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Acute Respiratory Distress Syndrome

Disease Details

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Description: Acute respiratory distress syndrome (ARDS) is a severe lung condition characterized by rapid onset of widespread inflammation in the lungs, leading to respiratory failure.
Type: Acute Respiratory Distress Syndrome (ARDS) is not typically classified as a genetic disorder. It is an acute, inflammatory process affecting the lungs, usually triggered by various direct and indirect insults. There is no specific type of genetic transmission associated with ARDS, although genetic predisposition may influence susceptibility and severity in some individuals.
Signs And Symptoms: The signs and symptoms of ARDS often begin within two hours of an inciting event, but have been known to take as long as 1–3 days; diagnostic criteria require a known insult to have happened within 7 days of the syndrome. Signs and symptoms may include shortness of breath, fast breathing, and a low oxygen level in the blood due to abnormal ventilation. Other common symptoms include muscle fatigue and general weakness, low blood pressure, a dry, hacking cough, and fever.
Prognosis: The overall prognosis of ARDS is poor, with mortality rates of approximately 40%. Exercise limitation, physical and psychological sequelae, decreased physical quality of life, and increased costs and use of health care services are important sequelae of ARDS.
Onset: The onset of Acute Respiratory Distress Syndrome (ARDS) typically occurs within 1 week of a known clinical insult such as pneumonia, sepsis, or trauma, or new or worsening respiratory symptoms.
Prevalence: The prevalence of Acute Respiratory Distress Syndrome (ARDS) varies widely depending on the population and criteria used for diagnosis. It is estimated that ARDS affects around 10% of patients admitted to intensive care units and up to 23% of mechanically ventilated patients. The condition is often underdiagnosed, which can affect prevalence statistics.
Epidemiology: The annual rate of ARDS is generally 13–23 people per 100,000 in the general population. It is more common in people who are mechanically ventilated with acute lung injury (ALI) occurring in 16% of ventilated people. Rates increased in 2020 due to COVID-19, with some cases also appearing similar to HAPE.Worldwide, severe sepsis is the most common trigger causing ARDS. Other triggers include mechanical ventilation, sepsis, pneumonia, Gilchrist's disease, drowning, circulatory shock, aspiration, trauma—especially pulmonary contusion—major surgery, massive blood transfusions, smoke inhalation, drug reaction or overdose, fat emboli and reperfusion pulmonary edema after lung transplantation or pulmonary embolectomy. However, the majority of patients with all these conditions mentioned do not develop ARDS. It is unclear why some people with the mentioned factors above do not develop ARDS and others do.Pneumonia and sepsis are the most common triggers, and pneumonia is present in up to 60% of patients and may be either causes or complications of ARDS. Alcohol excess appears to increase the risk of ARDS. Diabetes was originally thought to decrease the risk of ARDS, but this has shown to be due to an increase in the risk of pulmonary edema. Elevated abdominal pressure of any cause is also probably a risk factor for the development of ARDS, particularly during mechanical ventilation.
Intractability: Acute Respiratory Distress Syndrome (ARDS) is generally considered challenging to treat rather than strictly intractable. Its management is complex and usually requires intensive care, including mechanical ventilation and supportive therapies. The outcome can vary significantly depending on the underlying cause, the patient's overall health, and timely medical intervention. Some patients recover fully, while others may suffer from long-term complications or high mortality risk.
Disease Severity: Acute Respiratory Distress Syndrome (ARDS) is considered a severe condition. It is characterized by rapid onset of widespread inflammation in the lungs, leading to respiratory failure. The severity of ARDS can vary, but it is often life-threatening and demands immediate medical intervention.
Healthcare Professionals: Disease Ontology ID - DOID:11394
Pathophysiology: ARDS is a form of fluid accumulation in the lungs not explained by heart failure (noncardiogenic pulmonary edema). It is typically provoked by an acute injury to the lungs that results in flooding of the lungs' microscopic air sacs responsible for the exchange of gases such as oxygen and carbon dioxide with capillaries in the lungs. Additional common findings in ARDS include partial collapse of the alveoli(atelectasis) and low levels of oxygen in the blood (hypoxemia). The clinical syndrome is associated with pathological findings including pneumonia, eosinophilic pneumonia, cryptogenic organizing pneumonia, acute fibrinous organizing pneumonia, and diffuse alveolar damage (DAD). Of these, the pathology most commonly associated with ARDS is DAD, which is characterized by a diffuse inflammation of lung tissue. The triggering insult to the tissue usually results in an initial release of chemical signals and other inflammatory mediators secreted by local epithelial and endothelial cells.Neutrophils and some T-lymphocytes quickly migrate into the inflamed lung tissue and contribute in the amplification of the phenomenon. The typical histological presentation involves diffuse alveolar damage and hyaline membrane formation in alveolar walls. Although the triggering mechanisms are not completely understood, recent research has examined the role of inflammation and mechanical stress.One research group has reported that broncho-alveolar lavage fluid in later-stage ARDS often contains trichomonads, in an amoeboid form (i.e. lacking their characteristic flagellum) which makes them difficult to identify under the microscope.
Carrier Status: Acute Respiratory Distress Syndrome (ARDS) is not a genetic condition, so the concept of carrier status does not apply. ARDS is typically caused by factors such as severe infections, trauma, or inhalation injuries that lead to widespread inflammation in the lungs.
Mechanism: Acute Respiratory Distress Syndrome (ARDS) is characterized by rapid onset of widespread inflammation in the lungs. The primary mechanisms and molecular mechanisms involved include:

