Hypersensitivity Pneumonitis
Hypersensitivity Pneumonitis
Definition
Hypersensitivity pneumonitis refers to an inflammation of the lungs caused by repeated breathing in of a foreign substance, such an organic dust, a fungus, or a mold. The body's immune system reacts to these substances, called antigens, by forming antibodies, molecules that attack the invading antigen and try to destroy it. The combination of antigen and antibody produces acute inflammation, or pneumonitis (a hypersensitivity reaction), which later can develop into chronic lung disease that impairs the lungs' ability to take oxygen from the air and eliminate carbon dioxide.
Description
Hypersensitivity pneumonitis (HP) is sometimes called "allergic alveolitis." "Allergic" refers to the antigen-antibody reaction, and "alveolitis" means an inflammation of the tiny air sacs in the lungs where oxygen and CO2 are exchanged, the alveoli. It also is known as "extrinsic" allergic alveolitis, meaning that the antigen that sets up the allergic reaction (also called an allergen) comes from the outside. Most of the antigens that cause this disease come from plant or animal proteins or microorganisms, and many of those affected are exposed either at work or in the course of some hobby or other activity. The first known type of HP, farmer's lung, is caused by antigens from tiny microorganisms living on moldy hay. An example of disease connected with a hobby is pigeon breeder's lung, caused by inhaling protein material from bird droppings or feathers. After a time, very little of the allergenic material is needed to set off a reaction in the lungs.
Roughly one in every 10,000 persons develops some form of HP. A mysterious aspect of this condition is that, even though many persons may be exposed to a particular antigen, only a small number of them will develop the disease. Genetic differences may determine who becomes ill; this remains unclear. Probably between 5% and 15% of all persons who are regularly exposed to organic materials develop HP. Most of those who do get it do not smoke (smoking may create the type of cells that take up antigens and neutralize them). The amount of antigen is an important factor in whether HP will develop and what form it will take. Sudden heavy exposure can produce symptoms in a matter of hours, whereas mild but frequent exposures tend to produce a long-lasting, "smoldering" illness. HP may be more likely to develop in persons exposed to polluted air or industrial fumes.
Typical changes occur in the lungs of persons with HP. In the acute stage, large numbers of inflammatory cells are found throughout the lungs and the air sacs may be filled by a thick fluid mixed with these cells. In the subacute stage, disease extends into the small breathing tubes, or bronchioles, and the inflammatory cells collect into tiny granules called granulomas. Finally, in the chronic stage of HP, the previously inflamed parts of the lungs become scarred and unable to function, as in pulmonary fibrosis.
Causes and symptoms
A number of different types of HP are known, since a wide range of allergens may produce an allergic reaction in the lungs. Many of them produce similar symptoms and abnormal physical findings, but some have their own typical features. Some of the more common forms are:
- Farmer's lung. Can affect any farmer who works with wet hay or other moldy dust. Small farmers who have to directly thresh and handle their hay are most at risk, as are those living in cold and humid areas where damp weather is common.
- Pigeon breeder's lung. Also called "bird fancier's lung," it is second to farmer's lung as the best known type of HP. A substance has been found in pigeon droppings that may cause the allergic reaction, but there may be more than one such substance. Besides pigeons, the disorder may follow exposure to ducks, geese, pheasants, and even canaries. Parakeets produce an especially severe form of disease. Most patients are middle-aged women, who usually care for birds either at home or on bird breeding farms.
- Bagassosis. Caused by bagasse, a substance produced when juice is extracted from sugar cane and is used in making paper and explosives. A fungus is probably responsible. Young and middle-aged men who work in the sugar industry are at risk.
- Byssinosis. A similar condition affecting workers who inhale dust from cotton, flax, or hemp.
- Humidifier lung. An acute form of HP caused by inhaling actinomycyetes, the same organisms that cause farmer's lung, which grow in contaminated humidifier vents, air conditioners, heating systems, and even saunas.
- Other antigens. HP has been seen in persons working with detergents, silicone, mushrooms, cheese, wood dust, maple bark, coffee, and furs.
In the acute stage, patients with HP begin coughing, develop fever, and note tightness in the chest as well as extreme tiredness and aching, four to eight hours after the most recent exposure. Most patients are well aware of the connection between their work (or an activity) and their symptoms. After a time, patients may have trouble breathing. They also may lose their appetite, lose weight, and generally feel ill. Finally, in the chronic stage, the patient will have increasing trouble breathing and may sometimes wheeze. With advanced disease, the skin may appear bluish (because too little oxygen is getting into the blood). When the physician listens to the patient's chest with a stethoscope, there may be crackling sounds or loud wheezing. In the late stages, club-shaped fingertips are a sign that the patient has not been getting enough oxygen for an extended period of time.
Diagnosis
No single test can make a definite diagnosis of HP. The key is to relate some specific exposure or activity to episodes of symptoms. The chest x ray may be normal in the acute stage, but later may show a hazy appearance that looks like "ground glass." There may be linear or rounded shadows in the central parts of the lungs. Studies of lung function in the acute stage typically show abnormally small lung volume. The ability to breathe at a fast rate is impaired. Blood from an artery typically has a low level of oxygen. Later, when the lungs have begun to scar, the airways (breathing tubes) are obstructed and the rate of air flow is reduced.
