• Disease Overview
  • Synonyms
  • Signs & Symptoms
  • Causes
  • Affected Populations
  • Disorders with Similar Symptoms
  • Diagnosis
  • Standard Therapies
  • Clinical Trials and Studies
  • References
  • Programs & Resources
  • Complete Report

Cryptococcosis

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Last updated: July 23, 2007
Years published: 1987, 1989, 1997, 1998, 2006, 2007


Disease Overview

Cryptococcosis is caused by a fungus known as Cryptococcosis neoformans. The infection may be spread to humans through contact with pigeon droppings or unwashed raw fruit. Contact with an infected individual may also spread the infection. Individuals with disorders characterized by lowered immunity (for instance, HIV infection) are at high risk for contracting these infections.

Cryptococcosis may appear in various forms depending on how the infection is acquired. In most cases, the infection begins in the lungs (pulmonary form) and may then spread to the brain, urinary tract, skin, and/or bones (disseminated form). When the infection is limited to the lungs, symptoms may be minimal or not apparent at all. Respiratory symptoms may include coughing and chest pain. When the infection spreads, it tends to seek out the central nervous system, especially the brain. In some affected individuals, inflammation of the membranes surrounding the brain and spinal cord (meningitis) may occur as a serious complication. Symptoms associated with meningitis may include dizziness, blurred vision, severe headache, and/or stiffness of the neck. In such cases, immediate treatment is essential to help prevent potentially life-threatening complications.

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Synonyms

  • Busse-Buschke Disease
  • Cryptococcic Meningitis
  • Cryptococcosis Lung
  • Cryptococcosis Skin
  • European Blastomycosis
  • Torular Meningitis
  • Torulosis
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Signs & Symptoms

The pulmonary forms of this disease include acute infections and chronic pulmonary infections. The disseminated forms include central nervous system (brain) infections, cutaneous (skin) infections, and infections involving other organs or systems.

Pulmonary forms.

Acute infections are only rarely diagnosed except in patients with weakened immune systems. In people with normal immune systems, cryptoccosis may not result in any symptoms at all (asymptomatic).

Chronic pulmonary infections may generate rather large masses in the lobes of the lungs, as well as segmental pneumonia (involving parts of lungs), fluid in the lungs (pleural effusions), and swollen lymph nodes.

Disseminated forms

Central nervous system infections, especially of the brain, may present as only modestly severe rather than acute. Complications may include an abnormally large head (hydrocephalus) and failing sight, among others.

Cutaneous or skin infections may present as fluid-filled bumps (papules), hardened plate-like patches (plaques), and ulcerous sores.

Infections of other organs or systems may involve sight (chorioretinitis), ears (otitis), the heart (myocarditis, endocarditis), the digestive system (gastroduodenitis, hepatitis), and the kidneys.

In general, symptoms of various forms of cryptococcosis may include: chest pain, dry cough, headache, nausea, confusion, blurred or double vision, fatigue, fever, unusual and excessive sweating at night, swollen glands without the appearance of infection in nearby areas, skin rash, pinpoint red spots (petechiae), bleeding into the skin, bruises, unintentional weight loss, appetite loss, abdominal bloating, abdominal pain, abdominal swelling, weakness, bone pain, and numbness and/or tingling.

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Causes

Cryptococcosis is caused by the fungus Cyptococcus neoformans. It is spread by contact with pigeon droppings, unwashed raw fruit or by infected individuals. People with immune deficiencies or lowered immunity (such as people undergoing cancer chemotherapy or organ transplants or those infected with HIV-AIDS) are at high risk for contracting this fungal infection.

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Affected populations

Cryptococcosis occurs worldwide. In the United States it occurs predominately in the Southeastern states and usually in adults aged forty to sixty years of age. It tends to occur more often in males than females. Individuals with disorders involving reduced or impaired immunity to infection are particularly at risk.

