WordNet
- a rise in the temperature of the body; frequently a symptom of infection (同)febrility, febricity, pyrexia, feverishness
- intense nervous anticipation; "in a fever of resentment"
- a person suffering with rheumatism
- highly excited; "a fevered imagination"
PrepTutorEJDIC
- radio frequency無線周波数
- 〈U〉《しばしばa fever》(身体の異常な)『熱』,発熱 / 〈U〉『熱病』 / 〈U〉〈C〉(…に対する)『興奮』,熱狂《+『for』+『名』》
- リューマチの / リューマチにかがった / リューマチ患者
- (比較変化なし)《名詞の前にのみ用いて》熱のある,熱病にかかった / 熱にうかされたような,興奮した / (憎しみなどが)異常に強い
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2017/08/27 04:41:38」(JST)
wiki en
| Rheumatic fever | |
|---|---|
| Synonyms | Acute rheumatic fever (ARF) |
| Streptococcus pyogenes bacteria (Pappenheim's stain) the trigger for rheumatic fever. | |
| Specialty | Cardiology |
| Symptoms | Fever, multiple painful joints, involuntary muscle movements, erythema marginatum[1] |
| Complications | Rheumatic heart disease, heart failure, atrial fibrillation, infection of the valves[1] |
| Usual onset | 2–4 weeks after a streptococcal throat infection, age 5-14 years[2] |
| Causes | Autoimmune disease triggered by Streptococcus pyogenes[1] |
| Risk factors | Genetics, malnutrition, poverty[1] |
| Diagnostic method | Based on symptoms and infection history[3] |
| Prevention | Antibiotics for strep throat, improved sanitation[4][1] |
| Treatment | Prolonged periods of antibiotics, valve replacement surgery, valve repair[1] |
| Frequency | 325,000 children a year[1] |
| Deaths | 319,400 (2015)[5] |
|
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Rheumatic fever (RF) is an inflammatory disease that can involve the heart, joints, skin, and brain.[1] The disease typically develops two to four weeks after a streptococcal throat infection.[2] Signs and symptoms include fever, multiple painful joints, involuntary muscle movements, and occasionally a characteristic non-itchy rash known as erythema marginatum. The heart is involved in about half of cases. Damage to the heart valves, known as rheumatic heart disease (RHD), usually occurs after repeated attacks but can sometimes occur after one. The damaged valves may result in heart failure, atrial fibrillation and infection of the valves.[1]
Rheumatic fever may occur following an infection of the throat by the bacterium Streptococcus pyogenes.[1] If the infection is untreated rheumatic fever can occur in up to three percent of people.[6] The underlying mechanism is believed to involve the production of antibodies against a person's own tissues. Due to their genetics, some people are more likely to get the disease when exposed to the bacteria than others. Other risk factors include malnutrition and poverty.[1] Diagnosis of RF is often based on the presence of signs and symptoms in combination with evidence of a recent streptococcal infection.[3]
Treating people who have strep throat with antibiotics, such as penicillin, decreases the risk of developing rheumatic fever.[4] In order to avoid antibiotic misuse this often involves testing people with sore throats for the infection, which may not be available in the developing world. Other preventive measures include improved sanitation. In those with rheumatic fever and rheumatic heart disease, prolonged periods of antibiotics are sometimes recommended. Gradual return to normal activities may occur following an attack. Once RHD develops, treatment is more difficult. Occasionally valve replacement surgery or valve repair is required. Otherwise complications are treated as per normal.[1]
Rheumatic fever occurs in about 325,000 children each year and about 33.4 million people currently have rheumatic heart disease.[1][7] Those who develop RF are most often between the ages of 5 and 14,[1] with 20% of first-time attacks occurring in adults.[8] The disease is most common in the developing world and among indigenous peoples in the developed world.[1] In 2015 it resulted in 319,400 deaths down from 374,000 deaths in 1990.[5][9] Most deaths occur in the developing world where as many as 12.5% of people affected may die each year.[1] Descriptions of the condition are believed to date back to at least the 5th century BCE in the writings of Hippocrates.[10] The disease is so named because its symptoms are similar to those of some rheumatic disorders.[11]
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Video explanation
Contents
- 1 Signs and symptoms
- 2 Pathophysiology
- 2.1 Rheumatic heart disease
- 3 Diagnosis
- 3.1 Major criteria
- 3.2 Minor criteria
- 4 Prevention
- 5 Treatment
- 5.1 Vaccine
- 5.2 Infection
- 5.3 Inflammation
- 5.4 Heart failure
- 6 Epidemiology
- 7 See also
- 8 References
- 9 External links
Signs and symptoms
A culture positive case of streptococcal pharyngitis with typical tonsillar exudate in a 16-year-old.
