Disseminated Coccidioidomycosis

Disseminated Coccidioidomycosis

Article
Brain & Nerve
Bone, Muscle, & Joint
+5
Contributed byMaulik P. Purohit MD MPHSep 03, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • Systemic Coccidioidomycosis

What is Disseminated Coccidioidomycosis? (Definition/Background Information)

  • Coccidioidomycosis is an infectious disease caused by inhaling the spores of two related fungi, namely Coccidioides immitis and Coccidioides posadasii
  • C. posadasii is found in the soil of dry and low rainfall areas  and desert regions in certain parts of the South Western U.S, Mexico, Central and South America; C. immitis is found in California
  • A mild attack of coccidioidomycosis affects the upper respiratory system, lungs, and lymph nodes. A serious attack of coccidioidomycosis may spread to other parts of the body, such as the bones, joints, skin, tissues, and brain meninges, and may cause some serious complications

The types of coccidioidomycosis include:

  • Acute Coccidioidomycosis
  • Chronic Coccidioidomycosis
  • Disseminated Coccidioidomycosis

Disseminated Coccidioidomycosis: When the fungal infection spreads through the bloodstream and affects various organs of the human body, such as the skin, bones, joints, lymph nodes, and also the central nervous system, the medical condition is termed as Disseminated Coccidioidomycosis.

  • The major signs and symptoms exhibited by Disseminated Coccidioidomycosis are fever and chills, cough, muscle stiffness and aches, altered mental status, and swelling of the joints. The complications could include meningitis, severe pneumonia, and draining sinus tracts on the skin
  • Disseminated Coccidioidomycosis is treated with antifungal medications. It is a fatal disease if left untreated and the prognosis is typically poor
  • 95% of those infected by acute pulmonary coccidioidomycosis (a mild and initial form of coccidioidomycosis infection), recover completely without any treatment. The prognosis of the acute form is excellent, since it is self-limiting

Who gets Disseminated Coccidioidomycosis? (Age and Sex Distribution)

  • Any individual may be affected by coccidioidomycosis. Disseminated Coccidioidomycosis can easily affect infants, children, and older adults. Individuals with weak immune systems are at high risk of contracting the condition
  • Males are more easily affected by Disseminated Coccidioidomycosis than females
  • Coccidioides immitis is confined to the San Joaquin Valley region of California, while Coccidioides posadasii is more widespread in its distribution, ranging from the arid and dry regions of South America, Mexico, to the American southwest. The infection is not found in any other parts of the world
  • Individuals living in or traveling to these regions (lower sonoran life zone) may be affected, since the fungus is endemic to this region

What are the Risk Factors for Disseminated Coccidioidomycosis? (Predisposing Factors)

The risk factors for Disseminated Coccidioidomycosis include:

  • Individuals living in the endemic regions; travelling to areas that include the South Western New Mexico, Arizona, California, San Joaquin Valley, and Western Texas
  • Individuals with weak immune systems, which may be caused by:
    • AIDS/HIV infection
    • Cancer therapy, major surgeries
    • Diabetes
    • Taking medicines that suppresses or weakens the immune system (individuals taking immunosuppressive therapy after organ transplant)         
  • Pregnancy
  • Consuming medicines that weaken the immune system such as:
    • Steroids
    • TNF-inhibitors
    • Chemotherapy drugs           
  • Cancers such as lymphoma

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Disseminated Coccidioidomycosis? (Etiology)

  • Coccidioidomycosis is an airborne-infection that is acquired by inhalation of the coccidioides spores present in air (carried by dust)
  • This infection may be caused by any of the two coccidioides species of fungus that include C. immitis and C. posadasii
  • The infection may be mild or severe depending on whether the infection is confined to the respiratory system or spreads to other body regions such as the bone and joints, skin, brain, etc.
  • Complications of acute pulmonary coccidioidomycosis include progression of the condition to its chronic infection form and spread of the fungus throughout the body. This causes Disseminated Coccidioidomycosis that can be fatal without proper treatment

What are the Signs and Symptoms of Disseminated Coccidioidomycosis?

