The aetiological agents of coccidioidomycosis, Coccidioides immitis and C posadasii, inhabit ecological niches found only in the western hemisphere (areas of low precipitation), primarily in the southwestern deserts of the US (California, Arizona, New Mexico, and western Texas) and northern deserts of Mexico.[3]Sunenshine RH, Anderson S, Erhart L, et al. Public health surveillance for coccidioidomycosis in Arizona. Ann NY Acad Sci. 2007 Sep;1111:96-102.
http://www.ncbi.nlm.nih.gov/pubmed/17513465?tool=bestpractice.com
[4]Thompson GR 3rd, Le T, Chindamporn A, et al. Global guideline for the diagnosis and management of the endemic mycoses: an initiative of the European Confederation of Medical Mycology in cooperation with the International Society for Human and Animal Mycology. Lancet Infect Dis. 2021 Dec;21(12):e364-74.
http://www.ncbi.nlm.nih.gov/pubmed/34364529?tool=bestpractice.com
They are also endemic in limited areas of Utah, Nevada, and eastern Washington state, as well as Central and South America. Coccidioidomycosis is an infection identified primarily in people residing within these areas; cases outside the endemic areas may be identified in visitors returning to non-endemic areas. An estimated 150,000 cases of coccidioidomycosis occur in the US annually, although many of these are misdiagnosed and unreported.[5]Galgiani JN, Ampel NM, Blair JE, et al. 2016 Infectious Diseases Society of America (IDSA) clinical practice guideline for the treatment of coccidioidomycosis. Clin Infect Dis. 2016 Sep 15;63(6):e112-46.
http://www.ncbi.nlm.nih.gov/pubmed/27470238?tool=bestpractice.com
In 2019, approximately 20,000 cases were reported to the Centers for Disease Control and Prevention (CDC), most of which were among people living in Arizona or California.[6]Centers for Disease Control and Prevention. Valley fever (coccidioidomycosis) statistics. Apr 2022 [internet publication].
https://www.cdc.gov/fungal/diseases/coccidioidomycosis/statistics.html
One retrospective analysis of data from 2007 to 2016 found that coccidioidomycosis was diagnosed in significant numbers outside the historical geographic distribution, with 69% of the US states above the clinically relevant threshold for coccidioidomycosis in at least one county.[7]Mazi PB, Sahrmann JM, Olsen MA, et al. The geographic distribution of dimorphic mycoses in the United States for the modern era. Clin Infect Dis. 2023 Apr 3;76(7):1295-301.
http://www.ncbi.nlm.nih.gov/pubmed/36366776?tool=bestpractice.com
The number of reported cases in the US varies year-to-year, but continues to increase yearly.[4]Thompson GR 3rd, Le T, Chindamporn A, et al. Global guideline for the diagnosis and management of the endemic mycoses: an initiative of the European Confederation of Medical Mycology in cooperation with the International Society for Human and Animal Mycology. Lancet Infect Dis. 2021 Dec;21(12):e364-74.
http://www.ncbi.nlm.nih.gov/pubmed/34364529?tool=bestpractice.com
This variation is not fully understood, but suggested reasons include changes to: the number of people exposed to Coccidioides (owing to travel or relocation); environmental factors affecting fungal growth and circulation (such as temperature and rainfall); and the way cases are detected and reported.[6]Centers for Disease Control and Prevention. Valley fever (coccidioidomycosis) statistics. Apr 2022 [internet publication].
https://www.cdc.gov/fungal/diseases/coccidioidomycosis/statistics.html
Coccidioidomycosis is acquired when airborne fungal arthroconidia (spores) are inhaled; therefore, occupational (e.g., construction, digging) or recreational (e.g., gardening) activities that increase the likelihood of dust inhalation also increase the likelihood of infection. Outbreaks have been associated with activities such as archaeological excavation, construction, and military training exercises.[8]Centers for Disease Control and Prevention. CDC Yellow Book 2024: health information for international travel. Section 5: travel-associated infections and diseases - coccidioidomycosis/valley fever. May 2023 [internet publication].