The disease typically develops two to four weeks after a throat infection.[2] Symptoms include: fever, painful joints with those joints affected changing with time, involuntary muscle movements, and occasionally a characteristic non-itchy rash known as erythema marginatum. The heart is involved in about half of cases. Damage to the heart valves usually occurs only after multiple attacks but may occasionally occur after a single case of RF. The damaged valves may result in heart failure and also increase the risk of atrial fibrillation and infection of the valves.[1]
Pathophysiology
Rheumatic fever is a systemic disease affecting the connective tissue around arterioles, and can occur after an untreated strep throat infection, specifically due to group A streptococcus (GAS), Streptococcus pyogenes. It is believed to be caused by antibody cross-reactivity. This cross-reactivity is a type II hypersensitivity reaction and is termed molecular mimicry. Usually, self reactive B cells remain anergic in the periphery without T cell co-stimulation. During a streptococcal infection, mature antigen-presenting cells such as B cells present the bacterial antigen to CD4+T cells which differentiate into helper T2 cells. Helper T2 cells subsequently activate the B cells to become plasma cells and induce the production of antibodies against the cell wall of Streptococcus. However the antibodies may also react against the myocardium and joints,[12] producing the symptoms of rheumatic fever. S. pyogenes is a species of aerobic, cocci, gram-positive bacteria that are non-motile, non-spore forming, and forms chains and large colonies.[13]
S. pyogenes has a cell wall composed of branched polymers which sometimes contain M protein, a virulence factor that is highly antigenic. The antibodies which the immune system generates against the M protein may cross-react with heart muscle cell protein myosin,[14] heart muscle glycogen and smooth muscle cells of arteries, inducing cytokine release and tissue destruction. However, the only proven cross-reaction is with perivascular connective tissue.[citation needed] This inflammation occurs through direct attachment of complement and Fc receptor-mediated recruitment of neutrophils and macrophages. Characteristic Aschoff bodies, composed of swollen eosinophilic collagen surrounded by lymphocytes and macrophages can be seen on light microscopy. The larger macrophages may become Anitschkow cells or Aschoff giant cells. Rheumatic valvular lesions may also involve a cell-mediated immunity reaction as these lesions predominantly contain T-helper cells and macrophages.[15]
In rheumatic fever, these lesions can be found in any layer of the heart causing different types of carditis. The inflammation may cause a serofibrinous pericardial exudate described as "bread-and-butter" pericarditis, which usually resolves without sequelae. Involvement of the endocardium typically results in fibrinoid necrosis and wart formation along the lines of closure of the left-sided heart valves. Warty projections arise from the deposition, while subendocardial lesions may induce irregular thickenings called MacCallum plaques.
Rheumatic heart disease
Pathophysiology of rheumatic heart disease
Micrograph showing an Aschoff body (right of image), as seen in rheumatic heart disease. H&E stain.
Chronic rheumatic heart disease (RHD) is characterized by repeated inflammation with fibrinous repair. The cardinal anatomic changes of the valve include leaflet thickening, commissural fusion, and shortening and thickening of the tendinous cords.[15] It is caused by an autoimmune reaction to Group A β-hemolytic streptococci (GAS) that results in valvular damage.[16] Fibrosis and scarring of valve leaflets, commissures and cusps leads to abnormalities that can result in valve stenosis or regurgitation.[17] The inflammation caused by rheumatic fever, usually during childhood, is referred to as rheumatic valvulitis. About half of patients with rheumatic fever develop inflammation involving valvular endothelium.[18] The majority of morbidity and mortality associated with rheumatic fever is caused by its destructive effects on cardiac valve tissue.[17] The pathogenesis of RHD is complex and not fully understood, but it is known to involve molecular mimicry and genetic predisposition that lead to autoimmune reactions.
