The average age of onset is the early to mid 30s. Exertional dyspnea and spontaneous pneumothorax have been reported as the initial presentation of the disease in 49% and 46% of patients, respectively.

Diagnosis is typically delayed 5 to 6 years. The condition is often misdiagnosed as asthma or chronic obstructive pulmonary disease. The first pneumothorax precedes the diagnosis of LAM in 82% of patients. The consensus clinical definition of LAM includes multiple symptoms:

- Fatigue

- Cough

- Hemoptysis (rarely massive)

- Chest pain

- Chylous complications arising from lymphatic obstruction, including

- Chylothorax

- Chylous ascites

- Chylopericaridium

- Chyloptysis

- Chyluria

- Chyle in vaginal discharge

- Chyle in stool.

- Angiomyolipomas (fatty kidney tumors) are present in about 30% of patients with sporadic LAM and up to 90% of patients with TSC-LAM. Angiomyolipomas can sometimes spontaneously bleed, causing pain or hypotension.

- Cystic lymphangiomas or lymph nodes with hypodense centers, which mimic necrotizing lymphomas, ovarian or renal cancers, or other malignancies can occur in the retroperitoneum, pelvis or mediastinum.

Lung destruction in LAM is a consequence of diffuse infiltration by neoplastic smooth muscle-like cells that invade all lung structures including the lymphatics, airway walls, blood vessels and interstitial spaces. The consequences of vessel and airway obstruction include chylous fluid accumulations, hemoptysis, airflow obstruction and pneumothorax. The typical disease course displays progressive dyspnea on exertion, spaced by recurrent pneumothoraces and in some patients, chylous pleural effusions or ascites.

Most people have dyspnea on exertion with daily activities by 10 years after symptom onset. Many patients require supplemental oxygen over that interval.

According to the International Labour Office (ILO), PMF requires the presence of large opacity exceeding 1 cm (by x-ray). By pathology standards, the lesion in histologic section must exceed 2 cm to meet the definition of PMF. In PMF, lesions most commonly occupy the upper lung zone, and are usually bilateral. The development of PMF is usually associated with a restrictive ventilatory defect on pulmonary function testing. PMF can be mistaken for bronchogenic carcinoma and vice versa. PMF lesions tend to grow very slowly, so any rapid changes in size, or development of cavitation, should prompt a search for either alternative cause or secondary disease.

Tumor-like disorders of the lung pleura are a group of conditions that on initial radiological studies might be confused with malignant lesions. Radiologists must be aware of these conditions in order to avoid misdiagnosing patients. Examples of such lesions are: pleural plaques, thoracic splenosis, catamenial pneumothorax, pleural pseudotumor, diffuse pleural thickening, diffuse pulmonary lymphangiomatosis and Erdheim-Chester Disease.

In disorders that are intrinsic to the lung parenchyma, the underlying process is usually pulmonary fibrosis (scarring of the lung). As the disease progresses, the normal lung tissue is gradually replaced by scar tissue interspersed with pockets of air. This can lead to parts of the lung having a honeycomb-like appearance.

Symptoms can vary greatly, but they include a persistent dry cough.

Exposure to asbestos fibers reach the pleura of the lungs through the lymphatic channels or blood stream. Historically, ship builders and insulation workers are at greater risk.

Affected persons are usually asymptomatic.

On radiological studies, pleural plaques are visualized using conventional chest x-rays and computed tomography scans (CT scans). The locations of the lesions are mostly in the parietal pleura of the lungs, especially in the posterior/lateral regions of the thorax, diaphragmatic domes, and lung fissures. In some cases, calcifications are also evident, especially with CT scans.

No treatment is required since pleural plaques are benign. However, studies have demonstrated that pleural plaques are an independent risk factor for developing bronchogenic carcinoma and/or mesothelioma.

Restrictive lung diseases (or restrictive ventilatory defects) are a category of extrapulmonary, pleural, or parenchymal respiratory diseases that restrict lung expansion, resulting in a decreased lung volume, an increased work of breathing, and inadequate ventilation and/or oxygenation. Pulmonary function test demonstrates a decrease in the forced vital capacity.

Progressive Massive Fibrosis (PMF), characterized by the development of large conglomerate masses of dense fibrosis (usually in the upper lung zones), can complicate silicosis and coal worker's pneumoconiosis. Conglomerate masses may also occur in other pneumoconioses, such as talcosis, berylliosis (CBD), kaolin pneumoconiosis, and pneumoconiosis from carbon compounds, such as carbon black, graphite, and oil shale. Conglomerate masses can also develop in sarcoidosis, but usually near the hilae and with surrounding paracitricial emphysema.

The disease arises firstly through the deposition of silica or coal dust (or other dust) within the lung, and then through the body's immunological reactions to the dust.

Signs and symptoms of flock worker's lung include rales (crackling noises caused by fluid in the lungs), dyspnea (shortness of breath), and coughing. Abnormalities seen on a computed tomography (CT) scan of the lungs can include ground glass opacity and reticular opacity. The typical histopathology in flock worker's lung is bronchiolocentric interstitial pneumonitis and lymphocytic bronchiolitis with lymphocytic hyperplasia. Occasionally, desquamative interstitial pneumonia and bronchiolitis obliterans organizing pneumonia can be seen.

