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$Title{Pulmonary Alveolar Proteinosis}
$Subject{Pulmonary Alveolar Proteinosis Phospholipidosis Pneumonia}
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Copyright (C) 1988, 1989, 1993 National Organization for Rare Disorders,
Inc.

594:
Pulmonary Alveolar Proteinosis

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

Synonyms

     Phospholipidosis

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

     Pneumonia

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 physician and/or the agencies listed in the "Resources" section
of this report.


Pulmonary Alveolar Proteinosis is a rare lung disorder characterized by
the filling of the air sacs of the lungs (alveoli) with grainy material
consisting mostly of protein and fat.  Breathing becomes progressively
difficult.  The disorder occurs in different forms, ranging from mild to
severe.

Symptoms

Pulmonary Alveolar Proteinosis is a rare lung disorder characterized in most
cases by breathing difficulty that gradually becomes more severe, especially
following exertion.  However, some patients show no symptoms of the disorder.
The air sacs in the lungs (alveoli) are filled with a granular material
consisting mostly of protein and fat (phospholipid).  Certain cells called
macrophages, that usually swallow inhaled particles in the lung alveoli, can
be found to contain the phospholipid material.  This disorder may spread
throughout the lungs or be confined to a small area.  The disorder may
progress, remain stable, or spontaneously clear.  The lower and rear lung
regions are most commonly affected.  Occasionally, only the front segments
may be involved.

Causes

The exact cause of Pulmonary Alveolar Proteinosis is not known.  Exposure to
aluminum dust has been related to onset of this disorder in rare cases.  A
few cases have occurred in patients with impaired immune systems.

ADD Disorder may, in some cases, be closely related to a generalized
resistance to thyroid hormone.  Researchers have studied the subject with the
conclusion being that there is evidence of a familial predisposition to the
disorder in some persons with a generalized resistance to thyroid hormone.

Affected Population

Pulmonary Alveolar Proteinosis affects males in greater numbers than females.
Most cases begin between 20 and 50 years of age.

Related Disorders

Symptoms of the following disorder can resemble those of Pulmonary Alveolar
Proteinosis.  Comparisons may be useful for a differential diagnosis:

Pneumonia is an acute infection of the alveolar spaces and/or the
interstitial tissue of the lung.  A whole lobe or only a part of a lobe can
be involved.  The pneumonia may be caused by different bacteria, viruses,
fungi, or protozoa.  Pneumonia is often preceded by upper respiratory
infection.  Symptoms usually start suddenly, with a shaking chill, sharp pain
in the affected side of the chest, a cough, a high fever, and a headache.
Breathing is difficult and painful.

Therapies:  Standard

Mild cases of Pulmonary Alveolar Proteinosis may spontaneously go into
remission.  More severe cases of this disorder may be treated by lavage under
general anesthesia; i.e., rinsing out one of the lungs while the other lung
keeps breathing.  In some cases only one lavage is necessary while in other
cases the lavage needs to be repeated several times.  Secondary lung
infections should be promptly identified and treated.

Therapies:  Investigational

This disease entry is based upon medical information available through
November 1988.  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 Pulmonary Alveolar Proteinosis, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     (203) 746-6518

     American Lung Association
     1740 Broadway
     New York, NY  10019
     (212) 315-8700

     NIH/National Heart, Blood and Lung Institute
     9000 Rockville Pike
     Bethesda, MD  20892
     (301) 496-4236

     Info-Line
     1-800-222-LUNG

References

BRONCHOPULMONARY LAVAGE IN PULMONARY ALVEOLAR PROTEINOSIS:  CHEST RADIOGRAPH
OBSERVATIONS:  M.E. Gale, et al.; AJR (May 1986:  issue 146(5)).  Pp. 981-985.

TOTAL LUNG LAVAGE FOR PULMONARY ALVEOLAR PROTEINOSIS IN AN INFANT WITHOUT
THE USE OF CARDIOPULMONARY BYPASS:  F. Moazam, et al.; Journal Pediatr Surg
(August 1985:  issue 20(4)).  Pp. 398-401.

MORPHOLOGIC DIAGNOSIS OF IDIOPATHIC PULMONARY ALVEOLAR LIPOPROTEINOSIS
REVISITED:  I. Rubinstein, et al.; Arch Int Med (April 1988:  issue 148(4)).
Pp. 813-816.