$Unique_ID{BRK04072} $Pretitle{} $Title{Osteonecrosis} $Subject{Osteonecrosis Avascular Necrosis Vasculitis Osteopetrosis Rheumatoid Arthritis Lupus Erytheomatosus Legg-Calve-Perthes Syndrome Sickle Cell Disease Gaucher's Disease Polycythemia Vera Caisson Disease} $Volume{} $Log{} Copyright (C) 1990, 1992 National Organization for Rare Disorders, Inc. 756: Osteonecrosis ** IMPORTANT ** It is possible that the main title of the article (Osteonecrosis) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Avascular Necrosis Information on the following diseases can be found in the Related Disorders section of this report: Vasculitis Osteopetrosis Rheumatoid Arthritis Lupus Erytheomatosus Legg-Calve-Perthes Syndrome Sickle Cell Disease Gaucher's Disease Polycythemia Vera Caisson Disease 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. Osteonecrosis is the destruction of a bone (necrosis) often due to an inadequate supply of blood to a bone. It most commonly affects the joints and bones of the hips, knees and shoulder. It often occurs as a result of bone injuries or in conjunction with other diseases and conditions. Symptoms Osteonecrosis is a prevalent slowly progressive prevalent disease frequently caused by a reduction of blood flow to an area that involves bones and joints causing the bones to crack and eventually collapse. This condition usually occurs as the result of other diseases, injuries or conditions. Pain is the primary symptom. It is a chronic and mild pain, usually occurring when standing, walking or lifting. The pain becomes worse when weight bearing activities exert pressure on the bones or joints. The pain may progress, eventually occurring while at rest or even disturbing sleep. Other symptoms include muscle spasms, joint stiffness and limitation of motion. Osteonecrosis most commonly affects the head of the femoral bone but may also involve the head of the humerus bone, the round protruding area at the end of the femur bone (condyles), the shin bone (distal tibia) and ankle (talus). Causes Osteonecrosis is the death of bone tissue associated with various diseases. A common cause is trauma that can cause a dislocation or fracture of the neck of the femur bone. Bones may also be affected by the use of certain drugs such as corticosteroids (glucocorticoids), or radiation and chemotherapy used in treating cancer patients. Osteonecrosis may also be a complication of kidney transplantation, sickle cell disease, alcoholism and other disorders. Affected Population Osteonecrosis is a common progressive disorder that can occur at any age, but is more frequently seen in people between 30 and 60 years of age. Osteonecrosis of the hip is slightly more common in men while Osteonecrosis that affects the knees is seen three times more often in women. It is also more common in those people with rheumatic diseases (e.g., rheumatoid arthritis, systemic lupus erythematosus), steroid users (such as cortisone), alcoholics, diabetics, and skin divers who have experienced a rapid reduction of air pressure (bends). Related Disorders Symptoms of the following disorder may be similar to those of Osteonecrosis. Comparisons may be useful for a differential diagnosis: Osteopetrosis is a combination of several rare genetically caused symptoms grouped together as one disorder. It can be inherited as either a dominant or recessive trait and is marked by increased bone density, brittle bones, and in some cases skeletal abnormalities. Although symptoms may not initially be apparent to people with mild forms of this disorder, trivial injuries may cause bone fractures due to abnormalities of the bone. The dominantly transmitted form is milder than the recessive form and may not be diagnosed until adolescence or adulthood when symptoms first appear. More serious complications occur in the recessive form which may be diagnosed from examination of skeletal x-rays during infancy or childhood. (For more information on this disorder, choose "Osteopetrosis " as your search term in the Rare Disease Database). The following disorders may be associated with the development of Osteonecrosis. They are not necessary for a differential diagnosis: Vasculitis is a common disorder characterized by an inflammation of the blood vessel walls. This inflammation causes a narrowing of the inside of the vessel and can obstruct the flow of blood to the tissues (ischemia). The lack of blood may cause damage to the tissues (necrosis), possible formation of blood clots (thrombosis), or a weakening or ballooning which can possibly cause a rupture of the vessel wall (aneurysm). Osteonecrosis can occur in people with vasculitis when blood flow has been obstructed to the bone or joint. (For more information on this disorder, choose "Vasculitis" as your search term in the Rare Disease Database.) Rheumatoid Arthritis is an inflammatory autoimmune disease in which the body's natural defenses against foreign agents (antibodies & lymphocytes) attack healthy joints. This disorder is characterized by a lack of appetite (anorexia), fatigue, painful and deformed joints, early morning stiffness chiefly in the hands, knees, feet, jaw, and spine. People with Arthritis are at increased risk of also developing Osteonecrosis. (For more information on this disorder, choose "Arthritis" as your search term in the Rare Disease Database). Lupus (also known as SLE or Systemic Lupus Erythematosus) is an inflammatory connective tissue disease that can affect many parts of the body including the joints, skin and internal organs. Lupus is a disease of the body's immune system, most often striking young women between the ages of 15 and 35 years. People who have Lupus are also at increased risk of developing Osteonecrosis. (For more information on this disorder, choose "Lupus" as your search term in the Rare Disease Database). Legg-Calve-Perthes Syndrome is a rare disease affecting the hip joint. Abnormalities in bone growth early in life may result in permanent deformity of the hip joint several years later. Osteonecrosis of the femoral head is often seen in those people with Legg-Calve-Perthes Disease. (For more information on his disorder, choose "Legg-Calve-Perthes" as your search term in the Rare Disease Database.) Gaucher's Disease is an inherited disease of lipid metabolism caused by the failure to produce the enzyme glucocerebrosidase. It is the most common of the 14 known lipid storage disorders which includes Tay-Sachs, Fabry's Disease, and Niemann-Pick Disease. There are three types of Gaucher's Disease Type I, II and III. All three are characterized by the presence of Gaucher (lipid-laden) cells in the bone marrow and other organs such as the spleen and liver. Bone deterioration (Osteonecrosis) is a major symptom of this disease and can affect any part of the body. (For more information on this disorder, choose "Gaucher" as your search term in the Rare Disease Database.) Sickle Cell Disease is an inherited blood disease. Symptomatic of this disease are the "crisis periods" which often occur in conjunction with other infections. It is characterized by joint pain (arthralgia), fever, severe abdominal pain, vomiting, and damage to the head of the femur bone (osteonecrosis). (For more information on this disorder, choose "Sickle Cell" as your search term in the Rare Disease Database). Polycythemia Vera is a chronic proliferative disorder of the bone marrow. It is characterized by an increase in the number of red blood cells (erythrocytosis) and hemoglobin concentration in the blood. Osteonecrosis can occur in Polycythemia Vera. (For more information on this disorder, choose "Polycythemia Vera" as your search term in the Rare Disease Database.) Caisson Disease, also called "Decompression Sickness" or the "Bends", is a disorder caused by the formation of nitrogen bubbles in the tissues and blood. This occurs from a very rapid reduction of air pressure after rising quickly from deep water with high atmospheric pressure, to normal air pressure. It is characterized by painful joints, bone deterioration (Osteonecrosis), chest tightness, giddiness, abdominal pain, vomiting and visual difficulties. In some with this disorder there may also be convulsions and paralysis. Therapies: Standard Treatment of Osteonecrosis consists of diagnosing, treating or eliminating the underlying cause. Bones damaged or weakened by this disorder will usually heal and regenerate with appropriate treatment. Limiting or avoiding alcohol, weight bearing activities, standing or walking may help in the recovery process. Bed rest and reducing stress to the affected area are helpful. The pain associated with this disorder can be relieved with aspirin or non-steroidal anti-inflammatory drugs such as ibuprofen. Warm baths, heating pads and electric blankets may also be helpful in relieving the muscle spasms and pain associated with Osteonecrosis. X-Ray's can be helpful in diagnosing Osteonecrosis and determining the extent of bone damage. Surgery may be necessary when there is a dislocation, fracture or if the bone has collapsed. Other treatment is symptomatic and supportive. The most often used method of treatment for advanced Osteonecrosis of either the knee or hip is replacement of the affected area with a prosthetic joint. Therapies: Investigational This disease entry is based upon medical information available through June 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 Osteonecrosis, please contact; National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 Arthritis Foundation 1314 Spring Street, NW Atlanta, GA 30309 (404) 872-7100 NIH/National Arthritis, Musculoskeletal and Skin Diseases Information Clearinghouse Box AMS Bethesda, MD 20892 (301) 495-4484 References TEXTBOOK OF MEDICINE, 18th ED.: James B. Wynngaarden, M.D., et al.; ed.-in- chief; W.B. Saunders Co. 1988. Pp. 1517 OSTEONECROSIS OF THE HIP IN THE SICKLE-CELL DISEASES. TREATMENT AND COMPLICATIONS. G. Hanker, et al.; J BONE SURG [AM] (April 1988, issue 70 (4)). Pp. 499-506. INFLUENCE OF ALCOHOL INTAKE, CIGARETTE SMOKING, AND OCCUPATIONAL STATUS ON IDIOPATHIC OSTEONECROSIS OF THE FEMORAL HEAD. K. Matsuo, et al.; CLIN ORTHOP (September 1988, (234)). Pp. 115-123. OSTEONECROSIS OF THE FEMORAL HEAD. PATHOGENIS AND LONG-TERM RESULTS OF TREATMENT. M. Meyers; CLIN ORTHOP (June 1988 (231)). Pp. 51-61. SURVEY OF THE LONG TERM INCIDENCE OF OSTEONECROSIS OF THE HIP AND ADVERSE MEDICAL EVENTS IN RHEUMATOID ARTHRITIS AFTER HIGH DOSE INTRAVENOUS METHYLPREDNISOLONE. I. Williams, et al.; ANN RHEUM DIS (November 1988, issue 47(11)). Pp. 930-933.