Molecular mimicry occurs when epitopes are shared between host antigens and Streptococcus antigens.[19] This causes an autoimmune reaction against native tissues in the heart that are incorrectly recognized as "foreign" due to the cross-reactivity of antibodies generated as a result of epitope sharing. The valvular endothelium is a prominent site of lymphocyte-induced damage. CD4+ T cells are the major effectors of heart tissue autoimmune reactions in RHD.[20] Normally, T cell activation is triggered by the presentation of bacterial antigens. In RHD, molecular mimicry results in incorrect T cell activation, and these T lymphocytes can go on to activate B cells, which will begin to produce self-antigen-specific antibodies. This leads to an immune response attack mounted against tissues in the heart that have been misidentified as pathogens. Rheumatic valves display increased expression of VCAM-1, a protein that mediates the adhesion of lymphocytes.[21] Self-antigen-specific antibodies generated via molecular mimicry between human proteins and streptococcal antigens up-regulate VCAM-1 after binding to the valvular endothelium. This leads to the inflammation and valve scarring observed in rheumatic valvulitis, mainly due to CD4+ T cell infiltration.[21]
While the mechanisms of genetic predisposition remain unclear, a few genetic factors have been found to increase susceptibility to autoimmune reactions in RHD. The dominant contributors are a component of MHC class II molecules, found on lymphocytes and antigen-presenting cells, specifically the DR and DQ alleles on human chromosome 6.[22] Certain allele combinations appear to increase RHD autoimmune susceptibility. Human leukocyte antigen (HLA) class II allele DR7 (HLA-DR7) is most often associated with RHD, and its combination with certain DQ alleles is seemingly associated with the development of valvular lesions.[22] The mechanism by which MHC class II molecules increase a host's susceptibility to autoimmune reactions in RHD is unknown, but it is likely related to the role HLA molecules play in presenting antigens to T cell receptors, thus triggering an immune response. Also found on human chromosome 6 is the cytokine TNF-α which is also associated with RHD.[22] High expression levels of TNF-α may exacerbate valvular tissue inflammation, contributing to RHD pathogenesis. Mannose-binding lectin (MBL) is an inflammatory protein involved in pathogen recognition. Different variants of MBL2 gene regions are associated in RHD. RHD-induced mitral valve stenosis has been associated with MBL2 alleles encoding for high production of MBL.[23] Aortic valve regurgitation in RHD patients has been associated with different MBL2 alleles that encode for low production of MBL.[24] Other genes are also being investigated to better understand the complexity of autoimmune reactions that occur in RHD.
Diagnosis
Rheumatic heart disease at autopsy with characteristic findings (thickened mitral valve, thickened chordae tendineae, hypertrophied left ventricular myocardium).
Modified Jones criteria were first published in 1944 by T. Duckett Jones, MD.[25] They have been periodically revised by the American Heart Association in collaboration with other groups.[26] According to revised Jones criteria, the diagnosis of rheumatic fever can be made when two of the major criteria, or one major criterion plus two minor criteria, are present along with evidence of streptococcal infection: elevated or rising antistreptolysin O titre or DNAase.[8] Exceptions are chorea and indolent carditis, each of which by itself can indicate rheumatic fever.[27][28][29] An April 2013 review article in the Indian Journal of Medical Research stated that echocardiographic and Doppler (E & D) studies, despite some reservations about their utility, have identified a massive burden of rheumatic heart disease, which suggests the inadequacy of the 1992 Jones' criteria. E & D studies have identified subclinical carditis in patients with rheumatic fever, as well as in follow-ups of rheumatic heart disease patients who initially presented as having isolated cases of Sydenham's chorea.[30] Signs of a preceding streptococcal infection include: recent scarlet fever, raised antistreptolysin O or other streptococcal antibody titre, or positive throat culture.[31]
Major criteria
- Polyarthritis:[32] A temporary migrating inflammation of the large joints, usually starting in the legs and migrating upwards.
- Carditis: Inflammation of the heart muscle (myocarditis) which can manifest as congestive heart failure with shortness of breath, pericarditis with a rub, or a new heart murmur.
- Subcutaneous nodules: Painless, firm collections of collagen fibers over bones or tendons. They commonly appear on the back of the wrist, the outside elbow, and the front of the knees.
- Erythema marginatum: A long-lasting reddish rash that begins on the trunk or arms as macules, which spread outward and clear in the middle to form rings, which continue to spread and coalesce with other rings, ultimately taking on a snake-like appearance. This rash typically spares the face and is made worse with heat.
