Rheumatic Involvement Research Articles

Rheumatic and nonrheumatic disease in the temporomandibular joint: gadolinium-enhanced MR imaging.

Magnetic resonance (MR) imaging of 36 temporomandibular joints (TMJs) in 27 patients and six healthy volunteers was performed before and after injection of gadopentetate dimeglumine. Twelve asymptomatic joints were used as controls, 12 TMJs had symptomatic internal derangement, and 12 TMJs had rheumatic inflammatory disease. A small or moderate joint effusion was seen in one asymptomatic joint, four joints with internal derangement, and one joint with rheumatic involvement; in all of these, contrast enhancement of the effusion was observed. A large effusion in one rheumatic joint was enhanced only after delayed imaging. In healthy controls and patients with internal derangement, no or only minimal enhancement of intraarticular tissues was seen. Eleven of the 12 rheumatic TMJs showed moderate or intense soft-tissue enhancement along the disk and articular surfaces (ie, in areas normally devoid of synovial membrane). The one rheumatic joint without enhancement had bony ankylosis and no remaining soft tissue within the joint space. Gadolinium-enhanced MR imaging of the TMJ may effectively depict the proliferating synovium of rheumatic inflammatory joint disease.

Co-existence of congenital and rheumatic heart disease in 404 post-mortem cases—comparison with findings in 2,352 cases with isolated rheumatic disease

SummaryWe reviewed 2,756 necropsy studies of patients who died because of rheumatic heart disease. The anatomic material was divided into two groups. The first group was made up of 404 cases (14.6%) having the association of rheumatic and congenital heartdisease. The second group comprised 2,352 cases with isolated rheumatic heart disease. The analysis of the site and types of congenital malformations in the first group showed 160 cases (39.6%) with aortic, 112 (27.7%) with pulmonary, 15(3.7%) with tricuspid and six (1.5%) with mitral lesions. Twenty patients (4.9%) had an atrial septal defect whilenine (2.2%) had patency of the arterial duct and four (1 %) a ventricular septal defect. The incidence of rheumatic aortic valvar disease in cases also having congenital lesions of the aortic valve (83.7%) was higher than in patients with isolated rheumatic involvement of the aortic valve (67.7%) (p<0.001). The difference in the incidence of rheumatic lesions found in patients with congenital pulmonary lesions (9.8%) and in the patients with isolated rheumatic disease (3.3%) was also significant (p<0.001). No differences were observed in rheumatic lesions to the mitral and tricuspid valves in those with or without associated congenital lesions. In patientswith an atrial septal defect, there was a higher incidence of combined mitral and tricuspid lesions (35% vs. 18%, p<0.02). Patients with patency of the arterial duct andco-existing rheumatic heart disease had a greater incidence of isolated rheumatic aortic disease (11% vs. 0.7%), and also of combined aortic and mitral lesions (33% vs. 17%), but the differences were not significant. The incidence of involvement of all four valves was 5.4% in those with associated congenital lesions and 3% in those in which rheumatic involvement was isolated (p<0.O2). We conclude that rheumatic heart disease has a tendency to involve preferentially cardiac valves that are the sites of congenital malformations.

M-mode, cross-sectional and color flow Doppler echocardiographic findings in acute rheumatic fever

AbstractM-mode, cross-sectional and color flow Doppler echocardiography were performed in 28 patients with acute rheumatic fever. The patients were grouped according to the presence or absence of carditis and congestive heart failure. Abnormal echocardiographic findings were found in 25 patients (89%), including five with no carditis. M mode echocardiography showed significant cardiac enlargement in all patients with carditis, and in two patients with no carditis. The myocardial contractility, as based on ejection fraction and fractional shortening, was normal in all patients except one. Prolapse of the leaflets of the mitral valve (57%) and increased echogenicity of the leaflets of the mitral valve (36%) were the most common findings seen on cross-sectional echocardiography. Pericardial effusion was seen in six patients (21%), all with heart failure. Color flow and Doppler echocardiography showed that mitral regurgitation, which was seen in 24 patients (86%), was the most common finding. Aortic regurgitation was found in 17 patients (60%). The presence of congestive heart failure in patients with carditis was related to the severity of the valvar regurgitation and the number of valves involved. Echocardiographic evidence of rheumatic involvement of the heart may be observed even in the absence of clinical signs of carditis.

