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Manifestations and treatment of extrapulmonary coccidioidomycosis

Carol A Kauffman, MD
Section Editor
Daniel J Sexton, MD
Deputy Editor
Jennifer Mitty, MD, MPH


Coccidioidomycosis is caused by the dimorphic fungi, Coccidioides immitis, or Coccidioides posadasii. These organisms are endemic to certain arid regions of the western hemisphere. Infection is virtually always acquired by inhalation, and primary infection frequently goes unrecognized [1,2]. Dissemination of infection beyond the lungs can occur, but it is difficult to estimate the incidence of overt disseminated disease. (See "Primary coccidioidal infection", section on 'Epidemiology' and "Primary coccidioidal infection", section on 'Microbiology'.)

The manifestations and treatment of extrapulmonary coccidioidomycosis other than involvement of the central nervous system will be reviewed here. Primary infection, laboratory diagnosis, pulmonary sequelae, coccidioidal meningitis, and infection in immunocompromised hosts are discussed separately. (See "Primary coccidioidal infection" and "Coccidioidomycosis: Laboratory diagnosis and screening" and "Management of pulmonary sequelae and complications of coccidioidomycosis" and "Coccidioidal meningitis" and "Coccidioidomycosis in compromised hosts".)


Coccidioidomycosis occasionally spreads from the initial pulmonary lesion to other parts of the body. Past estimates of this risk vary from approximately 4.7 percent of recognized infections [3] to 0.2 percent of all respiratory exposures to Coccidioides spp [4]. This substantial range in estimates is probably due to the under-recognition of mild infections [5]. Patient groups at risk for extrapulmonary infection include those who are of African or Filipino ancestry and those who are immunosuppressed.

The Arizona Department of Health Services sought to review medical charts of every 10th person newly reported with coccidioidomycosis from January 2007 to February 2008 and were 65.7 percent successful for a total of 324 cases [6]. Of these, 26 (8.0 percent) had clinical or laboratory evidence of infection beyond the lungs. If this sample was representative of all reported infections that year (approximately 5000), then, by extrapolation, there were a total of 400 newly diagnosed disseminated infections in Arizona.


When disseminated infection occurs, it usually becomes evident within weeks after the initial exposure. Thus, it is common for dissemination to be clinically apparent at the time infection is first diagnosed. In patients who do not receive treatment for early coccidioidal infections, new onset of disseminated disease after two years is very uncommon in the absence of immunosuppression. However, in studies of patients who have received early antifungal treatment, there are reports of delayed recognition of dissemination until up to two years after treatment is stopped [7]. In the author's practice, a patient's vertebral coccidioidal osteomyelitis first became evident seven years after a brief course of fluconazole was given for early coccidioidal pneumonia.

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Literature review current through: Oct 2017. | This topic last updated: Jul 05, 2017.
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