Severe combined immunodeficiency

Definition

Pneumocystis pneumonia is a lung infection that occurs primarily in people with weakened immune systems-- especially people who are HIV-positive. The disease agent is an organism whose biological classification is still uncertain. Pneumocystis carinii was originally thought to be a one- celled organism (a protozoan), but more recent research suggests that it is a fungus. Although its life cycle is known to have three stages, its method of reproduction is not yet completely understood. The complete name of the disease is Pneumocystis carinii pneumonia, often shortened to PCP. PCP is also sometimes called pneumocystosis.

Description

Pneumonia as a general term refers to a severe lung inflammation. In pneumocystis pneumonia, this inflammation is caused by the growth of Pneumocystis carinii, a fungus-like organism that is widespread in the environment. PCP is ordinarily a rare disease, affecting only people with weakened immune systems. Many of these people are patients receiving drugs for organ transplants or cancer treatment. With the rising incidence of AIDS, however, PCP has become primarily associated with AIDS patients. In fact, as many as 75% of AIDS patients have developed PCP. It has also been the leading cause of death in AIDS patients.

Transmission

The organism that causes PCP is widely distributed in nature and is transmitted through the air. When the organism is inhaled, it enters the upper respiratory tract and infects the tiny air sacs at the ends of the smaller air tubes (bronchioles) in the lungs. These tiny air sacs are called alveoli. Under a microscope, alveoli look like groups of hollow spheres resembling grape clusters. The exchange of oxygen with the blood takes place in the alveoli. It appears that P. carinii lives in the fluid in the lining of the alveoli.

Person-to-person infection does not appear to be very common; however, clusters of PCP outbreaks in hospitals and groups of immunocompromised people indicate that patients with active PCP should not be exposed to others with weakened immune systems. It is thought that many people actually acquire mild Pneumocystis carinii infections from time to time, but are protected by their immune systems from developing a full-blown case of the disease.

Causes & symptoms

Causes

High-risk groups

Some patients are at greater risk of developing PCP. These high-risk groups include:

• Premature infants
• Patients with immunodeficiency diseases, including severe combined immunodeficiency disease (SCID) and acquired immunodeficiency syndrome (AIDS).
• Patients receiving immunosuppressive drugs, especially cortisone-like drugs (corticosteroids)
• Patients suffering from protein malnutrition.

AIDS is currently the most common risk factor for PCP in the United States. PCP is, however, also found in countries with widespread hunger and poor hygiene.

Symptoms

The incubation period of PCP is not definitely known, but is thought to be between four and eight weeks. The major symptoms include shortness of breath, fever, and a nonproductive cough. Less common symptoms include production of sputum, blood in the sputum, difficulty breathing, and chest pain. Most patients will have symptoms for one to two weeks before seeing a physician. Occasionally, the disease will spread outside of the lung to other organs, including the lymph nodes, spleen, liver, or bone marrow.

Diagnosis

The diagnosis of PCP begins with a thorough physical examination and blood tests. Although imaging studies are helpful in identifying abnormal areas in the lungs, the diagnosis of PCP must be confirmed by microscopic identification of the organism in the lung. Samples may be taken from the patient's sputum, or may be obtained via bronchoscopy or lung biopsy. Because of the severity of the disease, many physicians will proceed to treat patients with symptoms of pneumocystis pneumonia if they belong to a high-risk group, without the formality of an actual diagnosis. The severity of PCP can be measured by x-ray studies and by determining the amount of oxygen and carbon dioxide present in the patient's blood.

Treatment

Treatment for PCP involves the use of antibiotics. These include trimethoprim-sulfamethoxazole (TMP-SMX, Bactrim, Septra) and pentamidine isoethionate (Nebupent, Pentam 300). Both of these anti-microbial drugs are equally effective. AIDS patients are typically treated for 21 days, whereas non-AIDS patients are treated for 14 days. TMP-SMX may be highly toxic in AIDS patients, causing severe side effects that include fever, rash, decreased numbers of white blood cells and platelets, and hepatitis. Pentamidine also causes side effects in immunocompromised patients. These side effects include decreased blood pressure, irregular heart beats, the accumulation of nitrogenous waste products in the blood (azotemia), and electrolyte imbalances. Pentamidine can be given in aerosol form to minimize side effects. Alternative drugs can be used for patients experiencing these side effects.

Prognosis

If left untreated, PCP will cause breathing difficulties that will eventually cause death. The prognosis for this disease depends on the amount of damage to the patient's lungs prior to treatment. Prognosis is usually better at a facility that specializes in caring for AIDS patients. Antibiotic treatment of PCP is about 80% effective.

Prevention

Medications

For patients at serious risk for PCP infection, low doses of TMP-SMX, given daily or three times a week, are effective in preventing PCP. The drug is, however, highly toxic. Researchers are currently evaluating the effectiveness and toxicity of aerosol pentamidine and dapsone in preventing PCP.

Lifestyle modifications

Patients who have previously had PCP often experience a recurrence. Healthy lifestyle choices, including exercising, eating well, and giving up smoking may keep the disease at bay.

Key Terms

Alveoli

Small, hollow air sacs found in the lungs at the end of the smaller airways (bronchioles). Air exchange occurs in the alveoli.

Azotemia

The presence of excess nitrogenous wastes in the blood.

Biopsy

A procedure in which a piece of tissue is obtained for microscopic study.

Bronchoscopy

A procedure that uses a fiber-optic scope to view the airways in the lung.

Fungus

A single-celled form of plant life that lives on organic material, including human tissues.

Pentamidine isoethionate

An antibiotic used to treat and prevent PCP.

Pneumocystosis

Another name for active PCP infection.

Protozoan

A microorganism belonging to the Protista, which includes the simplest one-celled organisms.

Sputum

A substance obtained from the lungs and bronchial tubes by clearing the throat or coughing. Sputum can be tested for evidence of PCP infection.

Trimethoprim-sulfamethoxazole (TMP-SMX)

An antibiotic used to treat and prevent PCP.

Further Reading

For Your Information

Books

• Dobkin, Jay. "Pneumocystis Carinii Pneumonia (PCP)." In The Columbia University College of Physicians and Surgeons Complete Home Medical Guide. New York: Crown Publishers, Inc., 1995.
• Walzer, Peter D. "Pneumocystis Carinii." In Principles and Practice of Infectious Diseases, edited by G. L. Mandell, et al. New York: Churchill Livingston, 1995.

  Periodicals

• Korraa, H., and C. Saadeh. "Options in the Management of Pneumonia Caused by Pneumocystis Carinii in Patients with Acquired Immune Deficiency Syndrome and Intolerance to Trimethoprim/Sulfamethoxazole." Southern Medical Journal 89, no. 3 (March 1996): 272-277.

  Organizations

• American Lung Association. (800)LUNG-USA. http://www.lungusa.org.
• National Center for Infectious Disease, Centers for Disease Control and Prevention. 1600 Clifton Rd., NE, Atlanta GA 30333. http://www.cdc.gov.

Gale Encyclopedia of Medicine. Gale Research, 1999.