The symptoms of aspergillosis vary depending upon the specific form of the disorder present. The lungs are usually affected in individuals with aspergillosis. Aspergillosis can present as an allergic reaction, an isolated finding affecting a specific area of the body (e.g., the lungs, sinuses or ear canals), or as an invasive infection that spreads to affect various tissues, mucous membranes or organs of the body.
Allergic Bronchopulmonary Aspergillosis
This form of aspergillosis usually occurs in individuals with long-standing asthma or cystic fibrosis. It is an allergic reaction to the breathing in (inhalation) of the fungi spores. These spores set off an improper response from an individual’s immune system. Initial symptoms usually include fever, wheezing, shortness of breath (dyspnea), and a general feeling of poor health (malaise). Allergic bronchopulmonary aspergillosis may also cause chest pain, a cough that brings up blood or brown-colored globs (plugs) of mucus, and an excess of certain white blood cells (eosinophilia). The infection usually does not spread to other areas, but can cause to chronic widening (dilation) of the bronchial tubes (bronchiectasis).
The most common form of aspergillosis is the development of a fungal ball known as an aspergilloma. These growths consist of a tangled mass of fungus fibers, mucus, tissue debris, inflammatory cells, and blood clotting protein (fibrin). Aspergillomas form in air pockets or cavities found within the lungs that may have been formed from previous lung disease (e.g., tuberculosis or emphysema). Affected individuals may not have any apparent symptoms (asymptomatic) for years. Symptoms that can develop include wheezing, shortness of breath, chest pain, a chronic cough, fatigue, a cough that brings up blood (mild hemoptysis), and weight loss. Fever is rare unless individuals also have a bacterial infection. In some cases, hemoptysis can become severe, potentially causing suffocation (asphyxiation). An aspergilloma usually remains the same size, but can shrink or resolve without treatment. In rare cases, an aspergilloma may gradually grow larger, but usually does not spread to other areas. An aspergilloma may also be known as pulmonary aspergilloma.
Chronic Necrotizing Aspergillosis
This form of aspergillosis, also known as semi-invasive aspergillosis is characterized by infection of the lungs. Unlike an aspergilloma, it can spread to affect surrounding tissue. Chronic necrotizing aspergillosis shows a chronic, slowly progressive process that, unlike invasive aspergillosis, does not spread to other organ systems or the blood vessels (vascular invasion). In some affected individuals, an aspergilloma (fungal ball) may develop in a cavity created by the destruction of lung tissue by the original infection. Chronic necrotizing aspergillosis most often affects middle-aged and elderly individuals. General symptoms associated with this form of aspergillosis include fever, night sweats, a cough that brings up sputum and unintended weight loss for a period of 1-6 months.
Invasive aspergillosis is the most severe form of aspergillosis and usually affects individuals with weakened immune systems or those who have received bone marrow or solid organ transplants. It is characterized by infection that starts in the lungs and then rapidly travels through the bloodstream to affect various organs of the body potentially including the brain, kidneys, heart and skin. The specific symptoms associated with invasive aspergillosis vary based upon the organ system(s) involved. Affected individuals often develop fever, chills headaches, a cough that brings up blood (mild hemoptysis), shortness of breath (dyspnea), and chest pain. Severe life-threatening complications can develop including shock, delirium, massive bleeding from the lungs, and inflammation of the trachea and bronchi (tracheobronchitis), which may cause airway obstruction. Organ failure (e.g., liver or kidney) can occur. If the infection spreads to the brain, seizures, intracranial bleeding, or inflammation of the membranes surrounding the brain (meningitis) can occur.
Additional Forms of Aspergillosis
Aspergillosis may occur as an isolated infection of individual areas such as the nail beds, eyes, skin, sinuses or ear canals. Infection of the ear canals can cause itchiness, pain and the drainage of fluid from the ears. Infection of the sinuses may cause a stuffy nose, congestion, fever, facial pain and headache. An aspergilloma may form in the sinuses or the infection may spread to other areas including the brain. Aspergillosis of the sinuses is sometimes associated with bone loss of the facial bones.
