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rheumatic fever(ro͞omăt`ĭk), systemic inflammatory disease, extremely variable in its manifestation, severity, duration, and aftereffects. It is frequently followed by serious heart disease, especially when there are repeated attacks. Rheumatic fever usually affects children. It is closely related to a preceding streptococcal infection (e.g., streptococcal tonsillitis or pharyngitis). Some of its symptoms are tenderness and inflammation about the joints, fever, jerky movements, nodules under the skin, and skin rash. If inflammation of the heart, or myocarditis, is mild, there is no permanent heart damage, but if the valves of the heart become inflamed, they may become scarred and deformed, permanently impairing their function. Such heart damage can sometimes be corrected by surgery.
Treatment of rheumatic fever is with penicillin, salicylates, and steroids; extended rest is usually necessary. Rheumatic fever may be prevented by prompt treatment of all streptococcal infections. Cardiac damage may possibly be avoided if prophylactic measures are taken after a first attack of rheumatic fever, i.e., long-term maintenance doses of antibiotics, to discourage streptococcal infections and recurrences of rheumatic fever. Rheumatic fever has declined in incidence in the industrialized countries, but has increased in prevalence in the Third World. See also streptococcusstreptococcus
, any of a group of gram-positive bacteria, genus Streptococcus, some of which cause disease. Streptococci are spherical and divide by fission, but they remain attached and so grow in beadlike chains.
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An illness that follows an upper respiratory infection with the group A streptococcus (Streptococcus pyogenes) and is characterized by inflammation of the joints (arthritis) and the heart (carditis). Arthritis typically involves multiple joints and may migrate from one joint to another. The carditis may involve the outer lining of the heart, the heart muscle itself, or the inner lining of the heart. A minority of affected individuals also develop a rash (erythema marginatum), nodules under the skin, or Sydenham's chorea (a neurologic disorder characterized by involuntary, uncoordinated movements of the legs, arms, and face). Damage to heart valves may be permanent and progressive, leading to severe disability or death from rheumatic heart disease years after the initial attack. The disease occurs an average of 19 days after the infection and is thought to be the result of an abnormal immunologic reaction to the group A streptococcus. Initial attacks of rheumatic fever generally occur among individuals aged 5 to 15. Those who have had one attack are highly susceptible to recurrences after future streptococcal infections.
Initial attacks of rheumatic fever can be prevented by treatment of strep throat with penicillin for at least 10 days. Patients who have had an episode of rheumatic fever should continue taking antibiotics for many years to prevent group A streptococcal infections that may trigger a recurrence of rheumatic fever. See Streptococcus
(also called acute articular rheumatism), an infectious allergic disease characterized by widespread inflammation of connective tissue.
In man. In man, rheumatic fever is localized chiefly in the cardiovascular system. The disease usually develops in children and adolescents between seven and 15 years of age. It generally develops in winter but is independent of climatic and geographic zones. Two factors are decisive: a preceding infection such as sore throat, pharyngitis, or scarlet fever, caused by group A beta-hemolytic streptococci, and a special predisposition in the form of hyperreaction to the infection. Rheumatic fever thus occurs in only 1–3 percent of persons who have been affected by sore throat. Streptococcal infection is the cause of rheumatic fever and of acute forms of the disease. Familial rheumatism may be caused by prolonged exposure to an intensive streptococcal environment and to such unfavorable living conditions as overcrowding and the use of common dishes and linens. There is also evidence for a hereditary polygenic predisposition to the disease.
The most important factor in the pathogenesis of rheumatic fever is the reduction of immunity to streptococci. This impaired immunity is manifested by excess production of antibodies (circulating in the blood and bound to lymphocytes in the tissues) to the streptococci’s antigens. Autoantibodies are also produced to such tissues of the body as collagen. Since there is a structural similarity between the antigens of streptococci and of the heart, antistreptococcic antibodies may react with the autoantigens of cardiac tissue, for example, of the myocardium: these antibodies become lodged in the cardiac tissue and injure the heart. Other mechanisms occurring during the development of rheumatic fever are disturbance of the microcirculation with increased permeability of the capillary wall and the liberation of lysosomal enzymes from injured cells with resulting inflammation of vessels and connective tissue.
The involvement of connective tissue and vascular walls typical of rheumatic fever occurs in phases. In the phase of mucoid swelling, the processes of disorganization affect the interstitial substance and collagen fibers and are completely reversible if treated promptly. The phase of fibrinoid changes is an indication of more extensive destruction of connective tissue and causes necrosis of the tissue. In the next phase, cellular reactions arise around foci of the disorganization of connective tissue and gradually produce rheumatic nodules specific to rheumatic fever, the Aschoff bodies. Finally, there is sclerosis, or cicatrization, resulting from regression of the rheumatic granulomas, a process that lasts three to four months.
