently observed in adults, being, in general, of viral origin and associated to the infection of the upper airways. The excessive use of the voice, allergic reactions and inhaling irritating substances (tobacco) may cause acute or permanent (chronic) laryngitis. Acute epiglottitis is due to a larynx inflammation above the glottis, usually in children; it is caused by type B Haemophylus influenzae. Subglottis laryngitis is the larynx viral inflammation under the glottis. The commonest symptoms are hoarseness of voice (dysphonia), dry cough and pain in the throat. Children with acute epiglottitis present with fever and general malaise. Child may prefer to remain seated as the position makes breathing (airflow) easier. Odynophagia (pain during swallowing) may also be seen in some patients. Subglottic laryngitis affects younger children, and presents with dry cough and a characteristic inspiratory sound (stridor), produced due to airway narrowing. Diagnosis is based on clinical examination. Sputum culture may help in identification of the causative agent. Sometimes, an indirect laryngoscopy is necessary, although it is normally contraindicated in acute epiglottitis. The conventional treatment consists of avoiding the use of irritating substances, resting the voice and administration of anti-inflammatory agents. In acute epiglottitis and subglottic laryngitis, hospital admission and intravenous antibiotics are used; in severe cases, orotracheal intubation or tracheostomy may be required. Acute Respiratory Distress Syndrome(3) Rapidly evolving acute respiratory insufficiency, associated with the appearance of bilateral infiltrates, in the chest x-ray and with PaO2/FiO2≤200 that cannot be explained by left heart dysfunction. The most common cause is sepsis, but it may also be observed in thoracic trauma, bronchial aspiration, toxin inhalation, opiate consumption, etc. It is caused by an increase in the permeability of the alveolo-capillary membrane, consequent to dysfunction of type II pneumocytes, decrease in the surfactant surface and alveolar collapse. Chronic inflammation may lead to onset of fibrosis. The most frequent symptoms are tachypnea (increase of respiratory frequency) and dyspnea associated with symptoms related to the underlying cause. It is defined by the clinical picture, the thorax x-ray and the gas analysis (oxygen and carbon dioxide) in the peripheral blood, which shows mainly an important decrease of the partial pressure of oxygen in the blood (hypoxemia). Treatment of the triggering cause. Specific treatment consists of administering oxygen; the mainstay of supportive management of ARDS is invasive positive pressure ventilation. Allergic Bronchopulmonary Aspergillosis(4,5) Pulmonary disorder caused by an allergic reaction to Aspergillus fumigatus (heat resistant fungus that resides in moist areas or decomposing materials, and also in houses). The pathogenesis is related to genetic predisposition; inhaled conidia A. fumigatus persist and germinate into hyphae, release antigens that compromise the mucociliary clearance, stimulate and breach the airway epithelial barrier, and lead to inflammation. Destructive lesions, bronchiectasis, fibrosis and lung retraction are observed. The majority of patients present with low-grade fever, wheezing and bronchial hyper- reactivity (mimics asthma), hemoptysis, or productive cough. Expectoration of brownish black mucus plugs is characteristic. On the thorax x-ray, there are transitory and recurring infiltrates that look like pneumonias or atelectasis. It is based on clinical and laboratorial criteria (Rosenberg-Patterson criteria): asthma, increase in the number of eosinophils and IgE levels in the blood, presence of specific (IgE and IgG) anti-Aspergillus antibodies, skin hypersensitivity tests and history of pulmonary transitory infiltrates in the thorax x-ray. The key treatment is corticoids. Bronchodilators are only useful for controlling the symptoms. Asbestosis(6,7) Pulmonary condition secondary to inhalation of asbestos. Asbestos is used mainly in manufacturing breaks and as insulation in plumbing and boilers. It is a fibrotic process limited to the walls of alveoli immediately around the bronchioles in the early stages; as it progresses, it covers adjacent areas and becomes diffuse. It arises after prolonged exposure to asbestos. The most common symptoms are an insidious onset of dyspnea, cough and production of sputum. Patients have a greater risk of lung and pleural cancer. It is based on the clinical findings and the history of exposure. Examination shows findings of interstitial fibrosis (end-inspiratory crackles). Chest x-ray shows reticulolinear opacities, mainly in the lower zones. Pleural fibrosis may be seen on CT. Pulmonary function tests show a predominantly restrictive pattern. Occasionally, fiber optic bronchoscopy and bronchoalveolar lavage may demonstrate asbestos fibers. There is no known effective treatment. The patients should be followed periodically due to the greater risk of lung and pleural cancer. Asbestosis Lung Fiber Scar Bronchi Pleural lining Alveoli RESPIRATORY SYSTEM - Main Pathologies DESCRIPTION SYMPTOMS DIAGNOSIS TREATMENT Asthma(8,9) Chronic inflammatory disease of the airways, characterised by reversible limitation of the air flow and recurring episodes of bronchial spasm. An inflammatory mechanism, with predominant involvement of mast cells and immunoglobulin E, together with an alteration of the neural control of the airways (sympathetic and parasympathetic play an important role