Information for Health Professionals

Clinical Features: Symptomatic infections (40% of cases) usually present as flu-like illness with fever, cough, headaches, rash, and myalgias. Some patients fail to recover and develop chronic pulmonary infection or widespread disseminated infection (affecting meninges, soft tissues, joints, and bone). Severe disease may develop in anyone, but HIV-infected people or other immunocompromised patients, people receiving corticosteroids, pregnant women in their third trimester, and people of African, Asian, or Filipino descent are at increased risk for developing disseminated disease.

Etiologic Agent: Coccidioides immitis (typically found in California) and Coccidioides posadasii (typically found outside California)

Reservoir: Soil in semiarid areas (primarily in the Lower Sonoran life zone). Endemic in the south-western United States, parts of Mexico and South America.

Incidence: States that are endemic for Coccidioides include California, New Mexico, Arizona, Nevada, Texas, and Utah. A positive skin test was shown in 30% to 60% of people living in areas with endemic disease, indicating prior exposure and antigenic stimulation by the organism. There have been coccidioidomycosis infections reported in non-endemic states such as Colorado, Louisiana, Missouri, Ohio, Michigan, Maryland, New Hampshire, and Delaware.

Sequelae: In people who develop progressive, chronic or disseminated disease, symptoms may persist for months or even longer. Meningitis may lead to permanent neurologic damage. Mortality is high in HIV-infected people with diffuse lung disease.

Diagnosis and Confirmation: Serologic tests for blood or urine to determine exposure and disease severity, fungal cultures for confirmation and speciation, and histology analysis

Treatment: Oral azoles are a popular first line therapy. Although ketoconazole is the only one that has been approved by the FDA for use in coccidioidomycosis, most experts prefer either fluconazole or itraconazole. Some initial studies have indicated that azoles should be used at a minimum dose of 400mg daily and that relapses are frequent once therapy has been discontinued. Fluconazole has shown to be the most effective for treating coccidioidal meningitis. For the management of severely ill patients, some clinicians still turn to intravenous amphotericin B. However, amphotericin B intravenously is ineffective for coccidioidal meningitis and must be administered intrathecally.

Transmission: Inhalation of airborne arthroconidia after disturbance of contaminated soil or natural disasters (e.g., dust storms and earthquakes).

Risk Groups: People in areas with endemic disease who have occupations exposing them to dust (e.g., construction or agricultural workers, and archaeologists). Military trainees participating in training exercises in endemic areas are also at risk. Groups at increased risk for developing disseminated disease are African-Americans and Asians, people of Filipino descent, pregnant women during their third trimester, and immunocompromised patients.

Surveillance: Coccidioidomycosis is a disease under public health surveillance, and is a nationally notifiable disease. It is reportable in the following states: Arizona, California, Delaware, Louisiana, Maryland, Michigan, Minnesota, Missouri, Nevada, New Hampshire, New Mexico, Ohio, Rhode Island, Utah, and Wyoming.

Trends: Incidence appears to have increased in the United States over the past decade, especially in Arizona. There were over 16,000 cases reported in the United States during 2010, 60% of which were from Arizona.

Challenges:

• Developing an effective vaccine (vaccination offers the best prevention measure because infection provides life-long immunity)
• Identifying host factors associated with increased risk for dissemination in select racial groups to target prevention efforts
• Determining the factors associated with increasing incidence in endemic areas
• Determining the influence of climate change

Opportunities:

• Understanding the link between natural disasters and outbreaks of fungal infections
• Encouraging national clinical and epidemiological studies through state reporting and surveillance

Other Resources:

• For more detailed treatment guidelines, please refer to the Infectious Disease Society of America's treatment guidelines [PDF - 7 pages].
• The most recent case definition published by the CDC.
• Summary of Notifiable Diseases [PDF - 104 pages]