The onset of rheumatic fever is generally acute, with a general deterioration of health, fever rising to 38°-39°C, the sudden occurrence of joint pain, and swelling of the joints, which is usually temporary. If polyarthritis develops, there is often reddening and dysfunction of the joints owing to inflammation of the synovial membranes and periarticular tissues. As a rule, the large joints are affected: the knee, the talocrural, ulnar, and radiocarpal joints. Polyarthritis does not become chronic and disappears without a trace.
Within a week to ten days, symptoms of cardiac involvement (rheumatic carditis) appear. These include pain, palpitation of the heart, arrhythmia, dyspnea upon slight physical exertion, and pathological changes revealed by direct, instrumental, and laboratory examination. Involvement of the nervous system is usually manifested in children by chorea, a disorder characterized by emotional instability, muscular hypotonia, and involuntary movements of the arms, trunk, and facial muscles. In adults, involvement of the nervous system is manifested by rheumatic encephalitis and meningitis.
In rheumatic fever there is also involvement of vessels in different parts of the body (rheumatic vasculitis). Involvement of the skin takes the form of erythema annulare, erythema nodosum, and nodules. Also subject to involvement are the serous membranes, eyes, lungs, and kidneys. The erythrocyte sedimentation rate accelerates to 40–50 mm/hr, and the white blood cell count rises to 10,000–13,000 in 1 mm3. The content of such antistreptococcic antibodies as antistreptolysin, antistreptokinase, and antistreptococcic hyaluronidase increases, and C-reactive protein, hyperfibrinogenemia, and dysproteinemia may be detected.
The course of rheumatic fever takes several forms. Complete recovery is the rule if the course is acute or subacute and therapy is initiated early. A latent and protracted course of rheumatic carditis may result in organic heart diseases. Rheumatic fever is more severe in children than in adults: inflammatory changes are more pronounced, and the development of heart diseases are more common.
Rheumatic fever is treated by bed rest and a diet low in salt and fluids. The diet should contain sufficient proteins, fruits, vegetables, and such potassium-rich foods as dried apricots, potatoes, and buckwheat groats. Antibiotics and corticosteroids are used in combination with aspirin, Aminopyrine, butadion, and indomethacin or other antirheumatic agents. Cardiotherapy is used when needed. Foci of chronic streptococcal infection are removed by means of tonsillectomy, the filling of carious teeth, and the treatment of sinusitis and pharyngitis. Rehabilitation in a local rheumatologic sanatorium is desirable. Children with rheumatic fever should stay in a sanatorium for three to six months, until the disease is completely inactive. After adult patients have been discharged from the hospital or children from the sanatorium, they should be registered in a dispensary program in a cardiologic and rheumatologic center for three to five years or more. The length of this period depends on whether there is recurrence of the disease and whether foci of chronic infection exist. During this time, patients are given maintenance anti-inflammatory treatment throughout the year or in spring and fall. Bicillin is administered to prevent intensification of the disease.
REFERENCESTalalaev, V. T. Ostryi revmatizm, 2nd ed. Moscow, 1932.
Mikheev, V. V. Neirorevmatizm. Moscow, 1960.
Ioffe, V. I. Immunologiia revmatizma. Leningrad, 1962.
Liampert, I. M. Etiologiia, immunologiia i immunopatologiia revmatizma. Moscow, 1972.
Nesterov, A. I. Revmatizm. Moscow, 1973.
Aschoff, L. “Zur Myocarditisfrage.” In Verhandlungen der deutschen pathologischen Gesellschaft, 1904, book 2. Jena, 1905.
Hench, P. In the collection The Rheumatic Diseases. Philadelphia-London, 1952.
Kaplan, M. N. “Immunologic Relation of Streptococcal and Tissue Antigens.” Journal of Immunology, 1963, vol. 90, no. 4.
V. A. NASONOVA
In animals. Among animals, rheumatic fever affects mainly horses, cattle, asses, swine, and dogs. Chiefly involved are the muscles and joints, with accompanying swelling, tenderness, lameness, fever, and accelerated pulse and respiration rates. The affected animals are kept in a warm dry place, massaged with mildly stimulating and pain-relieving ointments, and wrapped up warmly. They also receive diathermy, ionotherapy, ultrahigh-frequency treatment of the affected joints and muscles, and such drugs as salicylates.
REFERENCEChastnaia patologiia i terapiia domashnikh zhivotnykh, vol. 2, book 4. Moscow, 1964. (Translated from German.)
N. M. PREOBRAZHENSKII