in the pathogenesis). There is an exaggerated bronchial response to multiple stimuli (bronchial hyper-reactivity), that precedes and predicts the development of asthma. Etiological and triggering factors include genetic predisposition, allergens (acarus, pets’ epithelium – cats and dogs, pollens and fungi), physical exercise, irritating gases, viral infections and drugs like acetylsalicylic acid and other non-steroid anti-inflammatory agents. Typical symptoms are: dyspnea (shortness of breath), wheezing (with a characteristic sound, like a whistle, made by the air passing through the narrowed airways) and cough. A temporal relationship between the appearance of the symptoms and the inciting factor is usually apparent. Wheeze is a distinctive, if non-specific, finding in the physical exam. Diagnosis is confirmed by spirometry; reversibility of obstruction of the air flow, after administration of a bronchodilator, is characteristic. The mainstay of treatment is administration of bronchodilators (beta blockers) and anti-inflammatory agents (corticosteroids, leukotriene inhibitors, mast cell stabilizers). Acute episodes are managed with inhaled bronchodilators and injectable/ inhaled steroids. Severe episodes require hospitalization. Other agents that are used in the treatment of chronic asthma include: disodium cromoglycate, nedocromil and theophyllines. Symptoms Normal bronchiole Hyper-responsive lower airway Hyperinflated lung Asthmatic bronchiole Cough Hissing Chest tightness Atelectasis(10,11) Collapse of a peripheral, segment or lobar region of the lung or of the entire lung, caused by an airway obstruction. It is not a pathology in itself, but is the consequence of a pulmonary or bronchial disorder, such as intraluminal bronchial obstruction (foreign body, mucus plugs, mass lesions) or extraluminal obstruction (pneumothorax, pleural effusion). After a bronchial obstruction, blood absorbs the peripheral alveolar gas and the consequent lung retraction causes the alveolar air content to disappear after a few hours. Atelectasis results in development of a left to right shunt, with attendant hypoxemia. Symptoms depend on the etiology, speed of development and the extent of the atelectasis. An acute bronchial occlusion with massive collapse causes pain in the affected side, dyspnea and tachycardia. There may be a concomitant pulmonary infection, usually with fever. Atelectasis that develops slowly may be completely asymptomatic or else causing only mild symptoms. The diagnosis is suspected on the basis of signs and the clinical setting. Radiology confirms the diagnosis: Chest x-ray shows homogenous parenchymal density (segment, lobar or pulmonary), decrease of the volume of the affected hemithorax and displacement of neighboring structures (heart, mediastinum and diaphragm, etc.) to the affected side. It is important to look for the cause of atelectasis. This may require thoracic axial computerized tomography (CT) and fiber optic bronchoscopy/biopsy. The treatment depends on the etiology of the atelectasis. Treatment is based on repositioning and suctioning of respiratory secretions, percussion therapy, incentive spirometry, or intermittent positive pressure ventilation. In some cases, bronchoscopic suctioning and fiberoptic bronchoscopy for extraction of foreign body or mucus plug may be necessary. Bronchiectasis(12,13) It is a chronic pulmonary condition characterized by persistent cough, excessive sputum production and recurrent chest infections. There is an abnormal and irreversible bronchial dilatation in one or more lobes, secondary to the destruction of the elastic and muscular components of the bronchial wall. It is associated with impairment of mucociliary clearance, leading to bacterial colonization and infection. Most causes are post-infective (pneumonia, tuberculosis); other etiologies include immune defects, connective tissue disorders, allergic bronchopulmonary aspergillosis, cystic fibrosis, obstruction or foreign body aspiration or inhalation and asthma. The most frequent complications are massive hemoptysis, recurrent pneumonia (affecting the parenchyma around the affected bronchus), empyema and lung abscess, respiratory failure and cor pulmonale. Chest x-ray may give characteristic images, however, it is non-specific. Diagnosis is confirmed by high resolution axial computerized tomography (HRCT) of the chest. Treatment is targeted at elimination of the bronchial obstruction, improving clearance of secretions (physiotherapy and postural draining) and treating the respiratory infections. According to the etiology and severity, bronchodilators and oxygen therapy may be necessary. Steroids are not recommended unless there is concomitant asthma or COPD. All patients must receive annual influenza and 5-yearly pneumococcal vaccination. Upper lobe: Cystic Fibrosis Tuberculosis Central: Cystic Fibrosis ABPA Congenital Tracheobronchomegaly Bronchiectasis Normal bronchi Lower lobe: Childhood Infection Aspirations Immunodeficiency Outer wall Mucus Cilia Destruction of wall Increased mucus RESPIRATORY SYSTEM - Main Pathologies DESCRIPTION SYMPTOMS DIAGNOSIS TREATMENT Bronchiolitis(14,15) A fibrotic disease of the respiratory bronchioles. In constrictive bronchiolitis, the fibrotic lesion develops external (peribronchiolar) to the airway lumen, leading to constriction of the airway in a concentric manner and eventual obliteration of the lumen. In proliferative bronchiolitis, lesion develops internally and fills the lumen with an inflammatory polypoid lesion or buds of