1. **Inflammation and Cytokine Release**: ARDS is often triggered by an underlying condition, such as sepsis, pneumonia, or trauma. This leads to an excessive inflammatory response, with immune cells releasing cytokines like TNF-α, IL-1, and IL-6.

2. **Increased Vascular Permeability**: The inflammatory cytokines increase the permeability of the alveolar-capillary barrier. This allows protein-rich fluid to leak into the alveoli, causing pulmonary edema.

3. **Neutrophil Activation**: Activated neutrophils migrate to the lungs and release reactive oxygen species (ROS) and proteases, damaging pulmonary tissue and contributing to alveolar injury.

4. **Epithelial and Endothelial Cell Damage**: The injuries to alveolar epithelial and capillary endothelial cells impair gas exchange, reduce lung compliance, and decrease oxygenation.

5. **Surfactant Dysfunction**: Damage to type II alveolar cells reduces the production and function of surfactant, which increases surface tension and leads to alveolar collapse (atelectasis).

6. **Coagulation Pathway Activation**: ARDS is associated with activation of the coagulation cascade, leading to fibrin deposition within the alveoli and microvascular thrombosis.

7. **Activation of NF-κB Pathway**: The nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB) pathway is a key molecular pathway involved in the inflammatory response of ARDS. Its activation leads to transcription of pro-inflammatory genes and perpetuates inflammation.

Understanding the interplay of these mechanisms provides insight into the pathophysiology of ARDS and can inform therapeutic strategies.
Treatment: Acute respiratory distress syndrome is usually treated with mechanical ventilation in the intensive care unit (ICU). Mechanical ventilation is usually delivered through a rigid tube which enters the oral cavity and is secured in the airway (endotracheal intubation), or by tracheostomy when prolonged ventilation (≥2 weeks) is necessary. The role of non-invasive ventilation is limited to the very early period of the disease or to prevent worsening respiratory distress in individuals with atypical pneumonias, lung bruising, or major surgery patients, who are at risk of developing ARDS. Treatment of the underlying cause is crucial. Appropriate antibiotic therapy is started as soon as culture results are available, or if infection is suspected (whichever is earlier). Empirical therapy may be appropriate if local microbiological surveillance is efficient. Where possible the origin of the infection is removed. When sepsis is diagnosed, appropriate local protocols are followed.
Compassionate Use Treatment: Acute Respiratory Distress Syndrome (ARDS) often requires supportive care and mechanical ventilation. While there are no specific FDA-approved treatments for ARDS, several off-label or experimental treatments have been explored:

1. **Corticosteroids**: These are sometimes used off-label to reduce inflammation in the lungs, though their efficacy remains controversial and they are generally more considered for later stages of ARDS.

2. **Prone Positioning**: While not a pharmacological treatment, prone positioning of patients can improve oxygenation and is widely practiced.

3. **Extracorporeal Membrane Oxygenation (ECMO)**: This is an invasive procedure used in severe cases to provide oxygen to the blood externally, effectively allowing the lungs to rest and heal.

4. **Inhaled Nitric Oxide**: This experimental treatment aims to improve oxygenation by dilating blood vessels in the lungs, though its benefits are still under review.

5. **Mesenchymal Stem Cells**: These are being researched for their potential to repair lung tissue and reduce inflammation.

6. **FDA Emergency Use Authorization (EUA) for COVID-19 related ARDS**: Certain antiviral and immunomodulating agents have been used under EUA during the COVID-19 pandemic.