Most often, individuals do not exhibit any signs and symptoms; but when it is present, it may be either mild or severe. The major signs and symptoms exhibited by Disseminated Coccidioidomycosis may include:

  • Fever and chills
  • Cough
  • Headache
  • Stiffness of the muscle in the neck and shoulder, muscle aches
  • Altered mental status
  • Increased sensitivity to light
  • Arthritis: Swelling of the joints, joint pain; swelling is generally found in the ankle, feet and legs
  • Erythema nodosum - rashes and painful lumps on the lower legs
  • Occurrence  of skin nodules, ulcers, and other skin lesions
  • The painful lesions starts appearing in the skull, spine, or other bones
  • Meningitis: Inflammation that occurs in the protective  coverings surrounding the brain and spinal cord

The combination of signs and symptoms of Disseminated Coccidioidomycosis depend upon the body systems affected by the infection.

How is Disseminated Coccidioidomycosis Diagnosed?

Disseminated Coccidioidomycosis is diagnosed using the following methods:

  • Complete evaluation of medical history along with a thorough physical examination (special focus on lung examination)
  • A neurological examination involving the brain and nervous system may show abnormalities
  • Diagnostic tests may include the following:
    • CBC with differential count
    • Chest x-ray (a chest radiograph): It is a painless, noninvasive procedure to take pictures of the structures, inside the chest
    • Coccidioides skin test: Also known as the spherulin test, this test helps to determine if an individual is infected with the fungus that causes coccidioidomycosis
    • Sputum KOH test: A laboratory test to check for the fungus in a sputum sample
    • Coccidioides complement fixation titer: A blood test that is conducted to detect antibodies to the fungus C. immitis
    • Sputum culture: This test is used for detecting and identifying fungus from a sputum culture. The test is generally used to diagnose a suspected respiratory tract infection          
  • A tissue biopsy may be performed to determine the site of disseminated disease and it may include:
    • Bone marrow biopsy
    • Liver biopsy
    • Open lung biopsy
    • Skin lesion biopsy
    • Family history of cardiovascular disease             

Tissue biopsy: A sample of cells and tissues is examined by a pathologist under the microscope. Pathological examination is generally considered to be a ‘gold standard’ for a definitive diagnosis. The pathologist arrives at a diagnosis after analyzing the pathology findings along with clinical information of the patient. A pathologist may perform special studies on tissue samples to aid in the final diagnosis.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Disseminated Coccidioidomycosis?

Complications that may arise due to Disseminated Coccidioidomycosis could include:

  • Meningitis (inflammation of the protective membranes covering the brain and spinal cord)
  • Severe pneumonia
  • Draining sinus tracts on the skin

Various other complications may arise depending upon the spread of infection, either to the bones, lungs, liver, brain, skin, or heart and pericardium.

How is Disseminated Coccidioidomycosis Treated?

The following treatment methods are adopted to treat Disseminated Coccidioidomycosis:

  • Antifungal medications: Amphotericin B, ketoconazole, fluconazole, and itraconazole are usually the drugs of choice
  • Complete bed rest
  • Improved nutrition and overall health status

How can Disseminated Coccidioidomycosis be Prevented?

Currently, there are no definitive methods available to prevent coccidioidomycosis; though, the following measures may be helpful:

  • Limit exposure to soil and dust if you are in coccidioides-endemic region
  • Avoid travelling to the regions where the fungus is endemic, especially if you have a poor immune system. Maintaining good health will help in reducing the severity of Disseminated Coccidioidomycosis
  • Being watchful of diseases and conditions that can suppress the immune system. This will help in preventing severe forms of the disease
  • If travelling to these regions is unavoidable, then efforts must be taken to avoid contact with the soil and dust in these regions, particularly if you have a weak immune system caused by HIV and other medical conditions
  • Medical attention should be sought immediately when the symptoms get worse and  do not improve with treatment, or when new symptoms begin to appear

What is the Prognosis of Disseminated Coccidioidomycosis? (Outcomes/Resolutions)

  • Without suitable treatment, Disseminated Coccidioidomycosis is a fatal condition
  • Generally, the prognosis is not very good for those with disseminated forms of the infection. A high death rate has been observed with Disseminated Coccidioidomycosis
  • Death may be rapid in patients with weak immune system

Additional and Relevant Useful Information for Disseminated Coccidioidomycosis:

Coccidioides immitis and coccidioides posadasii are endemic in New Mexico, Texas, and parts of Central and South America, which are generally areas with mild winters and arid summers.

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Maulik P. Purohit MD MPH picture
Approved by

Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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