Morphological study of resected mitral and aortic valves in rheumatic heart disease

Gross and histological changes in simultaneously resected mitral and aortic valves in patients with rheumatic heart disease are described. All the mitral valves showed characteristic postinflammatory changes. However, 14 per cent of the resected aortic valves did not show features indicative of postinflammatory pathology and were considered to be only haemodynamically altered valves. Myxoid change seen in these valves was at times indistin-guishable from that seen in primary myxoid change in the valves. Our findings indicate that histological features in rheumatic involvement of the valve although suggestive are not diagnostic. Critical gross examination along with histological features will give aetiological diagnosis in most of the cases.

Imaging temporomandibular joint abnormalities in patients with rheumatic disease: Comparison with surgical observations

The preoperative examination findings in the soft tissue and bone of 22 temporomandibular joints of 15 patients with rheumatic disease were compared with the diagnosis after TMJ surgery. Agreement was found in 15 joints with rheumatic involvement and in 4 with internal derangement. In 5 (with unsuccessful arthrotomography) of the 15 rheumatic joints, magnetic resonance imaging showed destruction of disks with soft-tissue replacement, corresponding to fibrous tissue/ankylosis observed at surgery. Bony fusions in 2 of these joints were depicted with computed tomography. In the remaining 10 joints, arthrotomography showed irregularly outlined small compartments corresponding to synovial proliferations observed during surgery. Similar arthrotomographic interpretation, however, was made in 2 of 3 temporomandibular joints with imaging-surgery disagreement; surgery showed fibrous adhesions. In the third joint with unsuccessful arthrotomography, magnetic resonance imaging showed internal derangement but no synovial proliferations that were surgically observed. As experienced with other joints, synovial proliferations (or fibrous adhesions) could not be depicted with magnetic resonance imaging. Thus, differentiation between internal derangement with and without rheumatic involvement could be impossible with both arthrotomography and unenhanced magnetic resonance imaging.

The importance of tricuspid valve structure and function in the surgical treatment of rheumatic mitral and aortic disease.

A significant proportion of individuals with rheumatic disease have tricuspid valve involvement which may be clinically important and alter the medical or surgical approach to treatment. Therefore 50 patients with rheumatic left-sided valvular lesions who were referred for operative treatment were studied. Thirty patients had angiographically significant tricuspid regurgitation (group I) and 20 had a competent tricuspid valve (group II). Pre-operative cardiac assessment included Doppler echocardiography and contrast ventriculography. Patients with tricuspid regurgitation more commonly had mitral valve disease or combined mitral and aortic valve lesions, (P less than 0.001) and were more likely to have atrial fibrillation than those without tricuspid regurgitation (P less than 0.001). Pulmonary arterial systolic and mean right atrial pressures were higher in group I (both P less than 0.01). A close relationship was found between the angiographic and Doppler assessment of the degree of tricuspid regurgitation (P less than 0.01). Doppler-derived measurement of the right ventricular-right atrial systolic pressure difference correlated well with the systolic trans-tricuspid pressure difference measured at cardiac catheterization (y = 0.7x + 22, r = 0.67, P less than 0.001) and the pulmonary arterial systolic pressure (y = 0.8x + 27, r = 0.71, P less than 0.001). Rheumatic involvement of the tricuspid valve identified by pre-operative echocardiography was confirmed in five patients at surgery. Of the 13 patients with functional tricuspid regurgitation at operation, only two had been diagnosed as having organic disease by echocardiography. Furthermore, in all 18 cases where Doppler suggested grade 3 or 4+ tricuspid regurgitation, surgical repair or replacement of the valve was performed.(ABSTRACT TRUNCATED AT 250 WORDS)

Rheumatic disease of the temporomandibular joint: MR imaging and tomographic manifestations.

Thirty-six temporomandibular joints (TMJs) in 28 symptomatic patients (aged 14-40 years) with rheumatic disease (mostly rheumatoid arthritis) were studied with magnetic resonance (MR) imaging and hypocycloidal tomography. MR images of four TMJs were normal. Another four TMJs showed internal derangement. Of the 28 TMJs presumed to show rheumatic disease involvement (26 with condylar destruction or deformation), 23 showed abnormal disk structure--five showing severe disk destruction and 18 showing less severe abnormalities (inhomogeneous structure, fragmentation, poor delineation, and severe flattening). MR images showed bone abnormalities in 27 of the 36 TMJs, and tomography showed abnormalities in 25 of the 36 TMJs. Good agreement between the two imaging modalities regarding surface irregularities was found. However, MR imaging demonstrated more extensive bone abnormalities than did tomography in 11 TMJs. The potential of MR imaging for depicting bone and soft-tissue abnormalities associated with rheumatic TMJ involvement was clearly demonstrated.