Infection with Aspergillus may cause inflammation of the thin membrane (endocardium) lining the heart, a condition called endocarditis. This condition occurs when heart tissue is directly infected by Aspergillus during cardiac surgery (post-operative aspergillosis). Cases of aspergillosis affecting the site of surgery have also been reported following eye (ophthalmologic) and dental surgery.
As researchers learn more about aspergillosis, new clinical entities (e.g., severe asthma with fungal sensitization [SAFS]) are being identified. More research is necessary to clearly define and separate these diseases from existing forms of Aspergillus infection.
Aspergillosis is an infectious disease that occurs when susceptible individuals breathe in (inhale) fungal spores of the Aspergillus species of mold. Approximately 300 different species of Aspergillus have been identified, but only a few are known to cause diseases in humans. The most common of these are Aspergillus fumigatus, Aspergillus flavus, and Aspergillus niger. Aspergillus spores are found all over the environment both indoors and outdoors especially in decaying vegetation. It is also found in the soil, airborne dust, air vents, foods, spices, humidifiers, and potted plants.
It is not known why most people resist infection with this common fungus, while others appear more susceptible to developing infection. Individuals with a compromised or weakened immune system and those who have received a transplant, especially a bone marrow transplant, are at a greater risk of developing infection. Some individuals may have an improper immune system response to the fungal spores. Aspergillosis may also occur with greater frequency in individuals with diabetes mellitus, cystic fibrosis, asthma, AIDS, cancer or with low white blood cell counts (neutropenia). Individuals who take corticosteroid drugs are also at a greater risk of developing aspergillosis.
Some researchers believe that genetics plays a role in the development of aspergillosis. Researchers speculate that certain, as yet unidentified genes, may make some individuals more likely to develop an infection with Aspergillus (genetic predisposition). Researcher is underway to determine what affect genetics plays in the development of the various forms of aspergillosis.
Researchers have recently mapped the genomes for a few specific types of Aspergillus. A genome is the complete genetic make up of an organism and researchers hope that the genomes will lead them to novel treatment options and to a better understanding of the various differences betweens different species of Aspergillus.
Aspergillosis affects males and females in equal numbers. The exact incidence rate in the general population is unknown. Allergic bronchopulmonary aspergillosis occurs with greater frequency in individuals with asthma or cystic fibrosis. Invasive aspergillosis occurs with greater frequency in individuals who have received an organ transplant, especially a bone marrow transplant. One estimate suggests that 5-13 percent of individuals who have received a BMT have developed invasive aspergillosis. Aspergilloma may occur in individuals with chronic lung disease in which lung damage has left open spaces or cavities.
A diagnosis of aspergillosis is made based upon a detailed patient history, a thorough clinical evaluation, and a variety of specialized tests such as bronchoscopy with biopsy, x-rays, antigen skin tests or blood tests.
During a bronchoscopy, a physician inserts a metal tube called a cannula through the mouth and down an affected individual’s throat and obtains a sample of tissue to be analyzed (biopsy). X-rays of the chest are taken because to detect characteristic findings such as the presence of an aspergilloma in a lung cavity or the buildup (accumulation) of Aspergillus fungi in the lungs. During an Aspergillus antigen skin test, a physician will inject a needle into a specific area of the body. If the area becomes inflamed or irritated with 48-72 hours, the person has been exposed to the Aspergillus fungus. A physician may also perform a blood test to determine whether any aspergillosis antibodies are present. Antibodies, also known as immunoglobulins, are produced by the body to combat invading microorganisms, toxins, or other foreign substances.
Some forms of aspergillosis are easier to diagnose than others. For example, an aspergilloma is usually seen (often incidentally) on a chest x-ray. A diagnosis of invasive aspergillosis, however, is much more difficult. Researchers are studying ways to better and more rapidly diagnose aspergillosis.
In 2015, Cresemba (isavuconazonium sulfate) was approved to treat adults with the rare, serious, fungal infections invasive aspergillosis and invasive mucormycosis. Cresemba is marketed by Astellas Pharma US, Inc.
Individuals with allergic bronchopulmonary aspergillosis are treated with oral corticosteroids such as prednisone (inhaled steroids are ineffective). Corticosteroids suppress the immune system’s improper response to the Aspergillus infection. Some individuals may receive an anti-fungal medication such as itraconazole along with corticosteroids.
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.
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FROM THE INTERNET
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