- Sydenham's chorea (St. Vitus' dance): A characteristic series of involuntary rapid movements of the face and arms. This can occur very late in the disease for at least three months from onset of infection.
Minor criteria
- Fever of 38.2–38.9 °C (100.8–102.0 °F)
- Arthralgia: Joint pain without swelling (Cannot be included if polyarthritis is present as a major symptom)
- Raised erythrocyte sedimentation rate or C reactive protein
- Leukocytosis
- ECG showing features of heart block, such as a prolonged PR interval[31][33] (Cannot be included if carditis is present as a major symptom)
- Previous episode of rheumatic fever or inactive heart disease
Prevention
Rheumatic fever can be prevented by effectively treating strep throat with antibiotics.[34]
In those who have previously had rheumatic fever, antibiotics in a preventative manner are occasionally recommended.[34] As of 2017 the evidence to support long term antibiotics in those with underlying disease; however, is poor.[35]
The American Heart Association suggests that dental health be maintained, and that people with a history of bacterial endocarditis, a heart transplant, artificial heart valves, or "some types of congenital heart defects" may wish to consider long-term antibiotic prophylaxis.[36]
Treatment
The management of rheumatic fever is geared toward the reduction of inflammation with anti-inflammatory medications such as aspirin or corticosteroids. Individuals with positive cultures for strep throat should also be treated with antibiotics.[32]
Aspirin is the drug of choice and should be given at high doses.[37]
One should watch for side effects like gastritis and salicylate poisoning. In children and teenagers, the use of aspirin and aspirin-containing products can be associated with Reye's syndrome, a serious and potentially deadly condition. The risks, benefits, and alternative treatments must always be considered when administering aspirin and aspirin-containing products in children and teenagers. Ibuprofen for pain and discomfort and corticosteroids for moderate to severe inflammatory reactions manifested by rheumatic fever should be considered in children and teenagers.
Vaccine
No vaccines are currently available to protect against S. pyogenes infection, although research is underway to develop one.[38] Difficulties in developing a vaccine include the wide variety of strains of S. pyogenes present in the environment and the large amount of time and people that will be needed for appropriate trials for safety and efficacy of the vaccine.[39]
Infection
People with positive cultures for Streptococcus pyogenes should be treated with penicillin as long as allergy is not present. The use of antibiotics will not alter cardiac involvement in the development of rheumatic fever.[32] Some suggest the use of benzathine benzylpenicillin.
Monthly injections of long-acting penicillin must be given for a period of five years in patients having one attack of rheumatic fever. If there is evidence of carditis, the length of therapy may be up to 40 years. Another important cornerstone in treating rheumatic fever includes the continual use of low-dose antibiotics (such as penicillin, sulfadiazine, or erythromycin) to prevent recurrence.
Inflammation
While corticosteroids are often used, evidence to support this is poor.[1] Salicylates are useful for pain.
Steroids are reserved for cases where there is evidence of an involvement of the heart. The use of steroids may prevent further scarring of tissue and may prevent the development of sequelae such as mitral stenosis.
Heart failure
Some patients develop significant carditis which manifests as congestive heart failure. This requires the usual treatment for heart failure: ACE inhibitors, diuretics, beta blockers, and digoxin. Unlike normal heart failure, rheumatic heart failure responds well to corticosteroids.
Epidemiology
Deaths from rheumatic heart disease per million persons in 2012
0–7
8–14
15–20
21–25
26–32
33–38
39–45
46–52
53–63
64–250
Disability-adjusted life year for rheumatic heart disease per 100,000 inhabitants in 2004.[40]
no data
less than 20
20–40
40–60
60–80
80–100
100–120
120–140
140–160
160–180
180–200
200–330
more than 330
About 33 million people are affected by rheumatic heart disease with an additional 47 million having asymptomatic damage to their heart valves.[35] As of 2010 globally it resulted in 345,000 deaths, down from 463,000 in 1990.[41]
In Western countries, rheumatic fever has become fairly rare since the 1960s, probably due to the widespread use of antibiotics to treat streptococcus infections. While it has been far less common in the United States since the beginning of the 20th century, there have been a few outbreaks since the 1980s. Although the disease seldom occurs, it is serious and has a case-fatality rate of 2–5%.[42]
Rheumatic fever primarily affects children between ages 5 and 17 years and occurs approximately 20 days after strep throat. In up to a third of cases, the underlying strep infection may not have caused any symptoms.