Arthrographic findings in the temporomandibular joint in patients with rheumatic disease

Hypocycloidal multisection tomography and lower-space arthrotomography with videofluoroscopy were performed on 20 symptomatic temporomandibular joints (TMJs) of 17 patients. All patients (16 women, one man, aged 17 to 38 years) had definite or suspected rheumatoid arthritis (adult or juvenile type), ankylosing spondylitis, or psoriatic arthropathy. Bone abnormalities were found in 14 TMJs, primarily cortical erosion (11 joints), but also condylar flattening (three joints). Irregularity in outline of the contrast material, bone contour-contrast material gaps, evidence of adherent discs and/or small joint compartments indicated synovial hyperplasia/pannus formation in 15 joints. Six of these (four with normal disc position) showed perforation between the joint compartments. In those with normal disc position the perforation seemed to occur in the central portion. Ten of the 15 joints had normal disc position; the remaining five had anterior disc displacement without reduction. One joint showed only disc displacement. Most joints with bone abnormalities (12 of 14) showed arthrographic signs of rheumatic involvement. Such signs were also observed in two of the six joints with no bone abnormalities, indicating the value of arthrography in the early diagnosis of patients with rheumatic disease and TMJ problems.

Mitral annulus calcification: clinical implications.

M-mode echocardiographic features of 53 patients with mitral annulus calcification (MAC) are presented with an initial clinical analysis. Direct measurement of the thickness of the calcification is described as a new technique to define the severity of this process, possibly with a prognostic value as well. Moderate to severe degree of MAC (>5 mm average thickness) was found in 69.7% of patients, all without history of rheumatic heart involvement, and only one had chronic renal failure. Detailed electrocardiographic observations showed the presence of conduction disturbances in 53.6%; 6 patients with 3° atrioventricular block and 3 with sick sinus syndrome and widespread conduction disturbances required permanent pacemaker. Electrophysiologic study of 7 patients demonstrated a significant prevalence of involvement of the conduction system below the proximal His bundle, the region more directly in contact with the calcified annulus. We believe that this quantitative method of evaluating patients with MAC is of value for noninvasive, more complete clinical study of this disease.

The ascending aorta in aortic stenosis

Dilatation of the ascending aorta, a frequently reported sign of aortic stenosis, was assessed in 47 patients for whom aortic angiograms has been recorded. Twenty-eight of the patients had rheumatic valvar disease and 19 had congenital aortic stenosis. A simple ratio between the maximal width of the mid-ascending aorta and the width of the aortic root was calculated. Patients with congenital aortic stenosis had significantly greater supravalvar aortic dilatation than did those with rheumatic aortic valve involvement. There was no correlation between the pressure difference across the aortic valve and degree of dilatation of the ascending aorta.

Spontaneous Rupture of Mitral Chordae Tendineae

Seven patients with spontaneous rupture of mitral chordae tendineae confirmed by operation had a usual clinical profile consisting of a relatively abrupt onset of pulmonary congestion in a late middle-aged man, a systolic murmur, a vigorously pulsating heart, a normal rhythm, and a slight enlargement of the left atrium. Hemodynamic studies confirmed pulmonary congestion due to large pulmonary capillary v waves, and elevation of left ventricular diastolic pressure, although ventricular contractility in terms of total volume ejected was good. Mitral cusps showed atypical mucoid degeneration with no rheumatic or bacterial involvement. A relationship of this condition to others previously associated with mucoid degeneration of the mitral value is suggested. The cause of this syndrome is still unknown.

Coronary Artery Disease and Left Ventricular Function in Mitral Stenosis

Twenty-six men with mitral stenosis were studied to assess the importance of obstructive coronary disease in the production of symptoms and the effect upon left ventricular function. The patients were studied by cardiac catheterization including selective coronary arteriography and cineventriculography. Five patients (Croup 1) showed severe obstructive coronary lesions, eight patients (Group II) had minor arterial changes and 13 (Group III) had normal vessels. Angina pectoris was confined to the first group who also demonstrated significant left ventricular dysfunction, the consequence of coronary disease. Left ventricular impairment was also found in a significant proportion of those patients without important obstructive coronary disease. In these patients the etiology of the myocardial impairment is poorly understood. Rheumatic involvement of the myocardium or the small coronary vessels has been suggested as an explanation.

Use of the hypospray jet injector for intra-articular injection.