granulation tissue. Multiple etiological factors are known, such as: exposure to tobacco or toxic gases, viral infections (more frequent in children under the age of 3 years old), post-pulmonary or cardiopulmonary transplant. Many cases are idiopathic. Symptoms are non-specific and include cough and shortness of breath (dyspnea). The chest x-ray may show hyperinflation; HRCT shows a mosaic pattern and may show fibrosis in late stages. Biopsy is required for classification and definitive diagnosis. Treatment is symptomatic; corticosteroids, bronchodilators and oxygen are utilized. Occasionally other immune-suppressants may be required (cyclosporine). Chronic Obstructive Pulmonary Disease (COPD) (16,17) It is a disease characterized by irreversible or partially reversible airflow obstruction. Chronic bronchitis is characterized by the presence of cough and sputum for more than 3 months in an year, during two or more consecutive years. Pulmonary emphysema is characterized by abnormal and permanent increase of the air spaces besides the terminal bronchiole, secondary to destruction of alveolar walls. The restriction of the expiratory airflow is caused by inflammation or fibrosis and destruction of the alveolar architecture. The main risk factor for COPD is smoking; it impairs ciliary mobility, inhibits macrophage function, produces hypertrophy and glandular hyperplasia and acute bronchoconstriction. Other risk factors include exposure to noxious gases, pollutants, and chronic respiratory infections. Symptoms appear around middle age, in individuals who have a history of smoking for several years. Typically, patients present with chronic cough, which may be productive (mucopurulent sputum), and shortness of breath (dyspnea). Exacerbations are caused by respiratory infections. In advanced conditions, dyspnea is observed at rest or upon minimal effort. Symptoms and signs of right heart failure may appear (ankle swelling, bluish color of the skin – cyanosis, and hepatomegaly). Constrictive bronchiolitis Mucus Inflammed bronchiole wall Normal bronchiole Diagnosis is suspected on the basis of clinical presentation. Chest x-ray shows emphysematous changes (hyperinflated lung fields, depressed diaphragm) or nonspecific changes. In severe stage, right heart enlargement may be seen. Diagnosis is confirmed by the demonstrating a nonreversible obstruction of the airflow, on spirometry. Chonic bronchitis Inflammation and structural changes Smoking must be discontinued and exposure to other etiological agents should be minimized. Exercise is advised for all patients, as per clinical condition. Pharmacological treatment consists of bronchodilators; inhaled steroids are recommended for exacerbations. In the causes of exacerbation by respiratory infection, antibiotics may be useful. Anti-flu and anti-pneumococcal vaccines are necessary. In patients with severe disease, oxygen is recommended for several hours a day. Normal Increased mucus Bronchiole Bronchus Emphysema Bronchiole Destruction and enlargement of air spaces Alveoli Chylothorax(18) Accumulation of lymph, rich in long chain fatty acids (chyle), in the pleural cavity. Usually, it is caused by the rupture of the thoracic duct or one of its tributaries. The most frequent causes are tumors and trauma. Symptoms are similar to other pleural effusions. Large collections of lymphatic fluid are also associated with symptoms of malnutrition due to loss of proteins and long chain fatty acids. Pleural tap shows a milky fluid, which doesn’t become clear after centrifugation. Diagnosis is confirmed by demonstrating triglycerides > 110 mg/dl or by the presence of chylomicrons in the pleural fluid. If the cause is traumatic, pleural fluid is drained; surgical ligation of the thoracic duct may be required. Supplementation of diet with middle chain fatty acids (parenteral feeding or oral feeding) may be needed. If it is secondary to neoplasia, chemotherapy or radiotherapy may be effective. Idiopathic Pulmonary Fibrosis(19,20,21) Disease of unknown etiology, characterized by presence of scar tissue within the lungs. Cigarette smoking is strongly associated with IPF. Pathogenesis involves injury to the alveolar epithelium, followed by a burst of pro-inflammatory and fibroproliferative mediators that promote formation of fibrotic tissue. It is most frequently observed in patients between the ages of 50 and 70. The initial symptoms are non-productive cough and dyspnea on exertion. Pulmonary auscultation reveals early inspiratory crackles. Chest x-ray shows bilateral reticular markings that are dominant in the lower zones. HRCT chest shows subpleural honeycombing (palisades of small, round translucencies), traction bronchiectasis and thickened interlobular septae. Biopsy is the gold standard; BAL fluid analysis, and biopsy may be required to exclude other diagnosis. Corticosteroids and other immunesuppressants are used in carefully selected patients. Pulmonary transplant may be indicated in severe cases. Supportive measures, like supplemental oxygen, are used as indicated. RESPIRATORY SYSTEM - Main Pathologies DESCRIPTION SYMPTOMS DIAGNOSIS TREATMENT Larynx Cancer(22,23) It is the most common cancer of head and neck region. The most frequent type is the squamous cell carcinoma; the majority originate in the glottis. It is more frequent in men and is related to smoking and heavy alcohol consumption. The commonest symptom is progressive dysphonia. Occasionally patients may present with abnormal sensation in the throat, dyspnea. Diagnosis is suspected on the