The rate of development of rheumatic fever in individuals with untreated strep infection is estimated to be 3%. The incidence of recurrence with a subsequent untreated infection is substantially greater (about 50%).[43] The rate of development is far lower in individuals who have received antibiotic treatment. Persons who have suffered a case of rheumatic fever have a tendency to develop flare-ups with repeated strep infections.
The recurrence of rheumatic fever is relatively common in the absence of maintenance of low dose antibiotics, especially during the first three to five years after the first episode. Recurrent bouts of rheumatic fever can lead to valvular heart disease. Heart complications may be long-term and severe, particularly if valves are involved. In countries in Southeast-Asia, sub-saharan Africa, and Oceania, the percentage of people with rheumatic heart disease detected by listening to the heart was 2.9 per 1000 children and by echocardiography it was 12.9 per 1000 children.[44][45][46][47]
See also
- Rapid strep test
References
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- ^ Rothenbühler, Martina; O'Sullivan, Crochan J.; Stortecky, Stefan; Stefanini, Giulio G.; Spitzer, Ernest; Estill, Janne; Shrestha, Nikesh R.; Keiser, Olivia; Jüni, Peter (2014-12-01). "Active surveillance for rheumatic heart disease in endemic regions: a systematic review and meta-analysis of prevalence among children and adolescents". The Lancet Global Health. 2 (12): e717–726. ISSN 2214-109X. PMID 25433627. doi:10.1016/S2214-109X(14)70310-9.
- ^ Shrestha NR; Karki P; Mahto R; et al. (2016-03-02). "Prevalence of subclinical rheumatic heart disease in eastern nepal: A school-based cross-sectional study". JAMA Cardiology. 1: 89. ISSN 2380-6583. doi:10.1001/jamacardio.2015.0292.
- ^ Beaton, Andrea; Okello, Emmy; Lwabi, Peter; Mondo, Charles; McCarter, Robert; Sable, Craig (2012-06-26). "Echocardiography screening for rheumatic heart disease in Ugandan schoolchildren". Circulation. 125 (25): 3127–3132. ISSN 1524-4539. PMID 22626741. doi:10.1161/CIRCULATIONAHA.112.092312.
External links
| Classification |
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- Rheumatic fever information from Seattle Children's Hospital Heart Center
- Jones major criteria, Mnemonic
- Rheumatic Heart Disease Network
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UpToDate Contents
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- 1. 急性リウマチ熱：臨床症状および診断 acute rheumatic fever clinical manifestations and diagnosis
- 2. 急性リウマチ熱：治療および予防 acute rheumatic fever treatment and prevention
- 3. 急性リウマチ熱：疫学および病因 acute rheumatic fever epidemiology and pathogenesis
- 4. リウマチ性心疾患の自然史、スクリーニング、およびマネージメント natural history screening and management of rheumatic heart disease
- 5. 連鎖球菌扁桃咽頭炎の治療および予防 treatment and prevention of streptococcal tonsillopharyngitis
English Journal
- The Platelet-to-Lymphocyte Ratio as an Inflammatory Marker in Rheumatic Diseases.
- Gasparyan AY, Ayvazyan L, Mukanova U, Yessirkepov M, Kitas GD.
- Annals of laboratory medicine. 2019 Jul;39(4)345-357.
- The platelet-to-lymphocyte ratio (PLR) has emerged as an informative marker revealing shifts in platelet and lymphocyte counts due to acute inflammatory and prothrombotic states. PLR has been extensively examined in neoplastic diseases accompanied by immune suppression and thrombosis, which can be p
- PMID 30809980
- Poor long-term adherence to secondary penicillin prophylaxis in children with history of rheumatic fever.
- Amarilyo G, Chodick G, Zalcman J, Koren G, Levinsky Y, Somekh I, Harel L.
- Seminars in arthritis and rheumatism. 2019 Jun;48(6)1019-1024.
- Recurrent episodes of acute rheumatic fever may contribute to the development or worsening of rheumatic heart disease. Secondary penicillin prophylaxis (SPP) has been found to significantly reduce the incidence of rheumatic heart disease. This study sought to evaluate adherence to oral and intramusc
- PMID 30415945
- Clinical-Serological Characterization and Treatment Outcome of a Large Cohort of Italian Children with Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal Infection and Pediatric Acute Neuropsychiatric Syndrome.
