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$Unique_ID{BRK03499}
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$Title{Aspergillosis}
$Subject{Aspergillosis Pulmonary Aspergillosis Bronchopulmonary Allergic
Aspergillosis Pulmonary Mycetoma Aspergilloma Invasive Aspergillosis
Endocarditis Madu'ra Foot Mycetoma Histoplasmosis Crytococcosis Lymphomatoid
Granulomatosis Tuberculosis Cystic Fibrosis Leukemia Chronic Myelogenous
Hodgkin's Disease Sarcoidosis Churg-Strauss Syndrome AIDS Bronchiectasis}
$Volume{}
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Copyright (C) 1989, 1992 National Organization for Rare Disorders, Inc.

737:
Aspergillosis

** IMPORTANT **
It is possible that the main title of the article (Aspergillosis) is not
the name you expected.  Please check the SYNONYM listing to find the
alternate names and disorder subdivisions covered by this article.

Synonyms

     Pulmonary Aspergillosis

DISORDER SUBDIVISIONS

     Bronchopulmonary Allergic Aspergillosis
     Pulmonary Mycetoma; Aspergilloma
     Invasive Aspergillosis
     Endocarditis
     Madu'ra Foot; Mycetoma

Information on the following diseases can be found in the Related
Disorders section of this report:

     Histoplasmosis
     Crytococcosis
     Lymphomatoid Granulomatosis
     Tuberculosis
     Cystic Fibrosis
     Leukemia, Chronic Myelogenous
     Hodgkin's Disease
     Sarcoidosis
     Churg-Strauss Syndrome
     AIDS
     Bronchiectasis

General Discussion

** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only.  It should not be used for diagnostic or treatment
purposes.  If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.

Aspergillosis is a term used to describe a group of pulmonary infections
caused by inhaling the fungus Aspergillus.  Most people who are exposed to
this common fungus are not affected by it, but those with an impaired immune
system, or an abnormal immune response, can become ill.

Symptoms

There are several forms of Aspergillosis infections.  All are caused by one
or more of the many different Aspergillus fungus species; only a few of these
organisms are capable of producing disease in humans.  These species are
spread through the air and can be found almost any place in the world.
Because these spores are inhaled, they usually affect the lungs or other
airway passages such as the bronchial tubes, nose and sinuses.  The fungi can
be invasive, affecting any tissue, mucous membrane or vital organ of the
body.

Allergic Bronchopulmonary Aspergillosis (ABPA) is an immune disorder that
occurs in people with asthma or chronic obstructive pulmonary disease (COPD)
infected with Aspergillosis fungi.  It causes an excess of certain white
blood cells (eosinophils), an infiltration of the lungs, and impaction of the
bronchial tubes with eosinophils and aspergillus organisms.  Symptoms of this
disease may consist of fever, shortness of breath (dyspnea), chest pain,
wheezing, a cough with sputum (with or without blood) or a generalized
feeling of ill-health (malaise).  This form of Aspergillosis is not usually
invasive, but it can lead to a chronic dilation of the bronchial tubes
(bronchiectasis).

Pulmonary Mycetoma, also known as Aspergilloma or "fungus ball", is a
form of Aspergillosis that often occurs as a result of the Aspergillus fungus
growing together (colonization) in a cavity of the lungs.  These cavities are
usually caused by other pulmonary diseases such as tuberculosis, sarcoidosis,
histoplasmosis or coccioidomycosis.  The fungus ball can be seen on x-rays.
This form of Aspergillosis is characterized by a chronic cough, weight loss,
a generalized feeling of ill-health (malaise) and the spitting up of blood
(hemoptysis).

Fulminative or Invasive Aspergillosis is another form of this disorder
that can cause distribution of the Aspergillus fungus to other parts of the
body.  This disease can progress from a localized infection to a widespread
erosion and ulceration of the bronchial system and an inflammation of the
vascular system (vasculitis).  Vasculitis is a narrowing of the inside of a
blood vessel that can obstruct the flow of blood to the tissues.  This lack
of blood can cause damage to the tissues (necrosis), and a possible formation
of blood clots (thrombosis).  Invasive Aspergillosis is usually first
confined to the lungs but it can spread through the blood to other organs
especially the liver, brain, kidneys, skin, gastrointestinal tract and other
sites.  This can be a very serious disease which can have a slow or rapid
course.  It is seen in those whose immune system has been weakened by other
illnesses, especially people with cancer (e.g., Leukemia, Hodgkin's Disease),
kidney transplant patients or those undergoing certain drug therapies.  (For
more information on this disorder, choose "Vasculitis" as your search term in
the Rare Disease Database.