basis of symptoms. Laryngoscopy is performed to visualize the lesion and biopsy the lesion. Histopathology (biopsy sample) is used for delineating type of cancer. Tumor size and extent is used for making treatment decisions. In an early stage, the treatment consists of surgery or radiotherapy. When the vocal cords are compromised, radiotherapy is usually preferable, since it preserves voice. Advanced stages require partial or total excision of the larynx (partial or total laryngectomy) with radiotherapy. Lung Cancer(24,25,26,27) It is the commonest cause of cancer related deaths, worldwide. The commonest types are: small cell carcinoma, adenocarcinoma, squamous cell carcinoma and large cell carcinoma. Tobacco smoking is thought to be responsible for the majority of cases of lung cancer; the strongest association is with the squamous cell and small cell types. Among non-smokers, the most frequent is adenocarcinoma. Five year survival after diagnosis is under 50% in case of localized disease, and under 25% if it is disseminated. Patients may remain asymptomatic for months. The commonest symptoms are: persistent cough, chest pain (especially if tumor involves the pleura), fatigue, loss of appetite or weight loss; recurrent pneumonia; secondary to bronchial obstruction and atelectasis; swelling of neck and face; hemoptysis. When neighboring structures are involved by tumor extension, it may cause tracheal obstruction, dysphagia, dysphonia (by compression of the recurrent laryngeal nerve), paralysis of the phrenic nerve, superior vena cava syndrome may also be seen. Blood-borne metastasis and paraneoplastic syndromes are common. Chest x-ray and sputum test are rarely diagnostic. Diagnosis is established by means of tissue biopsy (fibre optic bronchoscopy). The extension of the disease is evaluated by radiological investigations including: computerized axial tomography, MRI, bone scan, mediastinoscopy, etc. Treatment is based on the stage of the disease, specific type of cancer cells, location in the lungs, metastatic spread and the general health of the patient. Various modalities are used including surgery, radiotherapy and chemotherapy. The basis for the treatment of small cells cancer is chemotherapy. In small cell cancer, the first line of treatment is chemotherapy, while surgery is recommended for non-small cell cancers. Nasal Polyp(28,29) Benign lesion arising from the nasal mucosa. It is related to an inflammatory or an allergic process. Nasal polyps are mostly bilateral. Samnter’s Triad consists of nasal polyposis, asthma, and aspirin hypersensitivity. Presents with nasal obstruction, rhinorrhea (watery nasal discharge), and post nasal drip. Decrease in sense of smell and taste is common. Rhinoscopy shows multiple polypoid masses, most frequently arising from the middle meatus. CT scan may be useful if surgery is planned. Medical treatment includes decongestants, antihistaminics and topical corticosteroids. Surgical therapy is reserved for cases refractory to medical treatment. Obstructive Sleep Apnea(30) It is a sleep disorder characterized by occurrence of apnea or hypopnea, repeatedly during sleep, leading to disruption of the sleep architecture. Apneas are characterized by the cessation of airflow for more than 10 s; hypopnea (in adults) is characterized by a reduction in nasal pressure by at least 30% of baseline for a duration of at least 10 s, accompanied by oxygen desaturation ≥ 4%. It is commonest in middle aged, obese men. In most cases, there is a functional impairment of the upper airway dilator muscle. Sleep fragmentation and chronic sleep deprivation lead to excessive daytime sleepiness. Excessive sleepiness during the day and loud snoring at night are the commonest symptoms. Other symptoms include decrease in attention and memory, headache, irritability and sexual dysfunction. OSA is associated with cardiovascular complications; it increases the risk of hypertension, arrhythmias, heart failure, and stroke. It is confirmed by polysomnography, which consists in registering different physiological parameters during the sleep-vigil cycle. The relation between the number of apneas and hypoapneas and the total minutes of sleep generate the apnea-hypoapnea index. Values over 10 are considered pathological. If the condition is mild, measures like weight reduction, avoiding alcohol and sedatives and the use of intra-oral devices may be useful. Continuous positive airway pressure (CPAP) ventilation is the primary treatment for patients with OSA. Very severe cases that don’t respond to treatment may require surgical correction (uvulopalatopharyngoplasty, maxillomandibular surgery and, rarely, tracheostomy). Normal breathing during sleep Obstructive Sleep Apnea Soft palate Tongue Uvula Blocked airway RESPIRATORY SYSTEM - Main Pathologies DESCRIPTION SYMPTOMS DIAGNOSIS TREATMENT Pleural Effusion(31,32) Abnormal accumulation of fluid in the pleural space. It may be: - Transudate: when it is produced by an increase in osmotic gradient across the pleural capillaries, e.g. by a decrease of plasmatic oncotic pressure. - Exudate: when it is produced by an increase in the permeability of the capillary or by a decrease in the lymphatic drainage. The most frequent causes of transudative effusion are: cardiac insufficiency, cirrhosis, nephrotic syndrome and rarely, myxedema. Exudative effusion usually appears in the context of pleuropulmonary infections, cancer, pulmonary thromboembolism, pancreatitis, collagen diseases, etc. Purulent pleural collection, with high leucocyte