Coccidioidomycosis - chronic pulmonary

Definition

Chronic pulmonary coccidioidomycosis is a lung infection caused by breathing in the fungus Coccidioides.

Causes, incidence, and risk factors

The infection is caused by breathing in the spores of a fungus found in the soil in desert regions of the southwestern U.S., Mexico, and Central and South America. The disease can have an acute, chronic, or disseminated form.

People get the chronic form months or years after having an infection that often is not diagnosed. Lung abscesses can form and may break open, causing pus in the lung cavity (empyema) or an abnormal connection between an air passage and the lung cavity (bronchopleural fistula). Scarring (fibrosis) and cavities can form in the upper lungs as chronic pulmonary coccidioidomycosis slowly gets worse over a period of months or years.

Most pulmonary coccidioidomycosis infections do not become chronic.

The following increase the risk for the chronic or disseminated forms of the disease:

• African or Phillippine descent
• Weak immune system
• Pregnancy
• Pre-existing heart or lung disease
• Diabetes

Symptoms

• Chronic cough
• Blood-tinged sputum
• Loss of appetite
• Weight loss
• Fever
• Shortness of breath

Other symptoms of this disease include:

• Wheezing
• Excess sweating
• Joint stiffness
• Chest pain
• Headache

Signs and tests

• Sputum KOH test
• Sputum fungal culture
• Coccidioides complement fixation titer to measure antibodies to the fungus in the blood
• CBC with differential
• Chest x-ray
• Chest CT scan
• Coccidioidin skin test
• Bronchoscopy (possibly with transbronchial biopsy)
• Open lung biopsy
• Mediastinoscopy
• Spinal tap (lumbar puncture) to rule out meningitis

Treatment

Antifungal medications are used to treat this disease. Treating any immune system problem (such as AIDS) and limiting the use of medications that suppress the immune system (such as steroids or chemotherapy) can help the body fight the coccidioidomycosis infection. Sometimes surgery is needed to remove the infected part of the lung.

Expectations (prognosis)

With treatment the outcome is usually good, although the disease can come back. Some patients, such as those with immune system problems (from AIDS or drugs that suppress the immune system) may need to keep taking antifungal medication to prevent a relapse.

Complications

• Pleural effusion
• Relapse of infection
• Disseminated coccidioidomycosis
• Coccidioidomycosis meningitis
• Arthritis
• Rash
• Empyema
• Pneumonia
• Bronchopleural fistula
• Lung scarring (fibrosis)
• Lung cavities

Calling your health care provider

Call for an appointment with your health care provider if:

• Coccidioidomycosis symptoms get worse or do not improve with treatment
• You have new symptoms

Prevention

Because coccidioidomycosis usually causes no symptoms -- and when symptoms do occur they usually get better quickly on their own -- there is no need to avoid infection. People with immune problems (such as AIDS patients and those on drugs that suppress the immune system) can avoid travel to areas where this fungus is found, if they want to reduce their risk of getting this rare, chronic disorder.

References

Galgiani J. Coccidioides species. In: Mandell GL, Bennett JE, Dolin R. Principles and Practice of Infectious Diseases. 6th ed. Philadelphia, Pa: Churchill Livingstone; 2005:chap 264.

Galgiani JN. Coccidioidomycosis. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 354.