https://wwwnc.cdc.gov/travel/yellowbook/2024/infections-diseases/coccidioidomycosis-valley-fever
They have also been reported in people who fight wildfires.[9]Laws RL, Jain S, Cooksey GS, et al. Coccidioidomycosis outbreak among inmate wildland firefighters: California, 2017. Am J Ind Med. 2021 Apr;64(4):266-73.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8935635
http://www.ncbi.nlm.nih.gov/pubmed/33484179?tool=bestpractice.com
Climatic variables‚ such as precipitation, drought, temperature, wind speed, and dust‚ also affect the amount of airborne spores.[3]Sunenshine RH, Anderson S, Erhart L, et al. Public health surveillance for coccidioidomycosis in Arizona. Ann NY Acad Sci. 2007 Sep;1111:96-102.
http://www.ncbi.nlm.nih.gov/pubmed/17513465?tool=bestpractice.com
Males are more likely to acquire infection; this may be due to increased likelihood of occupational or recreational dust exposure.[10]Centers for Disease Control and Prevention. CDC surveillance summaries: surveillance for coccidioidomycosis, histoplasmosis, and blastomycosis - United States, 2019. Aug 2022 [internet publication].
https://www.cdc.gov/mmwr/volumes/71/ss/ss7107a1.htm?s_cid=ss7107a1_w
Race is a strong risk factor for developing severe and disseminated infection but not for acquisition of infection.[3]Sunenshine RH, Anderson S, Erhart L, et al. Public health surveillance for coccidioidomycosis in Arizona. Ann NY Acad Sci. 2007 Sep;1111:96-102.
http://www.ncbi.nlm.nih.gov/pubmed/17513465?tool=bestpractice.com
African-Americans and Filipinos have the highest risk for dissemination, approximately 10 to 175 times greater than other races.[3]Sunenshine RH, Anderson S, Erhart L, et al. Public health surveillance for coccidioidomycosis in Arizona. Ann NY Acad Sci. 2007 Sep;1111:96-102.
http://www.ncbi.nlm.nih.gov/pubmed/17513465?tool=bestpractice.com
[11]Ruddy BE, Mayer AP, Ko MG, et al. Coccidioidomycosis in African Americans. Mayo Clin Proc. 2011 Jan;86(1):63-9.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3012635
http://www.ncbi.nlm.nih.gov/pubmed/21193657?tool=bestpractice.com
The evidence is poor on whether there exists an increased risk of dissemination for Asians, Hispanics, and American Indians. It is likely that race per se is not the predisposing risk but rather the associated genetic makeup that dictates the immune response. People with immunosuppression, such as organ transplant recipients, people with HIV, or pregnant people, are also at increased risk for developing disseminated disease.[3]Sunenshine RH, Anderson S, Erhart L, et al. Public health surveillance for coccidioidomycosis in Arizona. Ann NY Acad Sci. 2007 Sep;1111:96-102.
http://www.ncbi.nlm.nih.gov/pubmed/17513465?tool=bestpractice.com
[8]Centers for Disease Control and Prevention. CDC Yellow Book 2024: health information for international travel. Section 5: travel-associated infections and diseases - coccidioidomycosis/valley fever. May 2023 [internet publication].
https://wwwnc.cdc.gov/travel/yellowbook/2024/infections-diseases/coccidioidomycosis-valley-fever
[11]Ruddy BE, Mayer AP, Ko MG, et al. Coccidioidomycosis in African Americans. Mayo Clin Proc. 2011 Jan;86(1):63-9.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3012635
http://www.ncbi.nlm.nih.gov/pubmed/21193657?tool=bestpractice.com
[12]Crum NF, Ballon-Landa G. Coccidioidomycosis in pregnancy: case report and review of the literature. Am J Med. 2006 Nov;119(11):993.e11-7.
http://www.ncbi.nlm.nih.gov/pubmed/17071170?tool=bestpractice.com
[13]Spinello IM, Johnson RH, Baqi S. Coccidioidomycosis in pregnancy: a review. Ann NY Acad Sci. 2007 Sep;1111:358-64.
http://www.ncbi.nlm.nih.gov/pubmed/17363444?tool=bestpractice.com
People aged ≥65 years, people with diabetes, people who smoke, and people with high inoculum exposure are at increased risk of developing severe pulmonary complications.[8]Centers for Disease Control and Prevention. CDC Yellow Book 2024: health information for international travel. Section 5: travel-associated infections and diseases - coccidioidomycosis/valley fever. May 2023 [internet publication].
https://wwwnc.cdc.gov/travel/yellowbook/2024/infections-diseases/coccidioidomycosis-valley-fever