content or presence of microbes is termed empyema. Small effusions are asymptomatic. The common symptoms include a pleuritic chest pain, dyspnea and dry nonproductive cough. Symptoms of underlying etiologies will be apparent, e.g. fever in patients with an infectious disease. Chest x-ray and the ultrasound are useful for determining the location and volume of the effusion. Thoracocentesis (pleural tap) and examination of the pleural fluid is necessary to ascertain the etiology. In some cases a thoracoscopic pleural biopsy may be necessary. The primary cause should be treated. Large fluid collections and empyemas require placement of a pleural drain. In cases where there is loculation of fluid, intrapleural instillation of fibrinolytics (through the draining tube) may be done to facilitate the exit of pus. Another procedure is the decortication, which implies a thoracotomy with complete removal of existing pus. Pleurodesis consists of applying an irritant (talc, tetracycline, bleomycin) between the two pleural layers (parietal and visceral) aiming to cause an inflammatory reaction that results in the fusion of both layers. It is used in the symptomatic treatment of recurrent significant effusions of a malignant etiology. Pneumonia(33,34) Infectious process of the pulmonary parenchyma. Microorganisms invade the lung via aspiration of colonizers from the oropharynx, via blood from a non-pulmonary focus, or by contiguous spread from a neighboring structure. A failure in the defense mechanisms (ciliary movement, mucus secretion, immune response, etc.) or a very high load of microorganisms predispose to infection. In adults, the commonest etiological agents are Streptococcus pneumoniae, Staphylococcus aureus, Legionella and Haemophilus influenza and various viruses. Mycoplasma pneumoniae is a frequent cause of pneumonia in older children and young adults. Alcohol consumption, smoking, diabetes, cardiac insufficiency and COPD are predisposing factors. Children, the elderly and individuals with alterations in the immune system are more prone to develop pneumonia. Pneumonia is classified as community acquired (CAP; develops within or after 72 hours of hospital discharge), or nosocomial (occurs in patients hospitalized due to other pathology). The typical presentation consists of an acute fever, chills, productive cough and pleuritic chest pain, with high leukocyte count in the peripheral blood. Atypical pneumonia presents subacutely with fever, headache, myalgia (muscle pain), arthralgia (joint pain) and dry cough. The nosocomial pneumonias may be masked by the underlying disease; it must be suspected in patients with fever and new radiological infiltrates. Diagnosis is based on clinical history, examination and radiological investigations. The etiological diagnosis can only be confirmed by sputum’s exam, blood culture and bronchoscopy. In general, invasive procedures are not used regularly, since in a high percentage of CAP the responsible microorganisms are common and treatment is empirical. It is based on antibiotics and supportive measures. The choice of the antibiotic depends on the type of pneumonia (CAP or nosocomial), the type of presentation (typical or atypical), the presence of risk factors for unusual microorganisms (elderly, COPD, severe types of presentation). Antimicrobial choice may be modified by culture and sensitivity reports. Patients with severe disease require hospitalization and may need aggressive support measures including invasive ventilation. Pneumonitis by Hypersensitivity(35,36) Immunologically mediated group of conditions which follow repeated exposure to different antigens. Usually, it is seen as an occupational disease in cattle breeders, farmers, wood workers, pigeon breeders, etc. Exposure to the antigen (fungi, bacteria, animal or plants products) causes an exaggerated inflammatory response, which, when chronic, evolves to pulmonary fibrosis. May occur in an acute, subacute or chronic form. In the acute type, patient presents with fever, chills, dyspnea, cough and myalgia, which disappear a few days after the exposure is discontinued. Tachypnea, tachycardia, and bibasilar inspiratory crackles are usually present. The chronic type is secondary to prolonged exposure, and presents with cough, dyspnea, weight loss; there may be symptoms of pulmonary hypertension. Normal Pneumonia Windpipe (trachea) Lung Bronchiole Bronchus Air sacs (alveoli) Fluid in air sacs It is based on high index of suspicion. Physical examination shows non-specific findings; chest x-ray shows ground glass opacities in acute cases and increased reticular shadows/honeycombing in the chronic cases. Bronchoscopy and bronchoalvelolar lavage is also useful. Identifying and eliminating the causal agent is fundamental. Milder cases usually resolve on their own following discontinuation of exposure. Severe ones require treatment with corticosteroids. RESPIRATORY SYSTEM - Main Pathologies DESCRIPTION SYMPTOMS DIAGNOSIS TREATMENT Pneumothorax(37) Air or gas accumulation in the pleural cavity. It causes the separation of the parietal and visceral layers, leading to compression and collapse of the pulmonary parenchyma. It may be: - traumatic (open or closed trauma); - spontaneous, in thin, young males (rupture of apical bubbles) or in patients with COPD; - hypertensive, when by a valvular mechanism, air penetrates in the pleural space during inspiration and it is retained during expiration; - catamenial, in young women, related to menstruation. A tension pneumothorax is a medical emergency