Rheumatic fever
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| Rheumatic fever | |
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| Rheumatic Mitral Valvulitis: Gross; an excellent example of acute rheumatic fever lesion along line of closure of mitral valve Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology | |
| ICD-10 | I00-I02 |
| ICD-9 | 390–392 |
| DiseasesDB | 11487 |
| MedlinePlus | 003940 |
| MeSH | D012213 |
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Rheumatic fever Microchapters |
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Diagnosis |
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Treatment |
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Case Studies |
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Rheumatic fever On the Web |
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American Roentgen Ray Society Images of Rheumatic fever |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Lance Christiansen, D.O.; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2]
Overview
Historical Perspective
Pathophysiology
Epidemiology and Demographics
Natural History, Complications and Prognosis
Diagnosis
Jones criteria | Physical Examination
General information
Streptococcus pyogenes is a complex microorganism and it causes many disease entities both from the suppurative aspect of Streptococcus pyogenes infections and from nonsuppurative, systemic, autoimmunological sequelae to the infections. The nonsuppurative, systemic, autoimmunological sequelae are inflammatory in nature and therefore all tissues and organs are affected, but certain organs are noted to be affected in an important and acute fashion: the heart, kidneys and brain.
Since the heart, kidneys and brain are vital organs, and their functional abnormalities causes obvious clinical abnormalities, and even death, to individuals, acute rheumatic abnormalities to the heart, kidneys and brain, that individuals experience, are medically-noted more frequently than acute rheumatic damage to other organs. The symptoms and signs of acute rheumatic fever, however, indicates that virtually all tissues, and therefore organs, of the body are affected: connective tissue (arthritis); lethargy, stupor, seizures, coma, post-disease fatigue, tics, chorea, chronic mental disturbances such as obsessive-compulsive behavior and PANDAS(rheumatic encephalomyelitis); acute rheumatic myocarditis,endocarditis, pericarditis and cardiac arrhythmias with the myocarditis and endocarditis causing cardiac failure and pulmonary edema (the heart); autoimmune pneumonitis (the lungs); rheumatic, vasculitic renal failure (kidneys); erythema marginatum, papulatum, miliary rash, purpura, petechiae, and scarlet fever rash (the skin); anemia, including aplastic anemia (bone marrow); autoimmune hepatitis (the liver); acute, peripheral, painful neuropathies (peripheral nerves); nausea, vomiting, diarrhea, crampy abdominal pain (gastrointestinal organs); other organs such as the pancreas, endocrine organs, and elements of the circulatory system are also affected. Since rheumatic fever is, basically and initially, an autoimmune inflammatory disorder, and the autoimmunological elements travel throughout the body via the circulatory system. Vascular elements, arteries, arterioles, capillaries, venules, veins and lymph vessels are attracked initially and in that way rheuamtic fever becomes a systemic disease process.
Information in modern texts has emphasized for many decades the affect of rheumatic fever on the heart, because its inflammatory, autoimmunological effects are exacerbated in that organ, and since the heart is a vital organ the clinical ramifications are easily noted. The reason that rheumatic autoimmunity is exacerbated in the heart's tissues is because the cyclic, physiological compression developed by the heart also causes an elevated vascular triple response of Lewis phenomenon within the heart's tissues. The triple response of Lewis phenomenon is usually thought to be a dermatological concept since it can be elicited by stroking the skin of most individuals. Those who have elevated Streptococcus pyogenic autoimmunity exhibit a very high-grade triple response of Lewis phenomenon dermatologically. Surpisingly, the triple response of Lewis phenomenon that all people exhibit indicates that all people have, at least, a low-level of rheuamtic autoimmunity (from personal research).
More important, perhaps, are the more chronic, inflammatory, autoimmunologial disease states, which have not been well appreciated in modern times, but which were understood, at least to some degree, during prior eras. Rheumatic fever, an acute, inflammatory, autoimmune disease state was, from the mid-1600's at least, when Sydemham discribed rheumatic fever reasonably accurately, until the early 1900's was termed, often, acute rheumatism or acute articular rheumatism. The chronic disease state of rheumatic, inflammatory autoimmunity was termed chronic rheumatism or simply, rheumatism. Galen, during the second century A.D. coined the word, rheumatismos, and it was first used in post-renaissance times by Guillaume Bailou (1538-1616)(Rheumatic Fever and Streptococcal Infections, cited above).
