Coccidioidomycosis is a disease that exists in two forms: an acute self-limiting disorder involving only the respiratory system, and a progressive form that is chronic and involves almost any part of the body with granulomatous lesions.

The cause is a pathogenic fungus, Coccidioides immitis, found in southwestern USA, the central valleys of California, Arizona, parts of New Mexico, Texas, northern Mexico, and parts of Central America and Argentina. Inhalation of spore-laden dust is responsible for the infection.

The two types of the condition are as follows:

• Acute primary histoplasmosis which is usually asymptomatic, or has mild symptoms of fever, cough, and malaise. Occasionally pneumonia develops.

• Primary coccidioidomycosis which has non-specific respiratory symptoms. The symptoms mimic influenza, or acute bronchitis, e.g., fever, cough, production of sputum, sore throat, and hemoptysis. Rales may be present and, in addition, leukocytosis and eosinophilia are manifested. Some patients develop a syndrome with symptoms of rheumatism, arthritis, conjunctivitis, and erythema nodosum or erythema multiforme. The lesions in the lung resemble tuberculosis (TB) (see Diagnostic Code: 6730 Tuberculosis, pulmonary, chronic, active), or a neoplasm, and residual cavities sometimes fail to close. These cavities may rupture, causing hemoptysis, and require surgery.

• Progressive coccidioidomycosis which may show up months to years after the primary infection. Symptoms include a low-grade fever, anorexia, and weight loss. The pulmonary involvement may cause dyspnea, mucopurulent sputum, increasing cyanosis, and hemoptysis. The extrapulmonary lesions usually involve one or more tissue sites, e.g., bones, joints, skin, brain, abdominal organs, meninges, or skin. These lesions become chronic and recur after treatment.

Tissue specimens or culture of infected body fluids will establish a diagnosis. Complement fixation for antibodies is used frequently. Skin testing is not accurate as everyone in an endemic area is positive.

Untreated the disease is fatal if the disseminated form and meningitis are present. Human immunodeficiency virus (HIV) infected patients have a mortality rate of 70% within one month of diagnosis. Treatment does not alter this course. Primary disease may or may not require treatment. The drugs of choice are amphotericin B and fluconazole for patients with extrapulmonary involvement and acquired immunodeficiency syndrome (AIDS). Those with meningitis will be treated with medication directly into the cerebrospinal fluid.

Treatment for coccidioidomycosis of the meninges must be continued for months and probably for a lifetime. Amphotericin B is highly toxic to the kidneys. Creatinine and BUN levels should be monitored during treatment. If osteomyelitis is present, then surgical removal of the affected bone may be necessary.

• This disease shall be granted service connection although not otherwise established as incurred in or aggravated by service if manifested to a compensable degree within the applicable time limits under 38 CFR 3.307 following service in a period of war or following peacetime service on or after January 1, 1947, provided the rebuttable presumption provisions of 38 CFR 3.307 are also satisfied [38 CFR 3.309 [Disease subject to presumptive service connection], 38 CFR 3.309(a). [chronic disease].

• May be entitled to special monthly compensation where the Veteran has a single service-connected disability rated as 100% with additional service-connected disability or disabilities independently ratable at 60% or more, which are separate and distinct from the 100% service-connected disability and involves different anatomical segments or bodily systems. See  38 CFR 3.350(i)(1) – Special Monthly Compensation (SMC).

• Coccidioidomycosis has an incubation period up to 21 days, and the disseminated phase is ordinarily manifest within six months of the primary phase. However, there are instances of dissemination delayed up to many years after the initial infection which may have been unrecognized. Accordingly, when service connection is under consideration in the absence of record or other evidence of the disease in service, service in southwestern United States where the disease is endemic and absence of prolonged residence in this locality before or after service will be the deciding factor.

• Review special provisions regarding the evaluation of specific respiratory conditions under 38 CFR 4.96(a) - Rating co-existing respiratory conditions.