Other symptoms described in flock workers include pleuritic chest pain and atypical chest pain. Most cases described have been chronic and progressive. Lung function in individuals with flock worker's lung is generally diminished, with both restrictive and obstructive defects found.

Lymphangioleiomyomatosis (LAM) is a rare, progressive and systemic disease that typically results in cystic lung destruction. It predominantly affects women, especially during childbearing years.

A primary spontaneous pneumothorax (PSP) tends to occur in a young adult without underlying lung problems, and usually causes limited symptoms. Chest pain and sometimes mild breathlessness are the usual predominant presenting features. People who are affected by PSPs are often unaware of potential danger and may wait several days before seeking medical attention. PSPs more commonly occur during changes in atmospheric pressure, explaining to some extent why episodes of pneumothorax may happen in clusters. It is rare for PSPs to cause tension pneumothoraces.

Secondary spontaneous pneumothoraces (SSPs), by definition, occur in individuals with significant underlying lung disease. Symptoms in SSPs tend to be more severe than in PSPs, as the unaffected lungs are generally unable to replace the loss of function in the affected lungs. Hypoxemia (decreased blood-oxygen levels) is usually present and may be observed as cyanosis (blue discoloration of the lips and skin). Hypercapnia (accumulation of carbon dioxide in the blood) is sometimes encountered; this may cause confusion and – if very severe – may result in comas. The sudden onset of breathlessness in someone with chronic obstructive pulmonary disease (COPD), cystic fibrosis, or other serious lung diseases should therefore prompt investigations to identify the possibility of a pneumothorax.

Traumatic pneumothorax most commonly occurs when the chest wall is pierced, such as when a stab wound or gunshot wound allows air to enter the pleural space, or because some other mechanical injury to the lung compromises the integrity of the involved structures. Traumatic pneumothoraces have been found to occur in up to half of all cases of chest trauma, with only rib fractures being more common in this group. The pneumothorax can be occult (not readily apparent) in half of these cases, but may enlarge - particularly if mechanical ventilation is required. They are also encountered in patients already receiving mechanical ventilation for some other reason.

Upon physical examination, breath sounds (heard with a stethoscope) may be diminished on the affected side, partly because air in the pleural space dampens the transmission of sound. Measures of the conduction of vocal vibrations to the surface of the chest may be altered. Percussion of the chest may be perceived as hyperresonant (like a booming drum), and vocal resonance and tactile fremitus can both be noticeably decreased. Importantly, the volume of the pneumothorax can show limited correlation with the intensity of the symptoms experienced by the victim, and physical signs may not be apparent if the pneumothorax is relatively small.

Failure to have a pulmonary sequestration removed can lead to a number of complications. These include:

- Hemorrhage that can be fatal.

- The creation of a left-right shunt, where blood flows in a shortcut through the feed off the aorta.

- Chronic infection. Diseases such as bronchiectasis, tuberculosis, aspergillosis, bronchial carcinoid and bronchogenic squamous cell carcinoma.

A Simon focus is a tuberculosis (TB) nodule that can form in the apex of the lung when a primary TB infection elsewhere in the body spreads to the lung apex via the bloodstream. Simon focus nodules are often calcified.

The initial lesion is usually a small focus of consolidation, less than 2cm in diameter and located within 1 to 2 cm of the apical pleura. In adolescence, Simon foci may become reactivated and develop into Assmann foci. Such foci are sharply circumscribed, firm, gray-white to yellow areas that have a variable amount of central caseation and peripheral fibrosis.

A pneumatocele is a cavity in the lung parenchyma filled with air that may result from pulmonary trauma during mechanical ventilation.

Although multiple definitions exist, a tension pneumothorax is generally considered to be present when a pneumothorax (primary spontaneous, secondary spontaneous, or traumatic) leads to significant impairment of respiration and/or blood circulation. Tension pneumothorax tends to occur in clinical situations such as ventilation, resuscitation, trauma, or in patients with lung disease.

The most common findings in people with tension pneumothorax are chest pain and respiratory distress, often with an increased heart rate (tachycardia) and rapid breathing (tachypnea) in the initial stages. Other findings may include quieter breath sounds on one side of the chest, low oxygen levels and blood pressure, and displacement of the trachea away from the affected side. Rarely, there may be cyanosis (bluish discoloration of the skin due to low oxygen levels), altered level of consciousness, a hyperresonant percussion note on examination of the affected side with reduced expansion and decreased movement, pain in the epigastrium (upper abdomen), displacement of the apex beat (heart impulse), and resonant sound when tapping the sternum. This is a medical emergency and may require immediate treatment without further investigations (see below).

Tension pneumothorax may also occur in someone who is receiving mechanical ventilation, in which case it may be difficult to spot as the person is typically receiving sedation; it is often noted because of a sudden deterioration in condition. Recent studies have shown that the development of tension features may not always be as rapid as previously thought. Deviation of the trachea to one side and the presence of raised jugular venous pressure (distended neck veins) are not reliable as clinical signs.