The chronic non-suppurative target-organ manifestions of rheumatic fever, rheumatic heart-valve disease, is not caused by acute rheumatic fever, but it is due to a reasonably elevated, chronic, inflammatory, rheumatic, autoimmune state, which is caused by repeated, or at times chronic, infections by virulent strains of Streptococcus pyogenes. Rheumatic heart-valve disease is common in American society, but there is no outcry from treating cardiologists, and cardiac surgeons, concerning the ongoing development of the high-grade, chronic, rheumatic state within individuals in the American population.
Sir William Osler, in his famous text, Osler's Principles and Practice of Medicine, Twelfth Edition (McCrea, T, D. Appleton-Century Company, 1935) indicates that rheumatic fever can exist in the typically understood classic high-grade presentation (some of the signs and symptoms thereof are listed in the Jones Criteria), as subacute rheumatic fever, and as rheumatic fever of the less than subacute variety. Osler indicates the following: "Subacute rheumatic fever represents a milder form of the disease, in which all the symptoms are less pronounced. The fever rarely rises abo e 101 deg. F.; fewer joints are involved; and the arthritis is less intense. The cases may drag on for weeks or months. It should not be forgotten that mild or subacute forms may be associated with endocarditis or pericarditis...in young children there may not be any pronounced arthritis or any arthritis at all, and the discovery of endocarditis often suggests the diagnosis. Endocariditis and myocarditis are the prominent features in children in whom the picture may be very variable...The onset may be so insidious that it can hardly be termed even subacute. Ill health without any evident cause, loss of weight, anorexia, fatigue, complaint of slight pains and fever with no apparent cause should suggest the possibility." As an example of the "...complaint of slight pains...", mentioned in the above quote, growing pains, that many younger people have, is a sign of mild, semi-chronic rheuamtic fiver.
Diagnosis of Rheumatic Fever The diagnosis of rheumatic fever has been historically difficult, but certain physicians, since the 1500's, understood, at least in part, the complex of signs and symptoms that indicate its existence. Thomas Sydenham discribed a case of severe rheumatic fever in the late 1600's and a similar discription is provided in the Encyclopedia Britanica's first edition published in 1771: "The rheumatism chiefly attacks persons in the flower of their age, after violent exercise, or a great heat of the body from any other cause, and then being too suddenly cooled; but spares neither men nor women, old nor young...It begins with chiliness and shivering, followed by inquietude and thirst. Which is preceded with spontaneous lassitude, a heaviness of the joints, and coldness of the extreme parts. When the fever appears, there is an inward heat, chiefly about the praecordia, attended with anxiety. The pulse is quick and strait, the appetite is lost, and the body is coftive. In a day or two, sometimes sooner, the patient feels a racking pain, sometimes in one joint, sometimes in another, but more frequently in the wrists, shoulders and knees; frequently shifting from place to place, and leaving redness and swelling in the part visited last. The pain is exasperated upon the least motion; it sometimes attacks the loins and the coxendix. When it seizes the loins, it is called the lumbago; and there is a most violent pain in the small of the back, which sometimes extends to the os sacrum, and is like fit of the gravel...it may continue for months or years, but not always with the same violence, but by fits. If it continues and increases, it may cause a stiff joint, which will scarce yield to any remedy." Further,"Its proximate cause seems to be the inflammation of the lymphatic arteries." And finally, "The chronic rheumatism is either the remains of a rheumatic fever, or a continuation of pains that proceeded at first from lesser but neglected colds."
The above quote is very important for it specifies that since the late 1700's physicians knew that a contagion caused acute rheuamtism, also termed, a rheumatic fever, but also that it caused chronic rheumatism. At times the word, rheumatism, was used as a general term for both acute rheumatism and chronic rheumatism since certain physicians from the time of Galen knew that a respiratory disease, which caused the development of "a defluxion of rheum", phlegm (Rheum means, "to flow or to flow down" in Greek), would cause a painful systemic malady in humankind. Galen, in fact, coined the word rheumatismos about 200 AD.
