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innovative therapies, that were unimaginable just a few years ago.

Ongoing NIAMS epidemiological investigation of Native Americans in Alaska and Siberian Eskimos--populations that have a high prevalence of spondylitis--promises to shed light on the roles of genetic factors and environmental triggers, such as bacterial infections.

Systemic lupus erythematosus (lupus) is an immune-mediated inflammatory disease that afflicts numerous Americans, largely young women, especially black women of child bearing age. The disease affects the skin, joints, kidneys, brain, and other organs. Recently, a new antibody was identified that is clearly associated with psychiatric disorders due to lupus; this antibody will make better diagnosis possible.

The Institute has set up a task force to study lupus in high-risk populations.

With advice from the task force, NIAMS is developing instructive kits that will help health care providers and community organizations conduct health promotion programs targeted to young black women. The Institute also plans to sponsor an international workshop on systemic lupus erythematosus to assess current knowledge and develop directions for future research.

Scleroderma is a serious disorder of connective tissue and the fine blood

vessels. Besides damaging the skin, muscles, and joints, the disease may also affect internal organs, such as the kidneys, heart, and lungs, making it lifethreatening in some people. Women are affected three times more often than men. The first symptom of scleroderma is usually Raynaud's phenomenon, wherein blood vessels in the fingers narrow as the result of vasospasm, causing the hands to become pale and cold. There is also pathologic evidence of vascular disease seen under the microscope. Accordingly, there is keen interest in exploring vascular abnormalities that may contribute to the etiology of the disease. NIAMS will issue a program announcement to stimulate more research on

causal mechanisms in scleroderma.

Over 100 different conditions are included among the heritable connective tissue disorders, all of which stem from abnormalities of connective tissue, the scaffolding that girds bone, skin, blood vessels, and the protective coverings of internal organs. Although the disorders are uncommon, they are

tremendously disabling.

In April 1990, NIAMS cosponsored a multidisciplinary

scientific workshop on heritable connective tissue disorders.


participants made it clear that sophisticated research methods are enabling

scientists to unravel the complex biology of connective tissue and to uncover

the several defects that cause the disorders.

The Institute is encouraging

additional research in this area, and is exploring the possibility of a patient

registry to facilitate progress against these diseases.

Reducing the risk of sports Injury is important to millions of Americans

who participate in exercise programs and athletics.

The Institute's mission

includes responsibility for research on exercise physiology and sports


Basic facts about how muscles function at the molecular level are

now being discovered.

Recent research has revealed a mechanism by which a

serious knee injury can destroy a joint.

In April 1991, the Institute,


concert with the National Advisory Board, will hold a workshop on scholastic

sports injuries to monitor the causes of athletic injuries and identify ways to

prevent them.

We are continuing to make progress in research on the numerous skin

diseases that affect so many Americans.

In epidermolysis bullosa, a

devastating inherited blistering disease that affects the skin and mucous

membranes, researchers have found that the anchoring fibrils-- which link the

outer layer of the skin to the inner layer--are conspicuously absent or


Recent investigations have also shown that type VII collagen, the

major structural protein of anchoring fibrils, can be broken down by both

collagenase and gelatinase.

Other research, utilizing patients assembled by

the National Epidermolysis Bullosa Registry, suggests that abnormal synthesis

of type VII collagen be responsible for the reduced number of anchoring


Alopecia areata is a disease of the hair follicle that results in patchy

or total loss of hair.

The psychological impact of the disease can be

debilitating, especially in young people.

In October 1990, NIAMS and the

National Alopecia Areata Foundation sponsored a 2-day research workshop on the

Experts in a wide variety of areas were convened for an open exchange factors controlling hair growth, autoimmune aspects of the disease,


of information on clinical and histopathologic features of alopecia areata,

pharmacologic interventions, and animal models.

A new anatomic site that

controls hair growth has been discovered, and the ability to grow hair in a

test tube has been achieved.

These findings open new avenues of research that

will be pursued.

Psoriasis is a common chronic skin disease characterized by scaling and

redness produced by skin cells that divide rapidly. For many years, psoriasis

was treated with preparations of tars and chemotherapeutic agents, such as

me thotrexate.

Recently, clinical studies established the efficacy of

cyclosporine A, an immuno-suppressive drug in the treatment of psoriasis.


turn, this has led investigators to explore the role of immune-mediated factors

in causing the disease and to search for alternative drugs that cause fever

side effects than does cyclosporine A.

The NIAMS is encouraging further

exploration of molecular, immunologic, and pharmacologic aspects of psoriasis.

Significant strides have been made in the Institute's Intramural Program.

This past year two new laboratories were established to expand the scope of

research: the Laboratory of Skin Biology and the Laboratory of Structural

Biology Research,

Fundamental studies of proteins responsible for naturation

of the epidermis, the skin's outer layer, are the focus of research in the

Laboratory of Skin Biology.

In the Laboratory of Structural Biology Research,

new sophisticated instrumentation is facilitating analysis of extremely small

structures, including membrane-associated components of the AIDS virus.

In the area of orthopaedic research, Intramural scientists have identified

the various phases through which an injured bone must pass to heal properly.