due to rising intrathoracic pressure from progressive air accumulation in the pleural cavity. The clinical presentation depends on the patient’s ventilatory reserve and the degree of lung collapse. Common symptoms include back pain, dyspnea, dry cough, sweating, tachycardia and pallor. In patients with tension pneumothorax, cyanosis and shock are common. It is confirmed by a chest x-ray. For visualizing small pneumothorax (< 15% area of hemithorax), CT scan may be required. Therapeutic options include bed rest, oxygen supplementation, manual aspiration, chest tube drainage, and thoracoscopic and surgical interventions. Small spontaneous pneumothoraxes without ventilatory compromise require only rest and observation. Those associated with respiratory compromise require thoracic draining. To avoid relapses, surgical treatment (by resection of bullae) is helpful in selected patients only. Tension Pneumothorax is considered an emergency that requires immediate administration of oxygen and insertion a high caliber needle in the 2nd intercostal space. The air exit confirms the diagnosis and should remain open until a permanent draining tube is placed. Pulmonary Hypertension(38) Condition characterized by elevated pulmonary arterial pressure (pulmonary artery pressure > 25 mmHg during rest or over 30 mmHg during exercise) and secondary right ventricular failure. Primary pulmonary hypertension is not very frequent and its cause is unknown. Secondary pulmonary hypertension is more frequent and is often the consequence of chronic respiratory or cardiovascular diseases which are associated with low oxygen level in the blood (hypoxemia). The most frequent cause is COPD. Other causes for secondary pulmonary hypertension are: congenital heart diseases, portal hypertension, human immunodeficiency virus (HIV) infection, drugs, chronic exposure to great heights, etc. Hypoxemia induces the constriction of pulmonary vessels (to maintain the gas-exchange equilibrium), which lead to change in the vascular wall and thrombosis. Cor pulmonale is the increase in size of the right ventricle secondary to chronic pulmonary hypertension. In primary pulmonary hypertension, the most frequent symptom is progressive dyspnea, which may occur after several years of onset of disease. Fatigue, weakness, as well as thoracic pain, are also observed; syncope and hemoptysis may occur. In secondary pulmonary hypertension, the symptoms overlap with those of the primary disease process. Physical examination shows signs of right ventricular insufficiency (engorged jugular veins; hepatomegaly, peripheral edema). Electrocardiographic features of right ventricular and atrial enlargement are seen. Cardiac catheterization is useful to directly measure the pulmonary artery pressure, as well as to rule out other secondary causes. Primary pulmonary hypertension is a progressive disease for which there is no cure. Oxygen supplementation is essential for all patients with arterial hypoxemia. Pulmonary transplantation is prescribed for patients with severe right cardiac insufficiency, who do not respond to treatment. Vasodilators, which act on pulmonary circulation, diuretics to reduce fluid retention, and anticoagulants are used. Medications include calcium channel blockers, prostanoids, endothelin receptor antagonists and phosphodiesterase type-5 inhibitors. urgical thrombo-endarterectomy is the primary surgical therapy for selected patients with thromboembolic obstruction of the proximal pulmonary arteries. Pulmonary transplant is prescribed for patients with severe right cardiac insufficiency, who do not respond to treatment. Pulmonary Hypertension Constriction of pulmonary arteries Normal heart Aorta Left atrium Pulmonary arteries (to lungs) Pulmonary veins (from lungs) Right atrium Enlarged right ventricle Right ventricle Left ventricle Lungs RESPIRATORY SYSTEM - Main Pathologies DESCRIPTION SYMPTOMS DIAGNOSIS TREATMENT Pulmonary Thromboembolism (PTE)(39,40) Pulmonary vessel obstruction by one or several emboli. Emboli, in most cases, are fragments of thrombi (clots), which travel through the blood until they obstruct a smaller caliber vessel. The most common source of emboli is thrombosed lower limbs veins or those of the pelvis (deep venous thrombosis). Emboli of fat, amniotic fluid, bone marrow or tumor fragments may also lodge in the pulmonary vasculature. The risk factors for venous thrombosis and pulmonary thromboembolism are: prolonged immobilization, pregnancy, recently diagnosed cancer, oral contraceptives and states of hypercoagulability. When the pulmonary blood flow is acutely interrupted, it creates a shunt (left to right) and leads to hypoxemia. Obstruction of large vessel or obstruction of multiple smaller vessels leads to strain on the right heart and increase in pressures. Dyspnea (a sensation of shortness of breath) and tachypnea (increase of respiratory frequency) of sudden onset are frequent. Acute embolism may present with dizziness, fainting, palpitations and even syncope. The presence of cyanosis (bluish color of the skin), or syncope (sudden loss of consciousness) indicate massive embolism. In a large proportion of cases, signs of deep venous thrombosis of the leg(s) are evident. Pulmonary hypertension, if severe, is followed by acute respiratory insufficiency, right ventricular insufficiency and even death. The diagnosis is suspected on the basis of symptoms in the context of the clinical setting (presence of risk factors). Pulmonary perfusion scintigraphy allows visualization of pulmonary areas