7. **Anakinra**: An interleukin-1 receptor antagonist that's been considered for severe inflammatory responses in ARDS.

8. **Statins**: These cholesterol-lowering drugs are being studied for their potential anti-inflammatory and protective lung effects.

These treatments are still under investigation, and their use should be guided by clinical trials and specialist recommendations.
Lifestyle Recommendations: For Acute Respiratory Distress Syndrome (ARDS), lifestyle recommendations primarily focus on prevention and overall lung health rather than direct management of the condition, as ARDS often requires intensive medical treatment. However, to reduce the risk and promote respiratory health:

1. **Avoid Smoking**: Refrain from smoking and avoid secondhand smoke exposure to preserve lung function.
2. **Healthy Diet**: Maintain a balanced diet rich in antioxidants, vitamins, and minerals to support immune function.
3. **Regular Exercise**: Engage in regular physical activity to enhance cardiovascular and respiratory health.
4. **Vaccinations**: Keep up-to-date with vaccinations, including influenza and pneumonia vaccines, to prevent infections that can lead to ARDS.
5. **Infection Control**: Practice good hand hygiene and avoid contact with individuals who have respiratory infections.
6. **Manage Chronic Conditions**: Effectively manage chronic diseases like diabetes and cardiovascular conditions that can predispose to severe respiratory issues.
7. **Avoid Pollutants**: Reduce exposure to environmental and occupational pollutants. Use protective gear if exposure is inevitable.

For those recovering from ARDS, follow physician-recommended rehabilitation programs and smoking cessation if applicable to promote recovery and lung function restoration.
Medication: As of 2019, it is uncertain whether or not treatment with corticosteroids improves overall survival. Corticosteroids may increase the number of ventilator-free days during the first 28 days of hospitalization. One study found that dexamethasone may help. The combination of hydrocortisone, ascorbic acid, and thiamine also requires further study as of 2018.Inhaled nitric oxide (NO) selectively widens the lung's arteries which allows for more blood flow to open alveoli for gas exchange. Despite evidence of increased oxygenation status, there is no evidence that inhaled nitric oxide decreases morbidity and mortality in people with ARDS. Furthermore, nitric oxide may cause kidney damage and is not recommended as therapy for ARDS regardless of severity.Alvelestat (AZD 9668) had been quoted according to one review article.
Repurposable Drugs: For acute respiratory distress syndrome (ARDS), repurposable drugs under investigation include:

1. **Corticosteroids**: Such as dexamethasone, which can reduce inflammation and modulate immune response.
2. **Statins**: Like simvastatin, believed to have anti-inflammatory and endothelial-stabilizing effects.
3. **Antiviral agents**: Remdesivir has been studied, especially in the context of COVID-19-related ARDS.
4. **Antimalarials**: Hydroxychloroquine has been evaluated, although its efficacy is still under scrutiny.
5. **Monoclonal antibodies**: Tocilizumab, which targets the IL-6 receptor to reduce inflammatory cytokine storms.

Clinical effectiveness and safety of these drugs vary and are subject to ongoing research and clinical trials.
Metabolites: Acute Respiratory Distress Syndrome (ARDS) is not typically associated directly with specific metabolites in routine clinical practice. However, research has identified certain biochemical changes and metabolite alterations in ARDS patients. These include changes in markers of inflammation, oxidative stress, and cellular damage, such as elevated levels of lactate, free fatty acids, and various cytokines. Additionally, some studies have reported altered lipid metabolism and changes in amino acid levels in ARDS patients. These metabolic shifts reflect the body's response to severe lung injury and inflammation that characterize ARDS.
Nutraceuticals: There is limited evidence to support the use of nutraceuticals (such as dietary supplements, vitamins, and herbal products) in treating Acute Respiratory Distress Syndrome (ARDS). While some studies suggest potential benefits from specific agents like omega-3 fatty acids or antioxidants, these findings are not conclusive, and more research is needed. It is important to follow established medical treatments and consult with healthcare professionals regarding the use of any supplements in ARDS management.
Peptides: Acute Respiratory Distress Syndrome (ARDS) involves severe lung inflammation and damage, leading to impaired oxygen exchange. Research on peptides is ongoing, focusing on their potential to reduce inflammation and promote lung repair. Nanotechnology, particularly nanoparticles, is being explored for targeted drug delivery to improve the precision and effectiveness of treatments. These advanced methods aim to enhance therapeutic outcomes and reduce side effects in ARDS management.