Occasionally, Aspergillosis can cause Infective Endocarditis which is an
infection of the inner lining of the heart muscle (endocardium).  A type of
infective endocarditis, prosthetic valvular endocarditis (PVE), may develop
in patients who have previously had artificial (prosthetic) heart valve
replacement.  This infection may occur as a result of contamination of the
operating room area by the Aspergillus spores.  Drug addicts are also more
susceptible to this form of Aspergillosis.  (For more information on this
disorder, choose "Endocarditis" as your search term in the Rare Disease
Database.)

Mycetoma, also known as Madu'ra Foot, is a chronic infection produced by
Aspergillosis as well as several other fungi.  It is a progressive fungal
disease that is characterized by lesions of the foot, face, trunk, hand or
leg.  These lesions can cause swelling, hardening, pus formation, sinus
drainage and abscesses that can lead to bone destruction and deformities.  It
is more commonly seen in tropical climates.

Causes

Aspergillosis is a group of infectious diseases that are caused by the
inhalation of the Aspergillus fungus.  The spores that cause these infections
can be found in decaying vegetable matter, grains, grass, leaves, soil, wet
paint, air conditioning systems, on refrigerator walls and in construction
and fireproofing materials.  It is not known why most people resist infection
from this common fungus, and why others are more susceptible to infection.  A
weakened immune system, or an abnormal immune response to the fungus, may
cause the fungus to proliferate and become a threat to one's health.

Affected Population

Aspergillosis is a rare disorder that affects males and females in equal
numbers.  It is seen more often in those people who have chronic respiratory
problems or whose immune system has been weakened by other serious illnesses
or drug therapy.

Related Disorders

Symptoms of the following disorders can be similar to those of Aspergillosis.
Comparisons may be useful for a differential diagnosis:

Histoplasmosis is a disease caused by the inhalation of a fungus.  It is
a self-limiting disease that is characterized by chills, headache, fever,
chest pain, cough and occasionally lumps on the legs.

Cryptococcosis is caused by a fungus known as Filobasidiella Neoformans
or Cryptococcosis Neoformans.  The infection can be spread to humans through
contact with pigeon droppings or unwashed raw fruit.  Contact with an
infected individual may also spread the infection.  Persons with disorders
characterized by lowered immunity are at high risk to contract this
infection.  Initially involving the lungs, it may later spread to the
kidneys, bones and skin.  (For more information on this disorder, choose
"Cryptococcosis" as your search term in the Rare Disease Database).

People with the following disorders may be more susceptible to developing
Aspergillosis.  They are not necessary for a differential diagnosis:

Cystic Fibrosis is an inherited disorder that affects the exocrine, or
outward secreting, glands of the body.  It affects children and young adults.
The main consequences are related to the mucous producing glands.  The
secreted mucous is thick and sticky, clogging and obstructing air passages in
the lungs and pancreatic ducts.  Cystic Fibrosis also causes dysfunction of
salivary and sweat glands.  (For more information on this disorder, choose
"Cystic Fibrosis" as your search term in the Rare Disease Database.)

Tuberculosis (TB) is an acute or chronic bacterial infection found most
commonly in the lungs.  The infection is spread like a cold, mainly through
airborne droplets breathed into the air by a person infected with TB.  The
bacteria causes formation of small tissue masses called tubercles.  In the
lungs these tubercles produce breathing impairment, coughing and release of
sputum.  TB may recur after long periods of inactivity (latency) if not
treated adequately.  Many variations of TB exist and are distinguished by the
area of the body affected, degree of severity and affected population.  (For
more information on this disorder, choose "TB" as your search term in the
Rare Disease Database).

Chronic Myelogenous Leukemia is characterized by an excess amount of
white blood cells in the bone marrow, spleen, liver and blood.  As the
disease progresses, the leukemic cells invade other areas of the body
including the intestinal tract, kidneys, lungs, gonads and lymph nodes.  (For
more information on this disorder, choose "Leukemia, Chronic Myelogenous" as
your search term in the Rare Disease Database.)

Hodgkin's Disease is a form of cancer of the lymphatic system, especially
the lymph nodes.  Lymph nodes and other organs manufacture white blood cells
that destroy foreign disease-causing invaders such as bacteria, viruses, etc.
Tumors occur in the lymph nodes (places where lymphatic vessels unite) and/or
the area around the nodes.  Fever, night sweats, and weight loss may occur
along with swollen lymph nodes.  (For more information on this disorder,
choose "Hodgkin" as your search term in the Rare Disease Database.)

Lymphomatoid Granulomatosis is a rare, progressive, vascular disease
characterized by infiltration and destruction of the veins and arteries by
lesions.  These lesions can affect various parts of the body, especially the
lungs.  It can be a benign or malignant condition.  People with Lymphomatoid
Granulomatosis are at high risk for developing Aspergillosis.  (For more
information on this disorder, choose "Lymphomatoid Granulomatosis" as your
search term in the Rare Disease Database.)