- Lepri G, Rigante D, Bellando Randone S, Meini A, Ferrari A, Tarantino G, Cunningham MW, Falcini F.
- Journal of child and adolescent psychopharmacology. 2019 May;().
- Array Array
- PMID 31140830
Japanese Journal
- 臨床研究・症例報告 当科で経験した急性リウマチ熱の2症例 (感染症)
- 小児科臨床 70(3), 321-327, 2017-03
- NAID 40021100549
- Mizoribineによると考えられた急性薬剤性肺障害の1例
- 気管支学 39(1), 22-27, 2017
- NAID 130005345079
- 病理組織診断でリウマチ熱による伝導路障害が死因であると疑われた1例
- 日本小児循環器学会雑誌 32(5), 423-428, 2016
- NAID 130005420622
Related Pictures
![Rheumatic Fever [USMLE Highly Tested Topic] - YouTube](https://1mg.info/0/r/i/0708.jpg)
★リンクテーブル★
| リンク元 | 「リウマチ熱」「僧帽弁狭窄症」「RF」「acute rheumatism」「polyarthritis rheumatica」 |
| 拡張検索 | 「acute rheumatic fever」 |
| 関連記事 | 「rheumatic」 |
「リウマチ熱」
- 英
- rheumatic fever, RF
- 同
- 急性関節リウマチ
- 関
- [[]]
- http://mymed.jp/di/ucm.html?PHPSESSID=2cc03d17984266cc113974b772d3934f
概念
- 膠原病の一つ
- 自己免疫性炎症性疾患
- A群β溶連菌
- 関節と心臓を冒す
病因
- A群β溶連菌の急性咽頭炎後 → (3-4週潜伏期) → A群β溶連菌との交差抗原性(M蛋白、多糖体 VS 心内膜、血管内皮細胞、脳細胞) ← II型アレルギーの機序によると考えられている
疫学
- 日本：著しく減少したが。開発途上国：主なリウマチ性疾患
- 5-15歳に好発。学童期に多い
病理
症状
- Jonesは森(心炎)林(輪状紅斑)で大きな(四肢の大関節から始まる)ブ(舞踏病)タ(多発性関節炎)に引っかけ(皮下結節)られた
- pancarditis, arthritis, chorea minor, erythema marginatum, subcutaneous nodule: PACES
- 1. 汎心炎 (50%)
- 心筋炎 ：房室伝導障害
- 心内膜炎：僧帽弁閉鎖不全症、三尖弁閉鎖不全症
- 心膜炎 ：→心膜摩擦音(friction rub)聴取、心嚢液貯留
- 2. 多関節炎 (70%)
- 3. 小舞踏病 chorea minor Sydenham's chorea
- 視床下部/尾状核ニューロンの障害
- 5. 皮下小結節 subcutaneous nodules
シデナム舞踏病、皮下結節、有縁性紅斑を認める。
症候スペクトル
| 病態 | レイノー現象 | 抗核抗体 | リウマトイド因子 | 抗好中球細胞質抗体 | 皮疹 | 皮下結節 | 関節炎 | 筋炎 | 漿膜炎 | 自己抗体 |
| 病理 | 壊死性血管炎 | 糸球体腎炎 | 間質性肺炎 | 心炎 | 唾液腺炎 | オニオンスキン病変 | ワイヤーループ病変 | ヘマトキシリン体 | LE細胞 | |
診断
- ジョーンズの基準 Jones criteria Jonesの基準(1992)
| 1 | 2 | 3 | 4 | 5 | ||
| 主症状 | 心炎 | 多関節炎 | 小舞踏病 | 輪状紅斑 | 皮下結節 | |
| 副症状 | 臨床症状 | 関節痛 | 発熟 | リウマチ熱、 あるいはリウマチ性疾患の既往 |
||
| 検査査所見 | 急性期反応 ESR↑、CRP↑、WBC↑ |
PR時間↑ | ||||
| 先行するA群レンサ球菌感染の証拠 | (関連抗体の高値または上昇、咽頭培養陽性または迅速反応陽性) | |||||
- 診断:先行するレンサ球薗感染が証明され、かつ主症状2項目以上または主症状1項目+副症状2項目以上
検査
治療
- 溶連菌治療にペニシリン、リウマチ性炎症抑制にサリチル酸剤ないし副腎皮質ステロイド剤、心炎抑制に副腎皮質ステロイド剤を用いる。
- 〔治療〕 急性期は安静臥床としサリチル酸剤を用い、心炎合併時には副腎皮質ホルモン剤を併用する。
- また、A群溶連菌に対し十分量のペニシリンを用い、治癒後も再発を防ぐために長期間予防的化学療法を行う。
予後
- 良好
- 急性の心炎(弁膜症)が生じると心不全につながる
予防
一次予防
- 抗菌薬による溶連菌性扁桃炎の治療(10日間のペニシリン内服)
- 心炎合併例ではステロイド
二次予防
- 再発予防、弁膜症重症化の予防
- 再発から5年間経口ペニシリンの内服
「僧帽弁狭窄症」
- MS
- 英
- mitral stenosis, MS, mitral valve stenosis
- 関
- 僧帽弁狭窄、弁膜症
- distolic murmur at mitral area.