OBJECTIVE: To determine the frequency of occurrence of malignancy concurrently with temporal arteritis (TA), as well as features and outcome of the vasculitis in such cases. METHODS: In a series of 271 consecutive patients with TA (219 biopsy-proven), we retrospectively analyzed the frequency and type of malignancy concurrent with vasculitis (less than 1 year before or after), as well as the main features and outcome of TA in this setting. We also surveyed all cases published in the French-British literature. RESULTS: We observed 20 patients with TA and concurrent malignancy and reviewed 27 similar published reports. GCA was documented pathologically in 86% of the cases. The time between diagnosis of TA and that of malignancy averaged 3.5 months (synchronous diagnoses in 27 patients). Various locations of cancers were found, particularly the gastrointestinal tract (9 cases); blood malignancies accounted for 45% of cases (lymphoid disorder in 9, myelodysplastic syndrome in 11, chronic myelogenous leukemia in 1). In our patients, logistic regression analysis failed to demonstrate differences between those with and without malignancy, except for a higher frequency of rheumatic involvement in the former group (60% vs 30%; p = 0.01). The initial response to steroid treatment was good in 92% of 40 assessable patients, and the vasculitis course mirrored that of malignancy in only 2 patients. Regarding the outcome of TA, no differences were observed in our patients with and without malignancy. CONCLUSION: Concurrent malignancy in TA is not a rare finding, being observed in up to 7.4% of the cases. Solid malignancies and hematological disorders, especially myelodysplastic syndromes, may represent precipitating factors for development of TA, which infrequently run a paraneoplastic course. Patients with and without malignancy seem almost indistinguishable regarding features and outcome of TA. Physicians who care for patients with TA should be mindful of this potential association, even in typical cases.

Congenital quadricuspid aortic valve

This case of a patient with a congenital quadricuspid aortic valve is the seventh to be reported in the world literature. Our report indicates that a quadricuspid aortic valve appears to function as well hemodynamically as a normal cuspid valve and therefore was discovered only as an incidental finding in this 67 year old man. Multiple fenestrations of the aortic cusps and an accessory right coronary artery were also noted. Review of the literature reveals that congenital malformations of the aortic and pulmonary valves are frequently associated with other cardiac abnormalities. The quadricuspid aortic and pulmonary valves do not appear to be particularly susceptible to bacterial endocarditis. The high incidence of bacterial endocarditis in the bicuspid aortic valve appears to be directly related to rheumatic involvement of this valve. The increased incidence of bacterial endocarditis in the bicuspid pulmonary valve may well be related to the coexisting ventricular septal defect.

Acquired aortic valvular stenosis. Its diagnosis by conventional radiological study.

Stenosis of the aortic valve may be of congenital or acquired origin. Among the congenital malformations of the heart and great vessels, aortic stenosis is not an uncommon lesion. It may occur as stenosis of the valve itself or as a localized area of constriction in the ascending aorta above the valve, so-called supravalvular stenosis. Congenital constriction of the outflow tract of the left ventricle is a well recognized entity, but since it does not involve the aorta per se, the lesion hardly deserves the oft applied designation of subaortic stenosis. In the average general practice of radiology, the acquired form of aortic valvular stenosis is much more frequently encountered than is the congenital type, and it is to the consideration of acquired constriction that the present communication will be limited. The etiology of acquired aortic stenosis has been the subject of considerable study. Two principal causes have been propounded: namely, aortic sclerosis or fibrosis and rheumatic valvulitis. Kiloh (1) and others (2, 3) have suggested that nonrheumatic sclerosis is the main cause of pure aortic stenosis. A different opinion is held by most authorities, as Wood (4) and McGinn and White (5), who believe that the disease is principally due to rheumatic involvement of the aortic cusps. Dry and Willius (6) and Karsner and Koletsky (7) have produced strong support for a rheumatic basis of acquired aortic stenosis. As in rheumatic mitral valvulitis, however, a definite history of rheumatic fever or its equivalent is not infrequently lacking. A rheumatic history has been variously recorded as ranging from 14 to 44 per cent (4, 8–11). It is well known that rheumatic involvement of the aortic valve may produce either stenosis or valvular incompetency, or varying combinations of the two. While it is not uncommon for dominant aortic stenosis to be accompanied by some element of aortic regurgitation, it is also well established that acquired stenosis does exist in “pure” form, unassociated with any aortic valvular insufficiency. The principal effect of aortic stenosis is to increase the work load of the left ventricle and, under conditions where this chamber is no longer able to compensate for the overwork, cardiac output falls below normal. Hemodynamically significant aortic stenosis results in a difference in systolic pressure between the left ventricle and the ascending aorta, a so-called left ventricular-aortic systolic pressure gradient. When this gradient of pressure exceeds 10 mm. of mercury, it is considered that aortic stenosis is present. The normal aperture of the aortic valve in human adults is approximately 3.0 sq. cm. The aperture (aortic valve area) can be estimated with reasonable accuracy by combined catheterization of both the right and left sides of the heart, with determinations of left ventricular and aortic pressures and of cardiac output, provided there is no aortic regurgitation.

