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$Unique_ID{BRK04206}
$Pretitle{}
$Title{Sarcoidosis}
$Subject{Sarcoidosis Besnier-Boeck Schaumann Disease Boeck Sarcoid
Hutchinson-Boeck Syndrome Benign Lymphogranulamatosis Schaumann Syndrome Lupus
Pernio Uveoparotid Fever }
$Volume{}
$Log{}
Copyright (C) 1986, 1992, 1993 National Organization for Rare Disorders,
Inc.
215:
Sarcoidosis
** IMPORTANT **
It is possible the main title of the article (Sarcoidosis) is not the
name you expected. Please check the SYNONYMS listing on the following page
to find the alternate names and disorder subdivisions covered by this
article.
Synonyms
Besnier-Boeck Schaumann Disease
Boeck Sarcoid
Hutchinson-Boeck Syndrome
Benign Lymphogranulamatosis
Schaumann Syndrome
Lupus Pernio
Including Uveoparotid Fever
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.
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.
Symptoms
Symptoms of Sarcoidosis depend on the site of involvement and may be absent,
slight, or severe. Function may be impaired by the active granulomatous
disease or by secondary fibrosis. Fever, weight loss, and arthralgias (joint
pain) may be initial manifestations. Persistent fever is especially common
with liver (hepatic) involvement. Peripheral lymphadenopathy (enlarged lymph
glands) is common and usually asymptomatic. Both enlarged and normal-sized
lymph nodes may contain the characteristic sarcoid tubercles.
The lymph glands between the two lungs are often affected and may be seen
during routine chest x-rays. Disease of the right lymph nodes next to the
trachea is common in this disorder. Lymph gland involvement is occasionally
unilateral.
Diffuse lung infiltration may accompany or follow lymph gland involvement;
this infiltration may have a diffuse fine ground-glass appearance on the
x-ray, and may occur as reticular (resembling a net) or miliary (resembling a
millet seed) lesions. They may also resemble metastatic tumors. Pulmonary
involvement is usually characterized by cough and difficulty in breathing
(dyspnea), but these symptoms may be minimal or even absent. Pulmonary
fibrosis, cystic changes, and cor pulmonale (heart disease due to pulmonary
hypertension) may occur as a result of long-standing progressive disease.
Skin lesions (i.e., plaques, papules, subcutaneous nodules) frequently
are present in patients with severe chronic Sarcoidosis. Granulomas of the
nasal mucous membranes and the conjunctiva may occur.
An acute inflammatory skin disease marked by tender red nodules (erythema
nodosum) with fever and pain in the joints (arthralgias) is a frequent
manifestation of Sarcoidosis.
Liver (hepatic) granulomas are found in 70% of patients examined by liver
biopsy, even if they are asymptomatic and have normal liver function tests.
An enlarged liver (hepatomegaly) is noted in fewer than 20% of patients.
Progressive and severe hepatic dysfunction with enlarged or tortuous veins
(esophageal varices and portal vein hypertension) is rare.
Granulomatous inflammation of the vascular middle layer of the eye occurs
in 15% of cases; it is usually bilateral, and may result in severe loss of
vision from secondary glaucoma if untreated. Inflammation of the tissues
around the veins in the retina (retinal periphlebitis), tear gland
enlargement, conjunctival infiltrations, and dry inflammation of the cornea
(keratitis sicca) occasionally are present.
Myocardial involvement may cause angina, congestive heart failure, or
severe conduction abnormalities. Acute polyarthritis may be prominent.
Central nervous system (CNS) involvement can be of almost any type, but
cranial nerve palsies, especially facial paralysis, are most common. Diabetes
insipidus may also occur. An excess of calcium in the blood (hypercalcemia)
and an excess of calcium in the urine (hypercalciuria) may cause kidney
stones or precipitated calcium phosphate in kidney tubules (nephrocalcinosis)
with consequential renal failure. Prednisone therapy has reduced the
frequency and adverse effects of disordered calcium metabolism in patients
with Sarcoidosis.
