Coccidioidmycosis
The 5 Minute Pediatric Consult
Molly (Martha) W. Stevens
DEFINITION
Coccidioidomycosis is infection by the dimorphic fungus Coccidioides immitis.
PATHOPHYSIOLOGY
- Inhalation of arthrospores from disturbed, arid soil is major route of infection.
- Sixty percent of acute infections are subclinical (asymptomatic).
- Most patients have infection limited to a localized area of lung and hilar nodes after mounting an intense inflammatory response with granuloma formation.
- Dissemination occurs in a minority of patients (<1% cases) via lymphatic or hematologic spread, causing widespread granulomatous lesions.
- The course of illness is highly variable and dependent on host immune response and amount of exposure. HIV-infected patients and other patients with immunosuppression due to T-lymphocyte dysfunction (lymphoma, organ transplantation) are particularly susceptible to severe forms of pulmonary and extrapulmonary coccidioidomycosis.
EPIDEMIOLOGY
- Coccidioides immitis is found in abundance in soil in the southwestern United States (west Texas, New Mexico, Arizona, California), northern Mexico, and parts of South and Central America. Estimations (by skin testing) of infection in up to one-third of the population in endemic areas.
- There is no person-to-person spread.
- The average incubation period is 10 to 16 days, with a range of approximately 6 to 30 days.
- Primary infection is most commonly seen in the summer and fall months.
- Susceptibility is unaffected by age, race, or sex, but dissemination rates after infection vary considerably (see Complications section below).
COMPLICATIONS
- Localized complications of primary pulmonary infection are infrequent and include pleural effusions and pericarditis.
- Factors increasing the susceptibility of a patient for disseminated coccidioidomycosis include immunosuppression; infancy; Filipino, African-American, or Hispanic populations; and patients in whom infection is characterized by mediastinal adenopathy or fever greater than 1 month in duration.
- Hydrocephalus is common after spread of infection to the CNS.
PROGNOSIS
- Primary infection of the lungs is usually self-limited, with a course of illness lasting 1 to 3 weeks; complications (see above) may prolong the course.
- Dissemination is infrequent (see above for risk factors). Morbidity and mortality have improved with use of amphotericin B, but immunocompromised patients with poor T-cell function still have a poor prognosis after the development of disseminated infection.
- Dissemination of disease to the CNS can cause a rapidly fatal meningitis if not recognized and treated.
ASSOCIATED DISEASES
- Primary pulmonary coccidioidomycosis is usually a nonapparent, asymptomatic infection.
- Symptomatic pulmonary infection is characterized by cough, fever, and chest pain and may be accompanied by rash or arthralgias. Chronic pulmonary lesions are rare in children.
- Skin is a second, infrequent, primary site of infection, which occurs via direct inoculation (trauma).
- Progressive systemic disease can spread from the lungs to involve lymph nodes, bones, joints, abdominal organs, skin, and CNS.
- Limited dissemination (spread to one or more sites) is common in children.
- Disseminated coccidioidomycosis in children resembles progressive tuberculosis, with spread weeks to months after primary infection. Children receiving chemotherapy and those with HIV may reactivate previous infection.
- Disseminated disease is less common in children than in adults. It is characterized by persistent fevers, toxicity, and development of granulomatous lesions outside the chest. Common extrapulmonary sites of infection include bone (fingers, toes, ribs, vertebrae), skin, and CSF.
Tuberculosis (lung or CSF)
- Mycoplasma
- Other pulmonary fungal diseases, such as histoplasmosis
- Influenza
- Other viral or bacterial infections that present as bronchopneumonia
HISTORY
- History of travel or residence in endemic areas
- Initial symptoms: fever, dry or productive cough, and pleuritic chest pain, which may be accompanied by myalgia, arthralgia, chills, night sweats, headache, and anorexia
- Hemoptysis is rare in children, but is reported in up to 15% of symptomatic adults.
- The severity of symptomatic illness is widely variable in acute infection: from mild flulike illness lasting a few days, to severe lower tract respiratory disease with lobar pneumonia, pleural effusions, and, rarely, pericarditis.
- Symptomatic children often have an erythematous maculopapular rash, usually limited to the lower trunk and thighs, but occasionally it is more diffuse and resembles measles.
- Erythema nodosum may occur later in the course of the infection, especially in children in the San Joaquin Valley.
- Young children may have a clinical finding of stridor after infection of subglottic tissues.
- Hematologic findings include elevated ESR, leukocytosis, and, often, eosinophilia.
- Coccidioidin or spherulin skin test positivity (delayed type hypersensitivity), along with characteristic signs and symptoms of coccidiodomycosis, is strongly suggestive of the infection.
- Skin tests can be positive 10 to 21 days after infection, but are frequently negative in progressive or disseminated disease.
- Culture of organism is possible in qualified laboratories.
- Visualization of large spherules is possible in stained specimens of sputum, tracheal aspirates, CSF, urine, or tissue biopsy.
- IgM agglutination tests are rapid and sensitive but not specific. Tube precipitin and immunodiffusion can also evaluate IgM response.
- IgG is detected by immunodiffusion or complement fixation from serum or CSF: High and persistent complement fixation titers are seen in severe disease; decreasing titers suggest resolution of infection.
- No characteristic CXR findings. Most common: well-circumscribed nodules, pulmonary infiltrates, pleural effusions, hilar adenopathy, and cavitations
- Uncomplicated or minor disease is self-limited and should not be treated with antifungal therapy (>95% of cases).
- Surgical debridement is used for localized and persistent lesions in bone and lung.
- Amphotericin B is recommended for severe progressive disease, for disease in immunocompromised patients (e.g., those with neoplasm or AIDS), and for patients with disseminated or CNS infection.
- Intrathecal amphotericin B is useful in CNS infections.
- High-dose ketoconazole with intraventricular or intrathecal miconazole has been used alternatively for CNS coccidioidomycosis.
PREVENTION
Infection Control
- No special isolation or precautions for the hospitalized patient
- Contaminated dressings from skin lesions should be handled and discarded with care.
- Preventive efforts are aimed at dust control and trials to eliminate organisms from soil.
- Avoidance of field activities or travel in highly endemic areas is recommended for children with a negative skin test.
PITFALLS
- Skin test in disseminated disease may be negative.
- Diagnosis in nonendemic areas can easily be missed due to low clinical suspicion or missed travel history. Clinicians in endemic areas should maintain a high level of clinical suspicion.
ICD-9-CM 114.9
American Academy of Pediatrics. Coccidioidomycosis. In: Peter G, ed. 1997 red book: report of the Committee on Infectious Diseases, 24th ed. Elk Grove Village, IL: American Academy of Pediatrics, 1997:181183.
Libke RD, Granoff DM. Coccidioidomycosis. In: Feigin RD, Cherry JD, eds. Textbook of pediatric infectious diseases, 3rd ed. Philadelphia: WB Saunders, 1992:19161925.
Stevens DA. Coccidioidomycosis. N Engl J Med 1995;332(16):10771082.
Stevens DA. Coccidioides immitis. In: Mandell GL, et al., eds. Principles and practice of infectious diseases, 3rd ed. New York: Churchill Livingstone, 1990:20082016.
Copyright © 2000 Lippincott Williams & Wilkins
M. William Schwartz, Louis M. Bell, Jr., Peter M. Bingham, Esther K. Chung, David F. Friedman and Andrew E. Mulberg, The 5 Minute Pediatric Consult