with reduced blood flow. It is performed in conjunction with pulmonary ventilation scintigraphy (V/Q scan to look for mismatch, which is diagnostic). CT scan, contrast spiral CT and determination of D-dimers in blood may be useful. D-dimer has a good negativepredictive value, especially in setting of low clinical suspicion. The gold standard for diagnosis is pulmonary angiography. The treatment of suspected PTE is anticoagulation with heparin. Either regular or low molecular weight heparin may be used. In acute cases with significant hemodynamic compromise, and in patients in whom > 50% of pulmonary perfusion is compromised, it is recommended to start treatment with intravenous fibrinolytics. After the acute period, treatment with oral anticoagulants should be continued for 3 to 6 months. In patients with recurring embolism or irreversible risk factors, life-long treatment with anticoagulants may be required. In cases where anticoagulation is contraindicated, inferior vena cava filters or embolectomy may be necessary. Rhinitis(41,42) Inflammation of the mucus membrane that covers the nasal pits. It is defined as the presence of at least one of the following: congestion, rhinorrhea, sneezing, nasal itching, and nasal obstruction. It may be due to viral infections (coryza), allergy, parasympathetic hyper-functioning (vasomotor rhinitis), associated with pregnancy, dry environment and contamination. Common symptoms are rhinorrhea (watery nasal discharge) and decrease in sense of smell; these may be associated with other flu-like symptoms. Allergic rhinitis may be due to a large variety of allergens and presents with repetitive sneezing, rhinorrhea, nasal obstruction and nasal or ocular itching. Vasomotor rhinitis is triggered by temperature change, irritative agents, hypothyroidism and oral contraceptives. Repeated episode of acute rhinitis can lead to a hypertrophic chronic rhinitis, with increase in the size of the inferior turbinate. Dry rhinitis presents as lesions with crusting and signs of bleeding. Ozena is a type of atrophic rhinitis that presents with smelly crusts and anosmia (absence of sense of smell). Usually, a detailed history and clinical exam is enough. Rhinoscopy for visualization of the nasal cavity and the turbinates is performed. The following medications are commonly utilized: decongestants, anti-inflammatories and anti-pyretics. In allergic rhinitis, besides avoiding contact with the allergen, decongestants (antihistamines) and topical corticosteroids are useful. Vasomotor rhinitis may require vidian nerve neurectomy or cryosurgery. Inferior turbinate surgery may be indicated in hypertrophic chronic rhinitis. Dry rhinitis requires abundant hydration. Sarcoidosis(43) Multisystem granulomatous disease of unknown cause, characterized by the formation of noncaseous epithelioid cell granulomas. It involves the respiratory system in > 90% of cases, usually the hilar and mediastinal nodes, and, less frequently, the lung tissue. There is an exaggerated response of the cellular immunity leading to formation of granulomas. The inflammation and coalescence of granulomas alters pulmonary architecture, causes fibrosis, cyst formation, bronchiolitis and bronchiectasis. Pulmonary involvement is frequently asymptomatic or presents with dyspnea and dry cough. Enlargement of the thoracic lymph nodes is frequent. Nasal mucosal membranes may be involved. Laryngeal granulomas may cause dysphonia. Other affected organs are: skin (pernio lupus, erythema nodosum); eyes (uveitis, choroiditis), liver, nervous system, bone marrow, spleen, nervous system, heart, endocrine system and reproductive organs. Diagnosis is confirmed by histopathology, which shows granulomas in the tissue biopsy. However, biopsy may not be possible in many cases and diagnosis is suspected on the basis of the clinico-radiological picture. CT scans of chest are commonly utilized for diagnosis and staging of the disease. The most accessible areas for biopsy are the bronchial tree or the lung (transbronchial biopsy). Blood tests that help in diagnosis include raised serum ACE levels and hypercalcaemia. A third of the cases may remit spontaneously after 1 or 2 years, one third show signs of progression and the rest remains stable. In severe disease or in patients with significant extra-pulmonary involvement, treatment with corticosteroids and other immunosuppressants is recommended. Airway disease is treated symptomatically; corticosteroids (oral/inhaled) are used for clinically significant disease. Systemic Involvement in Sarcoidosis Brain complications Eye problems (burning, itching tearing or pain) Salivary glands Lupus pernio (painful skin sores on the face) and skin lesions on back, arms, neck, face and scalp Enlarged lymph nodes in chest near windpipe and lungs Enlarged lymph nodes in neck and chest Heart complications Scarring and granulomas in lung Granulomas (Inflamed lumps) in lungs Liver enlargement Spleen enlargement Erthema nodosum (itchy and painful rashes) on the lower legs and ankles RESPIRATORY SYSTEM - Main Pathologies DESCRIPTION SYMPTOMS DIAGNOSIS TREATMENT Silicosis(44,45) Pulmonary pathology caused by silica dust inhalation (e.g. in quartzquarry workers). Silica dust is the main element in sand; exposure is frequent among miners, silicaceous rock and granite cutters, foundry workers and potters. When inhaled, silica initiates an inflammatory reaction that leads to pulmonary tissue fibrosis. Silica exposure is classically associated with restrictive lung disease. Usually, symptoms appear