Aspergillosis

Overview

Aspergillosis is an infection caused by a type of mold (fungus). The illnesses resulting from aspergillosis infection usually affect the respiratory system, but their signs and severity vary greatly.

The mold that triggers the illnesses, aspergillus, is everywhere — indoors and outdoors. Most strains of this mold are harmless, but a few can cause serious illnesses when people with weakened immune systems, underlying lung disease or asthma inhale their fungal spores.

In some people, the spores trigger an allergic reaction. Other people develop mild to serious lung infections. The most serious form of aspergillosis — invasive aspergillosis — occurs when the infection spreads to blood vessels and beyond.

Depending on the type of aspergillosis, treatment may involve observation, antifungal medications or, in rare cases, surgery.

Symptoms

The signs and symptoms of aspergillosis vary with the type of illness you develop:

Allergic reaction

Some people with asthma or cystic fibrosis have an allergic reaction to aspergillus mold. Signs and symptoms of this condition, known as allergic bronchopulmonary aspergillosis, include:

• Fever
• A cough that may bring up blood or plugs of mucus
• Worsening asthma

Aspergilloma

Certain chronic lung (pulmonary) conditions, such as emphysema, tuberculosis or advanced sarcoidosis, can cause air spaces (cavities) to form in the lungs. When people with lung cavities are also infected with aspergillus, fungus fibers may find their way into the cavities and grow into tangled masses (fungus balls) known as aspergillomas.

Aspergillomas may produce no symptoms or cause only a mild cough at first. Over time and without treatment, however, aspergillomas can worsen the underlying chronic lung condition and possibly cause:

• A cough that often brings up blood (hemoptysis)
• Wheezing
• Shortness of breath
• Unintentional weight loss
• Fatigue

Invasive aspergillosis

This is the most severe form of aspergillosis. It occurs when the infection spreads rapidly from the lungs to the brain, heart, kidneys or skin. Invasive aspergillosis occurs only in people whose immune systems are weakened as a result of cancer chemotherapy, bone marrow transplantation or a disease of the immune system. Untreated, this form of aspergillosis may be fatal.

Signs and symptoms depend on which organs are affected, but in general, invasive aspergillosis can cause:

• Fever and chills
• A cough that brings up blood (hemoptysis)
• Shortness of breath
• Chest or joint pain
• Headaches or eye symptoms
• Skin lesions

Other types of aspergillosis

Aspergillus can invade areas of your body other than your lungs, such as your sinuses. In your sinuses, the fungus can cause a stuffy nose sometimes accompanied by drainage that may contain blood. Fever, facial pain and headache may also occur.

When to see a doctor

If you have asthma or cystic fibrosis, see your doctor whenever you notice a change in your breathing. Although aspergillosis may not be the cause, it's important to have breathing problems evaluated.

If you have a weakened immune system and develop an unexplained fever, shortness of breath or a cough that brings up blood, get immediate medical care. In the case of invasive aspergillosis, prompt treatment is crucial. In some cases, treatment with antifungal medication begins as soon as aspergillosis is suspected, even before testing has confirmed the diagnosis.

Causes

Aspergillus mold is unavoidable. Outdoors, it's found in decaying leaves and compost and on plants, trees and grain crops.

Everyday exposure to aspergillus is rarely a problem for people with healthy immune systems. When mold spores are inhaled, immune system cells surround and destroy them. But people who have a weakened immune system from illness or immunosuppressant medications have fewer infection-fighting cells. This allows aspergillus to take hold, invading the lungs and, in the most serious cases, other parts of the body.

Aspergillosis is not contagious from person to person.

Risk factors

Your risk of developing aspergillosis depends on your overall health and the extent of your exposure to mold. In general, these factors make you more vulnerable to infection:

• Weakened immune system. People taking immune-suppressing drugs after undergoing transplant surgery — especially bone marrow or stem cell transplants — or people who have certain cancers of the blood are at highest risk of invasive aspergillosis. People in the later stages of AIDS also may be at increased risk.
• Low white blood cell level. People who have had chemotherapy, an organ transplant or leukemia have lower white cell levels, making them more susceptible to invasive aspergillosis. So does having chronic granulomatous disease — an inherited disorder that affects immune system cells.
• Lung cavities. People who have air spaces (cavities) in their lungs are at higher risk of developing aspergillomas.
• Asthma or cystic fibrosis. People with asthma and cystic fibrosis, especially those whose lung problems are long-standing or hard to control, are more likely to have an allergic response to aspergillus mold.
• Long-term corticosteroid therapy. Long-term use of corticosteroids may increase the risk of opportunistic infections, depending on the underlying disease being treated and what other drugs are being used.

Complications

Depending on the type of infection, aspergillosis can cause a variety of serious complications:

• Bleeding. Both aspergillomas and invasive aspergillosis can cause severe, and sometimes fatal, bleeding in your lungs.
• Systemic infection. The most serious complication of invasive aspergillosis is the spread of the infection to other parts of your body, especially your brain, heart and kidneys. Invasive aspergillosis spreads rapidly and may be fatal.