Laboratory findings include the following characteristics. A decrease in
white blood cells (leukopenia) frequently is present. An abnormally high
globulin content of the blood (hyperglobulinemia) is common among black
people with Sarcoidosis. Elevated serum uric acid is not uncommon, but gout
is rare. Serum alkaline phosphatase may be elevated as a result of liver
involvement. Depression of delayed hypersensitivity is characteristic, but a
negative second-strength tuberculin reaction is useful in excluding a
complicating tuberculosis. Pulmonary function tests show restriction,
decreased compliance (yielding to pressure or force without disruption), and
impaired diffusing capacity of the lungs. Carbon dioxide retention is
uncommon since ventilation rarely is obstructed except in patients with
endobronchial disease or severe pulmonary fibrosis. Serial measurements of
pulmonary function are a guide to treatment and to the course of the disease.
Causes
The cause of Sarcoidosis is not known. A single provoking agent such as a
slow virus, or disordered defense reactions triggered by a variety of agents
may be responsible. Genetic factors may be important.
Recent studies indicate that M. tuberculosis may be involved in the
development of Sarcoidosis. It is felt that the disease may be caused by a
form of M. tuberculosis that has no cell wall and as a result is not
susceptible to the usual TB therapy.
Affected Population
Sarcoidosis occurs predominantly between the ages and 20 and 40 years. It is
most common among people of northern European ancestry and American Blacks.
Therapies: Standard
Treatment of Sarcoidosis is symptomatic and supportive. Corticosteroids
often relieve symptoms, improve physiologic disturbances and reduce x-ray
changes. Corticosteroid therapy should be given to suppress troublesome or
disabling symptoms such as difficulty breathing (dyspnea), severe pain in the
joints (arthralgia), and fever; it should be begun promptly if active eye
disease, respiratory failure, liver insufficiency, cardiac arrhythmia,
central nervous system involvement, or hypercalcemia is present. Prednisone
therapy is required by 1/2 of white patients and 2/3 of black patients with
this disorder. Prednisone is given orally, and the treatment may be
necessary for weeks, years, or, in some cases, indefinitely. Low maintenance
doses are surprisingly effective in controlling symptoms.
Clinical examinations, x-rays, and pulmonary function studies should be
made at frequent intervals when dosage is being reduced or medication is
terminated. Serious complications of corticosteroid therapy are infrequent
when low doses are used for treatment of Sarcoidosis. A tuberculostatic
antibacterial agent (isoniazid) therapy is indicated only for the few
patients given corticosteroids who have positive tuberculin skin tests.
Methotrexate (a folic acid antagonist) and chlorambucil (a cytotoxic
alkylating agent) occasionally are effective in treatment of Sarcoidosis, but
dramatic improvement with these medications is rare. They should be tried
only when corticosteroids fail to improve the condition or are
contraindicated.
Therapies: Investigational
Clinical trials are underway to study bronchoalveolar lavage in Interstitial
Lung Disease. Interested persons may wish to contact:
Gary W. Hunninghake, M.D.
Dept. of Internal Medicine
University of Iowa
Iowa City, IA 52242
(319) 356-4187
to see if further patients are needed for this research.
This disease entry is based upon medical information available through
May 1993. 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 Sarcoidosis, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
National Sarcoidosis Resource Center
P.O. Box 1593
Piscataway, NJ 08855-1593
(908) 699-0733
The National Arthritis and Musculoskeletal and Skin Diseases Information
Clearinghouse
Box AMS
Bethesda, MD 20892
(301) 495-4484
NIH/National Institute of Allergy and Infectious Diseases
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-5717
American Lung Association
1740 Broadway
New York, NY 10019
(212) 315-8700
References
THE MERCK MANUAL 15th ed: R. Berkow, et al: eds; Merck, Sharp & Dohme
Research Laboratories, 1987. P. 252.
CECIL TEXTBOOK OF MEDICINE, 18th ed.: James B. Wyngaarden, and Lloyd H.
Smith, Jr., Eds.: W. B. Saunders Co., 1988. Pp. 360-1, 451-7.