- opening snap at mitral area
疫学
- 女性に多い。男女比1:3。
- 50%が20歳代。20-40歳に多い。
- 既往にリウマチ熱がある事が多い。
病因
- リウマチ熱 rheumatic fever
- その他
- 先天的な僧帽弁尖の狭窄
- 高齢者の前尖の石灰化
- 心内膜炎で弁尖に疣贅ができて弁口を閉塞
- 僧帽弁の弁口：MSでは2cm^2、健常人では4-6cm^2
病態
- 拡張期に左心房の血液を左室に駆出する抵抗が大きくなり、左心房に圧負荷がかかる！！
- 心房細動の合併で重症化する。
合併症
感染性心内膜炎：僧帽弁閉鎖不全症は感染性心内膜炎を来しやすい疾患であると認識してよいが，僧帽弁狭窄症の感染性心内膜炎は比較的少ない。よって僧帽弁狭窄症をハイリスクとすべきかどうかは議論が分かれる。(ガイドライン1より抜粋)
検査
- 聴診
- I音の亢進
- 僧帽弁開放音 (open snap)
- 心尖部拡張中期雑音：低音(low-pitched)、ランブル(rumbling)
- 前収縮期雑音：(洞調律の時に聴取され、心房細動では消失)
- 重症例 → グラハム・スティール雑音(肺高血圧を来した場合にPRを来し、拡張期に2-3LSBで聴取される。
- 超音波エコー検査
- 経胸心エコー：
- 経食道心エコー：左房内血栓の描出に適する。
治療
- 内科的治療
- NYHA I度、かつ軽度僧帽弁狭窄症
- 侵襲的治療
手術療法の適応
- ガイドライン2
- 次の1.～2.が適応を判断するのに重要である。ガイドライン2ではOMC, MVRの推奨基準やNYHA別のフローチャートが示されている。
- 1. PTMCはNYHA II度以上の臨床症状出現 ← 僧帽弁弁口面積≦1.5cm2で左房から左室への血液流入障害が生じる。弁口面積≦1.0cm2では安静時にも左室への血液流入が障害されると肺うっ血・肺高血圧、心房細動が固定化、TRによる右心不全もきたす。
- 2. 心房臓細動の出現
- 3. 血栓塞栓症状の出現
- QB. C-444
- PTMCはNYHA II度以上、OMC,MVRはNYHA III度以上
- 弁口面積1.5cm2以下で臨床症状を有する
- NYHA I度であっても塞栓の既往や左房内血栓が疑われる場合 ← 経食道エコーで確認
- 左房平均圧15mmHg以上
- 出典不明
- 症状と治療法の選択
- 無症状例：保存的療法。心不全と血栓を予防。
- 症状有り：経皮経静脈的僧帽弁交連裂開術、僧帽弁置換術
ガイドライン
- 1. 感染性心内膜炎の予防と治療に関するガイドライン（2008年改訂版）
- 2. 弁膜疾患の非薬物治療に関するガイドライン（2007年改訂版）
国試
「RF」
- 逆流率 regurgitant fraction
- リウマチ熱 rheumatic fever
- リウマチ因子 リウマトイド因子 rheumatoid factor
- 複製型 replicative form
- ラジオ波 radio frequency
「acute rheumatism」
「polyarthritis rheumatica」
リウマチ性多発関節炎
「acute rheumatic fever」
「rheumatic」
- adj.
- リウマチ性の、リウマチの