Acute Respiratory Distress Syndrome

National Organization for Rare Disorders, Inc.

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Important

It is possible that the main title of the report Acute Respiratory Distress Syndrome is not the name you expected.

Disorder Subdivisions

  • None

General Discussion

Acute respiratory distress syndrome (ARDS) is a type of severe, acute lung dysfunction affecting all or most of both lungs that occurs as a result of illness or injury. Although it is sometimes called adult respiratory distress syndrome, it may also affect children. Major symptoms may include breathing difficulties (dyspnea), rapid breathing (tachypnea), excessively deep and rapid breathing (hyperventilation) and insufficient levels of oxygen in the circulating blood (hypoxemia). ARDS may develop in conjunction with widespread infection in the body (sepsis) or as a result of pneumonia, trauma, shock, severe burns, aspiration of food into the lung, multiple blood transfusions, and inhalation of toxic fumes, among other things. It usually develops within 24 to 48 hours after the original illness or injury and is considered a medical emergency. It may progress to involvement of other organs.

Symptoms

Typically, ARDS develops within 24 to 48 hours of the original illness or injury. It may become a life-threatening condition characterized by inflammation of the lungs, which may begin in one lung but eventually affects both, and resulting damage to the air sacs (alveoli) and surrounding small blood vessels. The damaged alveoli close down or fill up with fluid (lung edema), thereby losing their ability to oxygenate the blood and eliminate carbon dioxide. Patients experience increasingly severe respiratory distress, associated with decreasing oxygen levels in arterial blood and tissues.



With the fluid buildup, the lungs become heavy, stiff, and unable to expand properly. Most patients require mechanical ventilation because of respiratory failure. The disorder may also be accompanied or followed by impairment of other vital functions, including cardiovascular, renal, hepatic, hematologic, and neurologic functions. Involvement of other organs in addition to the lungs may lead to a condition sometimes called multi-organ dysfunction syndrome.



The person with ARDS may initially appear agitated as a result of breathing difficulty, but later may become lethargic and or even comatose. He may appear pale, and the hands and feet may have a bluish-gray tone because of the diminished level of oxygen in the blood.

Causes

Risk factors for developing acute respiratory distress syndrome include infection in the body (sepsis), pneumonia, extensive trauma, severe low blood pressure (shock), severe burns, aspiration of food into the lung, inflammation of the pancreas, and multiple emergency blood transfusions. The disorder may also follow a near drowning or the inhalation of toxic fumes, or gases such as chlorine, phosgene, and nitrogen dioxide. Acute respiratory distress syndrome may affect people who have previously had healthy lungs, and it may affect children. It is not the same thing as infant respiratory distress syndrome, although the two share some similarities.

Affected Populations

Acute respiratory distress syndrome can affect persons of any age who suffer acute injury or illness affecting the lungs. The incidence is believed to be between 1.5 and 4.8 per 100,000 of the population. Men and women appear to be equally affected.

Diagnosis

The diagnosis is based on the presence of respiratory distress accompanied by low levels of oxygen in the blood and the presence of known risk factors such as sepsis, pneumonia, or trauma. Chest x-rays will show fluid filling spaces that should be filled with air. The presence of fluid in the air sacs and the "wet" breathing sounds sometimes made by patients may suggest congestive heart failure but a medical examination will distinguish between that condition and ARDS.

Standard Therapies

Treatment



Standard therapy consists of mechanical ventilation, supplemental oxygen, and a technique called positive end expiratory pressure (PEEP) to help push the fluid out of air sacs. These are combined with continuing treatment of the original illness or injury.



Because people with ARDS are less able to fight lung infections, they may develop bacterial pneumonia during the course of the illness. Antibiotics are given to fight infection. Also, supportive treatment such as intravenous fluid or food may be needed. If other organ systems become involved, measures may be needed to support those organs.



The introduction into standard practice of a recent recommendation to use smaller "tidal volumes" (the volume of each individual breath delivered by the ventilator) has resulted in improved outcomes. Earlier, ventilators were set to deliver 12 ml per kg of body weight. Now only 6 ml per kg of body weight are delivered.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222



TTY: (866) 411-1010



Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:



www.centerwatch.com

References

JOURNAL ARTICLES



Steinbrook R. How best to ventilate? Trial design and patient safety in studies of the acute respiratory distress syndrome. N Eng J Med. 2003;348:1393-1401.



Said SI, Dickman KG. Pathways of inflammation and cell death in the lung: modulation by vasoactive intestinal peptide. Reg Pept. 2000;93:21-29.



Bernard GR. Efficacy and safety of recombinant human activated protein C for severe sepsis. N Eng J Med. 2000;344:699-709.



Tobin MJ. Culmination of an era in research on the actue respiratory distress syndrome. N Engl J Med. 2000;342:1360-61.



Ware LB, Marthay MA. The acute respiratory distress syndrome. N Engl J Med. 2000;342:1334-39.



Hudson LD, et al. Protective ventilation for patients with acute respiratory distress syndrome. N Eng J Med. 1998;338:385-7.



Amato MBP, et al. Effect of a protective-ventilation strategy on mortality in the acute respiratory distress syndrome. N Eng J Med. 1998;338:347-54.



Weg JG, et al. The relation of pneumothorax and other air leaks to mortality in the acute respiratory distress syndrome. N Eng J Med. 1998;338:341-6.



Beal AL, Cerra FB. Multiple organ failure syndrome in the 1990s: systemic inflammatory response and organ dysfunction. JAMA 1994;271:226-33.



Villar J, Slutsky AS. The incidence of the adult respiratory distress syndrome. Am Rev Respir Dis 1989;140:814-16.

Supporting Organizations

American Lung Association

55 W. Wacker Drive
Suite 1150
Chicago, IL 60601
USA
Tel: 1-800-548-8252
Email: info@lung.org
Website: //www.lungusa.org

Genetic and Rare Diseases (GARD) Information Center

PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)251-4925
Fax: (301)251-4911
Tel: (888)205-2311
Website: //rarediseases.info.nih.gov/GARD/

NIH/National Heart, Lung and Blood Institute

P.O. Box 30105
Bethesda, MD 20892-0105
Tel: (301)592-8573
Fax: (301)251-1223
Email: nhlbiinfo@rover.nhlbi.nih.gov
Website: //www.nhlbi.nih.gov/

Nathaniel Adamczyk Foundation

715 Greythorne Road
Wynnewood, PA 19096
Tel: 610-582-1646
Fax: 610-667-8606
Email: iris@nafoundation.net
Website: //www.nafoundation.net

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.