Some experts believe that skin testing can help diagnose HP and show which particular antigen is causing the symptoms. Small amounts of several suspect antigens are injected just beneath the surface of the skin, usually on the arm or back, and the reactions compared to that caused by injecting a harmless salt solution. Another diagnostic test is to place a thin tube into the airways, inject a small amount of fluid, and draw it back up (bronchoalveolar lavage). A very large number of cells called lymphocytes is typical of HP, and mast cells, which are part of the immune system, may also be seen. Rarely, a tissue sample (biopsy) of lung tissue may be taken through a tube placed in the airways and examined under a microscope. Finally, a patient may be "challenged" by actually inhaling a particular antigen in the form of an aerosol and noting whether lung function suddenly becomes worse. This test is usually not necessary.
Treatment
Treatment of HP requires identifying the offending antigen and avoiding further exposure. Although it may sometimes be necessary for a patient to find a totally different type of work, often it is possible to simply perform different duties or switch to a work site where exposure is minimal. In some cases, (like pigeon breeder's lung), wearing a mask can prevent exposure. If acute symptoms are severe, the patient may be treated with a steroid hormone for two to six weeks. This often suppresses the inflammatory response and allows the lungs a chance to recover. In the chronic stage, steroid treatment can delay further damage to the lungs and help preserve their function.
Prognosis
In general, most of the symptoms of HP disappear when the patient is no longer exposed to the causative allergen. The actual chances of complete recovery depend in part on what form of HP is present. Older patients and those exposed repeatedly for long periods after initially developing symptoms tend to have a poorer long-term outlook. The worst outcome is that long repeated episodes of exposure will cause chronic lung inflammation, scar the lungs, and permanently make then unable to properly provide oxygen to the blood. Rarely, a patient will become permanently disabled.
Prevention
It is often not possible to prevent initial episodes of HP, because there is no way of predicting which individuals (such as farmers) will have an allergic reaction to a particular allergen. Once the connection is made between a type of exposure and definite hypersensitivity symptoms, prevention of further episodes is simple as long as further exposure can be avoided.
Exactly how to avoid exposure depends on a person'swork or activities and what he or she is reacting to. People with farmer's lung can dry hay thoroughly before storing it. For pigeon breeder's lung (and many other types of HP), a mask can be worn. In many industrial settings, it is possible to take precautions that will limit the amount of allergen that workers will inhale. If it is not possible to avoid exposure altogether, exposure can be timed and strictly minimized.
Resources
ORGANIZATIONS
American Lung Association. 1740 Broadway, New York, NY 10019. (800) 586-4872. 〈http://www.lungusa.org〉.
Asthma and Allergy Foundation of America. 1233 20th Street, NW, Suite 402, Washington, DC 20036. (800) 727-8462. 〈http://www.aafa.org〉.
KEY TERMS
Allergen— An outside substance, such as dust or a mold, that, when inhaled, sets off an allregic (hypersensitivity) reaction in the lungs.
Fibrosis— A result of long-standing inflammatory disease in which normal tissue is replaced by scar tissue that is functionally useless.
Granuloma— A collection of inflammatory cells forming a microscopic lesion, many of which are scattered throughout the lung tissue in patients who have had numerous acute episodes of HP.
Hypersensitivity— After the body's immune system attacks an outside invader (such as organic dust or a fungus) many times, exposure to even a tiny amount of this allergen can provoke a strong inflammatory response.
Pneumonitis— Inflammation of the lung tissues.
Steroid— A natural body substance may be given orally or by injection, and serves to dampen or even halt inflammation anywhere in the body, including the lungs.
Hypersensitivity Pneumonitis
HYPERSENSITIVITY PNEUMONITIS
Hypersensitivity pneumonitis, also called extrinsic allergic alveolitis, is an inflammatory condition of the lung caused by the inhalation of small organic or, less commonly, inorganic particles in susceptible individuals. Some thirty different varieties of the condition have been described, the most common of which is known as "farmer's lung." Hypersensitivity pneumonitis represents an important category of occupational lung disease. The inhaled particles are usually fungal spores or bacteria that are found in moldy or rotting material, including hay, compost, bark, wood dust, and grains. Hypersensitivity pneumonitis affects the tissue of the lung where gas exchange occurs and is distinguished from bronchial asthma, which affects the lungs' airways. The condition may be acute or chronic. Treatment consists of avoiding exposure and, in severe cases, the administration of corticosteroid drugs.
John L. Stauffer
(see also: Asthma; Occupational Lung Disease )
Bibliography
Murphy, D. M. F.; Morgan, W. K. C.; and Seaton, A. (1995). "Hypersensitivity Pneumonitis." In Occupational Lung Diseases, 3rd edition, eds. W. K. C. Morgan and A. Seaton. Philadelphia, PA: W. B. Saunders.