Introduction
Background
Acute respiratory distress syndrome (ARDS) was first described by Ashbaugh and colleagues in 1967.1 The authors reported the condition as an acute onset of severe respiratory distress, cyanosis (hypoxemia) that is refractory to oxygen therapy, diffuse abnormalities on chest radiographs (CXRs), and decreased lung compliance. In 1994, the American-European Consensus Conference (AECC) on ARDS formulated their definition of ARDS as follows2 :
- Acute onset of symptoms
- The ratio of the alveolar partial pressure of oxygen (PaO2) to the fraction of inspired oxygen (FIO2) of 200 mm Hg or less
- Bilateral infiltrates on CXRs
- Pulmonary arterial wedge pressure of 18 mm Hg or less or no clinical signs of left atrial hypertension
The radiographic abnormalities of ARDS reflect the leakage of fluid with a high protein content into the alveolar spaces because of alveolar epithelial injury, or diffuse alveolar damage. ARDS is a syndrome that is defined by its clinical features. The condition may result from intrathoracic or extrathoracic events of various etiologies, such as inflammatory, infectious, vascular, or traumatic etiologies. Determining the causative event may be clinically important for proper treatment.3,4,5,6,7
ARDS is a syndrome that commonly begins after exposure to a known risk factor. Why some people develop ARDS and others do not is still unknown. The risk factors for ARDS include primary pulmonary etiologies (eg, aspiration, pneumonia, toxic inhalation, pulmonary contusion) and extrapulmonary etiologies (eg, sepsis, pancreatitis, multiple blood transfusions, trauma, use of drugs such as heroin). Sometimes, ARDS is not only a reaction to another event but also the result of a known cause, such as an acute interstitial pneumonia (AIP) or a severe, extensive, infectious pneumonia.
For excellent patient education resources, visit eMedicine's Lung and Airway Center. Also, see eMedicine's patient education article Acute Respiratory Distress Syndrome.
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Pathophysiology
The diagnostic criterion standard for acute respiratory distress syndrome (ARDS) is pathologic evidence of diffuse alveolar damage that has been obtained from lung tissue via biopsy. However, biopsy may not be possible because of the patient's clinical condition. If a biopsy is performed, ARDS can be categorized by its pathologic phases, which are similar regardless of the cause of ARDS. The pathologic findings often follow a similar time course, but this can vary between patients. The ARDS phases are as follows:
- The exudative phase occurs within hours after the initial pulmonary insult and usually lasts 2-7 days. At this early stage of ARDS, the clinical findings are correlated with the microscopic findings of hyaline membranes, loss of the alveolar epithelium, edema, and hemorrhage (see Image 5).
- The proliferative phase, which usually occurs 7-28 days after the initial pulmonary insult, follows the exudative phase. In this early proliferative phase, type 2 pneumocytic proliferation is present, along with widening of the septa and interstitial fibroblast proliferation (see Image 6).
- The late proliferative, or fibrotic, phase of ARDS is the result of cellular proliferation that leads to the deposition of collagen and proteoglycans. Extensive fibroblast proliferation with incorporation of the hyaline membranes is a characteristic finding in this stage of ARDS (see Image 7).
- Interstitial fibrosis develops in some patients. Pulmonary vascular abnormalities are common, such as microvascular thrombi and vascular remodeling.
Frequency
United States
The annual incidence of acute respiratory distress syndrome (ARDS) has been reported to be 150,000 cases; however, this number is suspect because of differing definitions for ARDS. Previously, the National Institutes of Health (NIH) Acute Respiratory Distress Syndrome Network enrolled many ARDS patients into clinical trials. The ARDS incidence rate estimates from those trials agreed with an earlier NIH estimate of 75 cases per 100,000 persons per year.
International
The incidence for acute respiratory distress syndrome (ARDS) is about 18 cases per 100,000 persons per year for acute lung injury (ALI) and 13.5 cases per 100,000 persons per year for ARDS, as reported by the Acute Respiratory Failure (ARF) Study Group in Sweden, Denmark, and Iceland.8
Mortality/Morbidity
Mortality in acute respiratory distress syndrome (ARDS) is commonly secondary to multiorgan dysfunction. Less alveolar epithelial damage indicates a better likelihood of the patient recovering pulmonary function.9,10
Differential Diagnoses
Aspiration Pneumonia
Congestive Heart Failure
Pneumonia, Atypical Bacterial
Pneumonia, Pneumocystis Carinii
Pneumonia, Typical Bacterial
Pneumonia, Viral
Other Problems to Be Considered
Diffuse pneumonia of any origin (although pneumonia can be a cause of ARDS)
Cardiogenic edema
Massive aspiration (although aspiration, too, can be a cause of ARDS)
Pulmonary hemorrhage
Severe acute respiratory syndrome (SARS)
Congestive heart failure (CHF) can mimic ARDS. A Swan-Ganz catheter is used to measure the left ventricular end-diastolic pressure to rule out CHF. According to the AECC definition, the pulmonary artery wedge pressure (pulmonary artery occlusion pressure) for ARDS, as measured with a Swan-Ganz catheter, should be 18 mm Hg or less.
Some investigators believe that distinguishing CHF from ARDS may be difficult and arbitrary at times, and they propose a classification for permeability edema. The 4 categories are as follows: (1) hydrostatic edema, (2) permeability edema due to diffuse alveolar damage or ARDS, (3) permeability edema without diffuse alveolar damage, or (4) mixed (hydrostatic and permeability edema).11
The cause of ARDS is commonly an immune reaction to an otherwise nonrelated event; other times, the condition results from a direct insult to the lung that causes pathologically identical changes as ARDS. This is the case for diffuse pneumonias of any origin and is commonly seen with viral pneumonia. SARS is a good example of a probable infectious pneumonia that is ARDS, pathologically and clinically. Experts have speculated that the cause is from a coronavirus that may be transmitted via respiratory secretions and develops after 2-11 days of a febrile illness.
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Further Reading
Keywords
ARDS, acute respiratory distress syndrome, adult respiratory distress syndrome, pulmonary insufficiency, lung disease, respiratory tract disease, respiratory failure, respiratory syndrome, respiratory distress syndrome, severe respiratory distress, cyanosis, hypoxemia, diffuse alveolar damage, acute lung injury, ALI
Overview: Acute Respiratory Distress Syndrome