after 20 or 30 years of cumulative exposure to the dust. The severity of disease varies from simple or classic type - small lesions (silicotic nodule) that can be observed in the chest x-ray; may be asymptomatic or present with cough and mild dyspnea; - to a severe disease - massive progressive pulmonary fibrosis, which appears when simple nodules converge, forming large masses of fibrotic tissue; presents with severe dyspnea and cough. Silica exposure is also associated with lung cancer, pulmonary tuberculosis and COPD. Diagnosis is based on establishing a history of exposure and correlating the same with clinical findings and radiological investigations (chest x-ray/ CT). The exposure to dust at work should be minimized by measures such as wearing proper masks. It is necessary to perform regular control chest x-rays for the exposed workers. Once fibrosis sets in, the process is irreversible. Treatment is symptomatic, aimed at alleviation of dyspnea. Sinusitis(46,47) Inflammation of the mucus membrane that covers the nasal sinuses. Usually, it is caused by the obstruction of a perinasal sinus, which makes draining difficult and favors super-infection. The obstruction may be favored by deviation of the septum, polyps, decreased immunity (diabetes) or reduction of the mucociliary activity (contamination). The bacterial agents most frequently implicated in acute sinusitis are the Streptococcus pneumoniae, Hemophilus influenzae and Moraxela catarrhalis. A majority of sinusitis, however, is attributable to a viral etiology. In chronic sinusitis, anaerobic agents are also important etiological agents. In children, the sinus most commonly affected is the ethmoid; in the adult, it is the maxillary sinus. Acute sinusitis presents with headache (worsen with manoeuvres that increase the intra-sinus pressure, such as lowering the head), purulent rhinorrhoea and pain on pressure at sinus points. Other symptoms include halitosis (bad breath), fever and changes in the sense of smell. In chronic sinusitis the symptoms are milder, with rhinorrhoea, nasal respiratory insufficiency and a nasal voice (rhinolalia). The history indicates the diagnosis, which is confirmed by clinical examination and xray of the paranasal sinuses. CT scan may be used to complement the x-ray. It may show mucosal thickening, presence of inflammatory discharge and concomitant polyps. Acute cases are treated with antibiotics, vasoconstrictor decongestive medications, aiming to improve the ventilation of the paranasal sinuses. Surgical treatment may be necessary in chronic cases, or acute cases with inadequate or poor response to conventional medical treatment. Deviation of nasal septum Different causes of Sinusitis Nasal cicle (mucociliary clearance) Mucociliary clearance of frontal sinus Ostiomeatal complex Orbit Nasal polyposis Polyp in middle meatus Antral choanal polyp obstructs ostium of maxillary sinus Ostiomeatal obstruction Mucociliary clearance of maxillary sinus Nasal septum Fluid collected in sinus Tonsilitis(48,49) Inflammation of the tonsils, usually of bacterial etiology (streptococcus, staphylococcus), although it may also be viral. The incidence is higher in pre-school age, though it affects the majority of the population sometime during their life. The patient presents with high fever, malaise, pain, difficulty in swallowing and ear pain (reflex otalgia). A frequent complication is the peritonsillar abscess, a collection of pus in the peritonsillar area, which leads to uvular displacement. Systemic complications may appear after a streptococcal infection (glomerulonephritis or rheumatic fever). Examination of the throat shows erythema and congestion of the tonsils. There may be pus points or a thin white membrane over the tonsil. Collecting some pus or mucus from the posterior part of the throat with a swab allows isolation of the causative microorganism. Bacterial tonsillitis requires treatment with antibiotics, painkillers and antipyretics. If there have been seven or more episodes of tonsillitis in a year, five or more in the last two years or three or more in the past three years, a surgical excision of the tonsils may be recommended. Vocal Cords Nodule(50,51) Benign bilaterally symmetric swelling of the true vocal folds (anterior and mid-third of the folds). It is caused by chronic abuse of the voice. It is more frequent in women. It presents itself with dysphonia (hoarseness), episodic voice loss and vocal fatigue. Diagnosis is established by vocal cord examination (indirect laryngoscopy) and biopsy. The treatment starts with voice therapy; if it doesn’t improve, microsurgical excision is performed. Other indications for microsurgical treatment are: longstanding nodule (failure of voice therapy) and suspicion of a primary lesion with a reactive callus on the other vocal fold. Vocal Cords Polyp(50,51) This is a benign mass that develops on the true vocal fold; mostly secondary to voice abuse or vocal trauma. It is unilateral and more common in males. Usually, patients present with chronic dysphonia and vocal fatigue. Indirect laryngoscopy biopsy. Surgical treatment with the excision of the polyp. 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Author: Énio Pestana, MD; Proofreading: Maryna Apalaika and Carla Silva; Final review and bibliography: Monica Saini, MD; Art & Design: Vanessa Augusto and João Freitas; Illustration: Tiago Chagas; Consultant: Jorge Dias, MD. ISBN: 978-989-8488-90-9 Printed in: © 2011 © 2011 (The Publisher) All rights reserved. All rights reserved. Publisher: INFO