As rheumatic fever slowly, but progressively became less common after about 1850, as the advances of the industrial revolution were instituted into economically developed societies, especially after 1900, high-grade cases of rheumatic fever became less common in modernized parts of the world. Fewer physicians took care of patients with severe rheumatic fever so it slowly became difficult to diagnose, even in the late 1930's, by modern, western-educated physicians. One might think that physicians in modern countries have the least experience and therefore the least knowledge about rheumatic fever, however, they often have advanced knowledge about other aspects of medical science.
Sir William Osler indicates in his text, Osler's Principles and Practices of Medicine, Twelfth Edition, cited above, presents many clinical and epidemiological factors concerning rheumatic fever. All physicians dealing with patient sicknesses should read it completely, because modern texts, written by individuals who have never, or rarely, treated rheumatic fever patinets usually mimic information in prior texts. It does indicate that rheumatic fever is often a disease that exists commonly within family members (Streptococcus pyogenese is highly contagious), he indicates that it is due to a "hypersensitiveness" to...streptococci. "There is an initial lesion with more serious manifestations occuring elsewhere in the body...The initial lesion may be in the tonsils with resulting sensitization and subsequent attacks may be due to fresh infection or to areas of chronic infection (tonsils, sinuses)...A hypersensitive state once established, a chronic infection or a repeated slight infection may serve to prolong it and an acute infection may precipitate another attack."
Further, he indicates, "As a rule, the disease sets in abruptly, but it may be preceded by irregular pains in the joints, slight malaise, sore throat, and particularly by tonsillitis...A definite rigor is uncommon; more often there is a slight chilliness. The fever rises quickly, and one or more of the joints may become painful. Within twenty-four hours from the onset the disease is fully manifest. The temperature range is from 102 deg. to 104 deg. The pulse is frequent, soft, and usually above 100. The tongue is moist and covered wiht white fur. There are...loss of appetite, thirst, constipation, and scanty, highly acid urine. In the majority of cases there are profuse, acid sweats, of a paeculiar sour odor. Sudaminal and miliary vesicles are abundant, the latter being surrounded by a minute ring of hyperemia."
In addition Dr. Osler Indicates, "The affected joints become are painful to move, soon become swollen and hot, and present a reddish flush. The order of frequency of involvement of the joints in our series was knee, ankle, shoulder, wrist, elbow, hip, hand, foot. The joints are not attacked together, but successively. The amount of swelling is variable. Extensive effusion into a joint is ratre, and much of the enlargement is due to the infiltration of the periarticular tissues with serum."
Importantly, Dr. Osler indicates, "Perhaps no disease is more painful; the inability to change posture without agonizing pain, the drenching sweats, the prostration and helplessness, combine to make it a most distressing affection...In children there may be no arthritis whatever or if present only in a mild form...The blood is profoundly altered and there is no acute febrile disease in which an anemia occurs with greater rapidity...The average leucocyte count in our cases was about 12,000 per c. mm. Epistaxis is not uncommon...With a high fever a murmur may often be heard at the apex region...Febrile albuminuria is not uncommon. Hematuria occurs occassionally."
It is somewhat common for individuals who have chronic pain after spinal surgery to die, compared to age-matched groups. The reason they die more frequently, even at a young age, is because such individuals have relatively high-grade, chronic rheumatism, from, perhaps, chronic Streptococcus pyogenese stimulation, and so they can develop recurrent rheumatic fever somewhat frequently or they develop rheumatic autonomic neuropathies: cardiac arrhythmias, and so they die from those maladies and not from taking opiate analgesics that most of them need to fend off the chronic neurological pain from which they suffer.
Treatment
Medical therapy | Primary Prevention | Secondary Prevention
Case Study
Click here for a case study in rheumatic fever.
References
External links
- Rheumatic fever information from Seattle Children's Hospital Heart Center
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