Aortic insufficiency of unusual etiology.

Aortic insufficiency is usually caused by rheumatic or luetic involvement of the aortic valve. Unusual causes may include the following uncommon clinical conditions: (1) functional aortic insufficiency caused by dilatation of the aortic valve ring secondary to prolonged hypertension, (2) fenestrations of the aortic valve, (3) trauma to the aortic valve, (4) congenital abnormalities of the aortic valve, (5) dissecting aortic aneurysms, (6) bacterial endocarditis, (7) brucellosis, (8) Marfan's syndrome, (9) rheumatoid spondylitis, and (10) arteriosclerosis of the aortic valve per se, with or without calcific aortic stenosis. Three cases of aortic insufficiency which resulted from arteriosclerotic involvement of the valve are detailed. A fourth case, in which aortic insufficiency was caused by redundancy of the aortic valves, is perhaps the only case of its type recorded to date.

Quantitation of aortic valvular insufficiency by catheter thoracic aortography.

Aortic valvular insufficiency is, with few exceptions, a consequence of rheumatic endocarditis or syphilitic aortitis. Rheumatic involvement of the aortic valve may produce varying degrees of stenosis or valvular insufficiency, either alone or in combination. The advent of surgical methods for treatment of rheumatic aortic valvular lesions has increased the importance of radiological study of the valve, determination of the extent and character of its pathological changes, and the degree of its functional impairment. While roentgen visualization of the aortic valve can be achieved by angiocardiography, provided a good “levo” study is obtained, or by cardiac ventriculography with introduction of a radiopaque medium by either transthoracic needle puncture or transaortic catheterization of the left ventricle, the most satisfactory method for radiologic study is catheter thoracic aortography. The procedure of catheter thoracic aortography was introduced in 1948 by Radner (1), who inserted a catheter into the ...

Diagnosis of mandibular joint dysfunction

The material used in this investigation has consisted mainly of 186 cases examined and treated by me. The case histories and the results of clinical and roentgenographic examinations are recorded on a standard form, and an analysis of the findings indicates that female patients, particularly in the 20- to 30-year age group, predominate. In the majority of cases pain is the symptom which prompts the patient to seek treatment, and pain is most frequently experienced in the region of the joint itself. Malocclusion in one form or another was found in approximately one-half of the patients, but only a small proportion of these had overclosure. Although malocclusion was present in these cases, it was frequently considered a contributory rather than a primary etiological factor. Neuromuscular tension and trauma were frequently responsible for mandibular joint dysfunction. Rheumatoid arthritis, varying from mild atypical forms to the progressive crippling form, probably attacks the mandibular joint more often than is generally supposed, although there are usually accompanying etiological factors which render the joint more susceptible to rheumatic involvement. Etiological factors are placed as far as possible into primary and contributory categories. In most cases methods of treatment to be employed depend upon the factors considered responsible for symptoms. The differential diagnosis of mandibular joint dysfunction is made easier by the use of a standard form for recording case histories and clinical findings. Certain conditions which may present difficulties in differential diagnosis are discussed.

Endocardial fibrosis simulating constrictive pericarditis; report of a case with determinations of pressure in the right side of the heart and eosinophilia.

THE first physician to describe the association of endocardial fibrosis and eosinophilia as a clinical entity was Löffler1 in 1936. The similarity of this disease to constrictive pericarditis was noted, and it was postulated that in both entities the clinical picture results from restriction of diastolic filling by a thick fibrous plate; it makes little difference whether the thickened plate consists of pericardium or endocardium.Löffler considered the entity that he described to be a manifestation of the rheumatic state, as did Mumme2 and Berblinger,3 who have reported cases in which there was thought to be "rheumatic involvement of valve . . .

Active rheumatic heart disease in patients over sixty.

Rheumatic activity is not often considered in dealing with patients over 60 years of age, although evidences of old rheumatic involvement of the heart are common findings at autopsy in this age group. Acute rheumatic heart disease is usually a disease of children and young adults. Cohn and Lingg 1 made a study of 12,000 patients in New York City who had rheumatic heart disease. They found that only 16% of these patients were in the age groups past 45, and only 3% of the total 12,000 were first affected after the age of 45. Their statistics indicated that the mean age of onset in men was 14.5 ± 0.3 years and in women it was 15.0 ± 0.3 years. Children and young adults often present the typical textbook picture of fever, migratory polyarthritis, carditis, electrocardiographic changes, leukocytosis, and increased sedimentation rate. Older patients may not present all these signs,