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Diagnosis

The diagnosis depends on proof of the presence of Cyptococcus neoformans in either a bodily fluid or body tissue. The presence of the fungus may be seen under a light microscope in some circumstances; other circumstances require growing the organism from samples of fluid taken from a patient. An immunological test designed to detect the substance that would be mobilized in the body to fight this fungus if it were present (antigen) is available in commercial kits.

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Standard Therapies

Treatment

Among the antibiotics used to treat cryptococcosis are the anti-fungal agents Amphotericin B, Flucytosine, and Fluconazole. These drugs may have serious side effects, so it is important for their use to be monitored carefully. Individuals with compromised immune systems, or under immune suppressive therapy, should be given prolonged drug treatment to prevent relapses.

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Clinical Trials and Studies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: [email protected]

For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

A phase II/III study sponsored by Schering-Plough, is looking at the safety, tolerance, and efficacy of the anti-fungal agent Posaconazole in the treatment of invasive fungal infections. This study is being conducted at several locations. For information, contact the study registry at (888) 772-8734.

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References

TEXTBOOKS

Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:1218-20.

Berkow R., ed. The Merck Manual-Home Edition.2nd ed. Whitehouse Station, NJ: Merck Research Laboratories; 2003:1151-52.

Mandell GL, Bennett JE, Dolan R, eds. Mandell, Douglas and Bennettโ€™s Principles and Practice of Infectious Diseases. 4th ed. Churchill Livingstone Inc. New York, NY; 1995:1198-200.

REVIEW ARTICLES

Pasqualotto AC, Bittencourt Severo C, de Mattos Oliveira F, et al. Cryptococcemia. An analysis of 28 cases with emphasis on the clinical outcome and its etiologic agent. Rev Iberoam Micol. 2004;21:143-46.

French MA, Price P, Stone SF. Immune restoration disease after antiretroviral therapy. AIDS. 2004;18:1615-27.

Ruhnke M. Mucosal and systemic fungal infections in patients with AIDS: prophylaxis and treatment. Drugs. 2004;64:1163-80.

Perlin DS. Amphotericin B cochleates: a vehicle for oral delivery. Curr Opin Investig Drugs. 2004;5:198-201.

Ellerbroek PM, Walenkamp AM, Hoepelman AI, et al. Effects of the capsular polysaccharides of Cryptococcus neoformans on phagocyte migration and inflammatory mediators. Curr Med Chem. 2004;11:253-66.

Yamada H, Kotaki H, Takahashi T. Recommendations for the treatment of fungal pneumonias. Expert Opin Pharmacother. 2003;4:1241-58.

Perfect JR, Casadevall A. Cryptococcosis. Infect Dis Clin North Am. 2002;16:837-74.

FROM THE INTERNET

Levy D. Cryptococcosis. Medical Encyclopedia. MedlinePlus. Update date: 7/16/2004. 3pp.

www.nlm.nih.gov//medlineplus/ency/article/001328.htm

CDC. Division of Bacterial and Mycotic Diseases. December 2003. 2pp.

www.cdc.gov/ncidod/dbmd/diseaseinfo/cryptococcosis_t.htm

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Programs & Resources

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Ensuring that patients and caregivers are armed with the tools they need to live their best lives while managing their rare condition is a vital part of NORDโ€™s mission.

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More Information

The information provided on this page is for informational purposes only. The National Organization for Rare Disorders (NORD) does not endorse the information presented. The content has been gathered in partnership with the MONDO Disease Ontology. Please consult with a healthcare professional for medical advice and treatment.

GARD Disease Summary

The Genetic and Rare Diseases Information Center (GARD) has information and resources for patients, caregivers, and families that may be helpful before and after diagnosis of this condition. GARD is a program of the National Center for Advancing Translational Sciences (NCATS), part of the National Institutes of Health (NIH).

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Orphanet

Orphanet has a summary about this condition that may include information on the diagnosis, care, and treatment as well as other resources. Some of the information and resources are available in languages other than English. The summary may include medical terms, so we encourage you to share and discuss this information with your doctor. Orphanet is the French National Institute for Health and Medical Research and the Health Programme of the European Union.

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National Organization for Rare Disorders