In the Acquired Immune Deficiency Syndrome (AIDS), the body's ability to
ward off infection progressively deteriorates.  Organisms which in a healthy
person would either fail to cause disease, cause mild disease or at least
provoke immunity, completely overwhelm the AIDS patient.  Patients with
severe AIDS also contract various uncommon, life-threatening infections,
particularly pneumocystis carinii pneumonia, and have an unusually high
incidence of a rare cancer, Kaposi's sarcoma and also Aspergillosis.  (For
more information on this disorder, choose "AIDS" as your search term in the
Rare Disease Database.)

Sarcoidosis is a disorder which affects many body systems.  It is
characterized by small round lesions (tubercles) of granulation tissue.
Symptoms may vary depending on the severity of the disease and how much of
the body is affected.  (For more information on this disorder, choose
"Sarcoidosis" as your search term in the Rare Disease Database.)

Churg-Strauss Syndrome is a lung disorder often occurring as a
complication of other disorders.  Allergic blood vessel inflammation
(angiitis or vasculitis)  is accompanied by many inflammatory nodular lesions
(granulomatosis) which may be small and granular, and are made up of
compactly grouped cells.  The age of onset varies from 15 to 70 years of age.
(For more information on this disorder, choose "Churg-Strauss" as your search
term in the Rare Disease Database.)

Allergic Bronchopulmonary Aspergillosis may precede the development of
the following disorder:

Bronchiectasis is an abnormal dilation of the bronchial tubes with
increased mucous secretions.  These secretions can become infected and cause
an inflammation of the bronchial walls.  It is a progressive disease,
characterized by a cough, a large amount of foul smelling, pus-containing
blood-streaked phlegm, shortness of breath (dyspnea), anemia and a general
feeling of ill-health (malaise).

Therapies:  Standard

Treatment of Aspergillosis depends on the form of the disease and the area
affected.  The corticosteroid drug prednisone, has been found to be effective
in treating Bronchopulmonary Aspergillosis.  Invasive and scattered forms of
the disease should be treated promptly with the anti-fungal drug Amphotericin
B.  Surgery may be indicated in cases of Aspergillus endocarditis.

Therapies:  Investigational

At the present time, a study is being conducted on the effectiveness of the
therapeutic agents itraconazole, imidazoles, ketoconazole and fluconazole as
treatments for Aspergillosis.  More research must be conducted to determine
long-term safety and effectiveness of these drugs.

Therapies:  Investigational
Clinical trials are underway to study cells, cll products, and antibodies in
Aspergillosis.  Interested persons may wish to contact:

     Dr. Paul Greenberger
     Northwestern University, Allergy/Immunology Section
     303 E. Chicago Ave.
     Chicago, IL  60611
     [phone]

to see if further patients are needed for this research.

This disease entry is based upon medical information available through
January 1992.  Since NORD's resources are limited, it is not possible to keep
every entry in the Rare Disease Database completely current and accurate.
Please check with the agencies listed in the Resources section for the most
current information about this disorder.

Resources

For more information on Aspergillosis, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     [phone]

     NIH/National Institute of Allergy and Infectious Diseases (NIAID)
     9000 Rockville Pike
     Bethesda, MD  20892
     [phone]

     Centers for Disease Control (CDC)
     1600 Clifton Road, NE
     Atlanta, GA 30333
     [phone]

References

INTERNAL MEDICINE, 2nd Ed.:  Jay H. Stein, ed.-in-chief; Little, Brown and
Co., 1987.  Pp. 1765.

PULMONARY DISEASES AND DISORDERS, Volume 2, 2nd Ed.:  Alfred P. Fishman,
M.D., ed.-in-chief; McGraw-Hill Book Co., 1980.  Pp. 1639.

TEXTBOOK OF MEDICINE, 18th Ed.:  James B. Wyngaarden, M.D., et al.; ed.-
in-chief; W.B. Saunders Co., 1988.  Pp.1850.

RAPID DIAGNOSIS OF CANDIDIASIS AND ASPERGILLOSIS.  J. Bennett, REV INFECT
DIS (March-April 1987, issue 9(2)).  Pp. 398-402.

ALLERGIC BRONCHOPULMONARY ASPERGILLOSIS.  P. Bock; AM FAM PHYSICIAN
(January 1988, issue 37(1)).  Pp. 177-182.

INVASIVE ASPERGILLOSIS IN CHILDREN.  E. Golladay, et al.; J PEDIATR SURG
(June 1987, issue 22(6)).  Pp.  504-505.

ASPERGILLOMA IN SARCOID AND TUBERCULOSIS.  J. Tomlinson, et al.;
(September 1987, issue 92(3)).  Pp. 505-508.

ASPERGILLOSIS.  S. Levitz; INFECT DIS CLIN NORTH AM (March 1989, issue
3(1)).  Pp. 1-18.