They have detected the presence of a specific natural protein, transforming

growth factor-beta (TGF-beta), between and around the ends of fractured bone;

and determined that TGF-beta not only initiates a repair response, but also

controls the rate of fracture healing.

Another ground-breaking accomplishment achieved by intramural scientists

was the development of an animal model for eosinophilia-myalgia syndrome (EMS),

a new epidemic inflammatory disease associated with ingestion of contaninated

L-tryptophan. Symptoms range from those of the flu, to severe rashes and

permanent nerve and muscle damage.

The new animal model provides the first

direct, experimental evidence linking EMS to contaminated batches of the

dietary supplement L-tryptophan.

Collaborations between NIAMS and the Department of Defense continue in studies of skin manifestations in human immunodeficiency virus (HIV)-infected individuals. Establishment of a new interagency agreement will significantly increase the rate of accrual of HIV-positive subjects into the studies to give us an earlier estimate of the prevalence of skin disorders and accelerate the pace of this research.

Mr. Chairman, the budget request for NIAMS for fiscal year 1992 is $204,797,000. I will be pleased to answer any questions you may have.


July 25, 1919, Boston, Massachusetts


Education: llarvard University, A.B., 1941; Yale University Graduate
School, Ph.D., 1945; Yale University School of Medicine, M.D., 1949.
Professional History: 1949-50, Intern, Medicine, The Johns Hopkins
Hospital, Baltimore, Maryland. 1950-51, Ayerst, McKenna and Harrison
Fellow in Endocrinology, The Johns Hopkins University School of Medicine.
1951-52, Vernon D. Lynch Fellow in Internal Medicine, The Johns Hopkins
University School of Medicine. 1952-53, Assistant Resident, The Johns
Hopkins Hospital. 1954-present, Physician, The Johns Hopkins Hospital.
1955-75, Director, Connective Tissue Division, Department of Medicine, The
Johns Hopkins University School of Medicine. 1960-66, Physician-in-Charge,
Arthritis Clinic, The Johns Hopkins Hospital. 1956-67, Physician-in-
Charge, Connective Tissue Clinic, The Johns Hopkins Hospital.
Physician-in-Chief, Division of Chronic and Community Medicine, The
Baltimore City Hospitals, Baltimore, Maryland. 1970-73, Associate
Professor of Medical Care and Hospitals, The Johns Hopkins University
School of Hygiene and Public Health. 1964-present, Associate Professor of
Medicine, The Johns Hopkins University School of Medicine. 1976-80, Acting
Associate Director for Arthritis, Musculoskeletal, and Skin Diseases,
National Institute of Arthritis, Metabolism, and Digestive Diseases. 1980-
83, Associate Director, Arthritis, Musculoskeletal and Skin Diseases,
National Institute of Arthritis, Diabetes, and Digestive and Kidney
Diseases. 1983-1986, Director, Division of Arthritis, Musculoskeletal, and
Skin Diseases, National Institute of Arthritis, Diabetes, and Digestive and
Kidney Diseases. 1986, Acting Director, National Institute of Arthritis
and Musculoskeletal and Skin Diseases. 1987-present, Director, National
Institute of Arthritis and Musculoskeletal and Skin Diseases.

Professional Organizations: American Association for the Advancement of Science, American College of Physicians, American Federation for Clinical Research, American Rheumatism Association (President, 1974-75), American Society for Bone and Mineral Research, The Arthritis Foundation, Brazilian Society of Rheumatology, Carla Curzio Neapolitan Society, Lupus Foundation of America, New York Academy of Science, Pan American League Against Rheumatism (President, 1982-86), Society for Clinical Trials, Sydenham Society, Society for Investigative Dermatology.

Honors Awards: Senior Investigator Award, The Arthritis Foundation, 195762. Pemberton Memorial Lecture, 1963. Fellow, American College of Physicians, 1967. Wallace R. Graham Memorial Lecture: Toronto, 1973. The Heberden Medal for Research in the Rheumatic Diseases, Heberden Society, London, 1975. Distinguished Service Award, The Arthritis Foundation, 1979. Honorary Member, Brazilian Society of Rheumatology, 1980. Honorary Member, Chilean Society of Rheumatology, 1981. Knowles Lecture: San Francisco, 1982. Honorary Member, Costa Rican Association of Rheumatology, 1984. Honorary Member, Australian Rheumatism Association, 1985. The Public Health Service Superior Service Award, 1985. National Lupus Hall of Fame, 1986. Honorary Member, Swiss Society of Rheumatology, 1987. Elected Master, American Rheumatism Association, 1987.


Senator HARKIN. Thank you very much, Dr. Shulman.
How soon are we going to have this national plan, did you say?

I'm sorry.

Dr. SHULMAN. It is going to be before the next hearings.
Senator HARKIN. Good.

Well, I had a question on osteoporosis, but you covered that. And lyme disease-you covered that quite well. And lupus.

Just a moment. [Pause.] My apologies. I have to run over and cast another vote right now. My apologies.

(A brief recess was taken.)

QUESTIONS SUBMITTED BY THE SUBCOMMITTEE Senator HARKIN. Thank you very much. There will be some additional questions which will be submitted for your response in the record.

[The following questions were not asked at the hearing, but were submitted to the Institute for response subsequent to the hearing:)

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