Diagnosis can be made using chest X-ray; the lesion shows up as a small, round area filled with air. Computed tomography can give a more detailed understanding of the lesion. Differential diagnoses, other conditions that could cause similar symptoms as pneumatocele, include lung cancer, tuberculosis, and a lung abscess in the setting of Hyper IgE syndrome (aka Job's syndrome) or on its own, often caused by Staphylococcus aureus infection during cystic fibrosis.

Onset of symptoms is often gradual, but in necrotizing staphylococcal or gram-negative bacillary pneumonias patients can be acutely ill. Cough, fever with shivering, and night sweats are often present. Cough can be productive of foul smelling purulent mucus (≈70%) or less frequently with blood in one third of cases). Affected individuals may also complain of chest pain, shortness of breath, lethargy and other features of chronic illness.

Those with a lung abscess are generally cachectic at presentation. Finger clubbing is present in one third of patients. Dental decay is common especially in alcoholics and children. On examination of the chest there will be features of consolidation such as localized dullness on percussion and bronchial breath sounds.

Flock worker's lung is an occupational lung disease caused by exposure to flock, small fibers that are glued to a backing in order to create a specific texture. People who work in flocking are at risk of inhaling the small fibers, which causes interstitial lung disease. The disease was initially described in 1998, when a group of workers at a flocking plant developed interstitial lung disease of unknown cause.

Rare nowadays but include spread of infection to other lung segments, bronchiectasis, empyema, and bacteremia with metastatic infection such as brain abscess.

Symptoms may include coughing, an upper respiratory tract infection, shortness of breath, and chest pain. These symptoms are very non-specific, and can be caused by other types of tumor in the lung or mediastinum more generally, and by other conditions. Imaging (X-ray, CT, MRI) may be used to determine the presence and precise location of a tumor, but not a specific diagnosis of PPB or other tumor.

Doctors are unable to tell if a child has PPB right away, and not upper respiratory tract infection, until more test are taken and they show that there is no infection. Another symptom is pneumothorax.

A Ghon focus is a primary lesion usually subpleural, often in the mid to lower zones, caused by "Mycobacterium bacilli" (tuberculosis) developed in the lung of a nonimmune host (usually a child). It is named for Anton Ghon (1866–1936), an Austrian pathologist.

It is a small area of granulomatous inflammation, only detectable by chest X-ray if it calcifies or grows substantially (see tuberculosis radiology). Typically these will heal, but in some cases, especially in immunosuppressed patients, it will progress to miliary tuberculosis (so named due to the granulomas resembling millet seeds on a chest X-ray).

The classical location for primary infection is surrounding the lobar fissures, either in the upper part of the lower lobe or lower part of the upper lobe.

If the Ghon focus also involves infection of adjacent lymphatics and hilar lymph nodes, it is known as the Ghon's complex or primary complex. When a Ghon's complex undergoes fibrosis and calcification it is called a Ranke complex.

Ventilator-associated lung injury (VALI) is an acute lung injury that develops during mechanical ventilation and is termed ventilator-induced lung injury (VILI) if it can be proven that the mechanical ventilation caused the acute lung injury. In contrast, ventilator-associated lung injury (VALI) exists if the cause cannot be proven. VALI is the appropriate term in most situations because it is virtually impossible to prove what actually caused the lung injury in the hospital.

It most often arises centrally in larger bronchi, and while it often metastasizes to locoregional lymph nodes (particularly the hilar nodes) early in its course, it generally disseminates outside the thorax somewhat later than other major types of lung cancer. Large tumors may undergo central necrosis, resulting in cavitation. A squamous-cell carcinoma is often preceded for years by squamous-cell metaplasia or dysplasia in the respiratory epithelium of the bronchi, which later transforms to carcinoma in situ.

In carcinoma in situ, atypical cells may be identified by cytologic smear test of sputum, bronchoalveolar lavage or samples from endobronchial brushings. However, squamous-cell carcinoma in situ is asymptomatic and undetectable on X-ray radiographs.

Eventually, it becomes symptomatic, usually when the tumor mass begins to obstruct the lumen of a major bronchus, often producing distal atelectasis and infection. Simultaneously, the lesion invades into the surrounding pulmonary substance. On histopathology, these tumors range from well differentiated, showing keratin pearls and cell junctions, to anaplastic, with only minimal residual squamous-cell features.

Atypical adenomatous hyperplasia is a subtype of pneumocytic hyperplasia in the lung. It can be a precursor lesion of in situ adenocarcinoma of the lung (bronchioloalveolar carcinoma).

In prostate tissue biopsy, it can be confused for adenocarcinoma of the prostate. The needle biopsy rate is less than 1%.

Pulmonary neuroendocrine tumors are neuroendocrine tumors localized to the lung: bronchus or pulmonary parenchyma.

Pulmonary neuroendocrine tumors include a spectrum of tumors from the low-grade typical pulmonary carcinoid tumor and intermediate-grade atypical pulmonary carcinoid tumor to the high-grade pulmonary large cell neuroendocrine carcinoma (LCNEC) and pulmonary small cell carcinoma (SCLC), with significant clinical, epidemiologic and genetic differences.