Prevention

It's nearly impossible to avoid exposure to aspergillus, but if you have had a transplant or are undergoing chemotherapy, try to stay away from places where you're likely to encounter mold, such as construction sites, compost piles and buildings that store grain. If you have a weakened immune system, your doctor may advise you to wear a face mask to avoid being exposed to aspergillus and other airborne infectious agents.

Diagnosis

Diagnosing an aspergilloma or invasive aspergillosis can be difficult. Aspergillus is common in all environments but difficult to distinguish from certain other molds under the microscope. The symptoms of aspergillosis are also similar to those of other lung conditions such as tuberculosis.

Your doctor is likely to use one or more of the following tests to pinpoint the cause of your symptoms:

• Imaging test. A chest X-ray or computerized tomography (CT) scan — a type of X-ray that produces more-detailed images than conventional X-rays do — can usually reveal a fungal mass (aspergilloma), as well as characteristic signs of invasive aspergillosis and allergic bronchopulmonary aspergillosis.
• Respiratory secretion (sputum) test. In this test, a sample of your sputum is stained with a dye and checked for the presence of aspergillus filaments. The specimen is then placed in a culture that encourages the mold to grow to help confirm the diagnosis.
• Tissue and blood tests. Skin testing, as well as sputum and blood tests, may be helpful in confirming allergic bronchopulmonary aspergillosis. For the skin test, a small amount of aspergillus antigen is injected into the skin of your forearm. If your blood has antibodies to the mold, you'll develop a hard, red bump at the injection site. Blood tests look for high levels of certain antibodies, indicating an allergic response.
• Biopsy. In some cases, examining a sample of tissue from your lungs or sinuses under a microscope may be necessary to confirm a diagnosis of invasive aspergillosis.

Treatment

Aspergillosis treatments vary with the type of disease. Possible treatments include:

• Observation. Simple, single aspergillomas often don't need treatment, and medications aren't usually effective in treating these fungal masses. Instead, aspergillomas that don't cause symptoms may simply be closely monitored by chest X-ray. If the condition progresses, then antifungal medications may be recommended.
• Oral corticosteroids. The goal in treating allergic bronchopulmonary aspergillosis is to prevent existing asthma or cystic fibrosis from worsening. The best way to do this is with oral corticosteroids. Antifungal medications by themselves aren't helpful for allergic bronchopulmonary aspergillosis, but they may be combined with corticosteroids to reduce the dose of steroids and improve lung function.

• Antifungal medications. These drugs are the standard treatment for invasive pulmonary aspergillosis. The most effective treatment is a newer antifungal drug, voriconazole (Vfend). Amphotericin B is another option.

  All antifungal drugs can have serious side effects, including kidney and liver damage. Interactions between antifungal drugs and other medications are also common.

• Surgery. Because antifungal medications don't penetrate an aspergilloma very well, surgery to remove the fungal mass is the first-choice treatment when an aspergilloma causes bleeding in the lungs.
• Embolization. This procedure stops lung bleeding caused by an aspergilloma. A radiologist injects a material through a catheter that has been guided into an artery feeding a lung cavity where an aspergilloma is causing blood loss. The injected material hardens, blocking the blood supply to the area and stopping the bleeding. This treatment works temporarily, but the bleeding is likely to start again.

Preparing for your appointment

People who develop aspergillosis usually have an underlying condition, such as asthma or cystic fibrosis, or have a weakened immune system due to illness or to immune-suppressing medications. If you have symptoms of aspergillosis and are already being treated for a medical condition, call the doctor who provides your care for that condition. In some cases, when you call to set up an appointment, your doctor may recommend urgent medical care.

If you have a weakened immune system and develop an unexplained fever, shortness of breath or a cough that brings up blood, seek immediate medical care.

If you have time to prepare before seeing your doctor, here's some information to help you get ready for your appointment.

What you can do

• Be aware of any pre- or post-appointment restrictions. When you call for the appointment, ask if there's anything you need to do in advance.
• Write down your key medical information. If you are going to see a new doctor, bring a summary of other conditions for which you're being treated, as well as recent medical appointments or hospitalizations.
• Bring all of your medications with you, preferably in their original bottles. If the doctor you are seeing doesn't have access to your medical records or previous imaging tests, such as X-rays or computed tomography (CT) scans, try to get copies to take with you.
• Take along a family member or friend. Aspergillosis can be a medical emergency. Take someone who can understand and recall all the information your doctor provides and who can stay with you if you need immediate treatment.
• Write down questions to ask your doctor.

Prepare a list of questions so that you can make the most of your time with your doctor. For aspergillosis, some basic questions to ask your doctor include:

• What is likely causing my symptoms?
• Other than the most likely cause, what are other possible causes for my symptoms?
• What tests do I need?
• Do I need to be hospitalized?
• What treatment do you recommend?
• What are the possible side effects from the medications you're recommending?
• How will you monitor my response to treatment?
• Am I at risk of long-term complications from this condition?
• I have another health condition. How can I best manage these conditions together?

Don't hesitate to ask other questions.

What to expect from your doctor

Your doctor is likely to ask you some questions, including:

• What are your symptoms?
• Have you seen other doctors for this?
• When did you begin experiencing symptoms?
• How severe are your symptoms? Do they seem to be getting worse?
• Have you had a fever?
• Are you having difficulty breathing?
• Are you coughing up blood?
• What else concerns you?