Cardiac Involvement in Rheumatoid Disease

CME Rheumatological and immunological disorders – I Cardiac involvement with the severity of RA 1. The reasons for peripheral oedema and hepatic or this remain unclear. RHD, although gastrointestinal congestion may occur in common, rarely has haemodynamic constrictive disease. In tamponade, in rheumatoid consequences 2. Ischaemic heart disease symptoms and shock can rapidly develop (IHD) is a more likely cause; recent and require emergency intervention. disease studies suggest similarities of inflam- Pericardial rub occurs in 30–40% of matory pathogenic mechanisms in RA clinical cases. Other signs (tachycardia, and atherosclerosis, and an increased ectopy, diminished heart sounds) are George Kitas MD PhD FRCP , 1,3,4 non-specific. Pulsus paradoxus and Consultant/Clinical Senior Lecturer in prevalence of IHD in RA . Kussmaul’s sign (rise in height of jugular Rheumatology, Dudley Group of Hospitals NHS Trust and University of Birmingham pulsation during inspiration) may occur. Range and clinical presentation In constriction, a pericardial knock may Matthew J Banks MRCP, Cardiology of cardiac pathology in be heard due to early cessation of Specialist Registrar/Research Fellow, rheumatoid arthritis University of Birmingham ventricular filling. Constriction associ- ates with high jugular vein pulse and a Paul A Bacon FRCP, Professor of Pericardial disease prominent y descent of early ventricular Rheumatology, University of Birmingham The commonest cardiac complication of filling; in tamponade, the y descent is RA is pericarditis. It is found in 30–50% diminished, leaving a prominent x Clin Med JRCPL 2001;1:18–21 of autopsy cases 5–7 and in up to 30% by descent. Pericardial effusion and con- echocardiography 8,9. It is commoner in striction can co-exist (effusive- male, seropositive patients with active constrictive disease), and this should be Background RA, but can occur in seronegative and suspected if pericardiocentesis fails to quiescent RA or before the onset of rectify haemodynamic compromise 11. The relationship between joint inflam- synovitis. Histopathology shows chronic mation and heart disease was first inflammation and fibrosis. The peri- Non-specific endocarditis suggested by Bouillaud in 1836. In 1891, cardial fluid is usually a clear exudate Charcot described endocarditis and with high protein and lactate dehydroge- Non-specific endocarditis is found in pericarditis in ‘chronic rheumatism’, nase, low glucose, and containing mainly 9–70% of autopsy cases. Echo- differentiating cardiac disease in neutrophils 10. Cholesterol crystals can be cardiographic studies show a high preva- rheumatic fever (rheumatic heart seen in persistent effusions; they also lence of valvular thickening, but clinical disease) from that in other forms of occur in tuberculous pericarditis. disease is rare 8,9. The frequency of valve rheumatism (rheumatoid heart disease Staphylococcal pyopericardium has also involvement is similar to rheumatic heart (RHD)). It is now clear that cardiac been described in RA, so infection is an disease (mitral, aortic, tricuspid, pathology is common in rheumatoid important differential diagnosis. Only pulmonary). Non-specific inflammation arthritis (RA) (Table 1). This may be 2–4% of patients have symptoms, and and fibrosis cause thickening and calcifi- important. Cardiovascular mortality fewer than 0.5% experience haemo- cation, mainly in the base of the valve accounts for 40–50% of all deaths in RA; dynamic compromise 2. The commonest and the valve ring, but this rarely has it is increased and occurs earlier than in symptom is dull central or sharp haemodynamic consequences. The most the general population and may associate pleuritic chest pain. Dyspnoea, distinctive lesions are rheumatoid granu- lomata within the valve leaflets which can cause incompetence. Most patients Table 1. Pathological, echocardiographic and clinical prevalence of rheumatoid heart have no symptoms because the left disease. ventricle (LV) can adapt to significant AutopsyEchocardiography Clinical mitral or aortic regurgitation without (%) (%) (%) decompensating. The effect on ventricular performance is mainly defined by Pericarditis 11–50 20–40 1–4 the rapidity of onset of regurgitation; in Myocarditis 30 rare rare severe cases, lesions can develop over a Focal, non-specific 4–30 – – Diffuse, necrotising rare – – few days and cause rapid LV failure. Granulomatous 3–5 – – Amyloid infiltration rare – – Conduction system disease unknown – rare Myocarditis Endocarditis Myocarditis is found in up to 30% of Valvular disease 6–50 30–40 rare autopsy cases 5–7. It can be diffuse or Coronary arteritis 15–20 – rare Any cardiac lesion 30–50 30–50 1.6–6 focal, non-specific or pathognomonic nodular rheumatoid myocarditis. Its 18 Clinical Medicine Vol 1No 1January/February 2001 CME Rheumatological and immunological disorders – I clinical significance is unknown. The endocarditis, cor pulmonale or are insensitive and non-specific first-line overwhelming majority of patients are constrictive pericarditis. investigations. Echocardiography allows asymptomatic. However, the compact evaluation of both cardiac anatomy and anatomy and relationship of the atrio- Ischaemic heart disease function, and may be helpful in several ventricular node to the aortic root and situations8,9,16: interventricular septum make it Myocardial perfusion imaging under pericarditis (fluid and thickening) vulnerable to damage from inflam- pharmacological stress detects IHD in imminent tamponade (diastolic mation of adjacent structures and can about 50% of RA patients, a prevalence collapse) lead to conduction defects. Complete double that of matched controls 18. This is constriction (preserved LV function heart block has been reported in RA reflected in the incidence of MI and heart but abrupt termination of and penicillamine-induced myositis 12,13. failure as causes of cardiovascular mor- ventricular filling) tality in RA 1. Alarmingly, more than half valvular lesions (grading of Arteritis of RA patients with IHD have no ischaemic symptoms. Classical cardio- regurgitation and serial assessment Arteritis is present in up to 20% of vascular risk factors appear to be of LV end-diastolic dimension) autopsy cases, affecting mainly medium important, but RA, like diabetes, confers amyloidosis and small intramyocardial arteries 5–7. significant extra risk 18. The long-term assessment of LV systolic and This may lead to patchy myocardial significance of this is obvious, but diastolic function. necrosis due to microinfarction or strategies to prevent it are yet to be In cases of constriction, computed ischaemia. Severe arteritis of the epi- established. tomography (CT) scanning is useful to cardial vessels has been reported and confirm pericardial thickening, and tends to be non-occlusive. Its relation- Investigation of cardiovascular helps to differentiate constrictive peri- ship to myocardial infarction (MI) is involvement in rheumatoid carditis from restrictive cardiomyopathy controversial 14,15. arthritis (Table 2). Cardiac catheterisation is essential if pericardectomy is considered. Myocardial dysfunction Overall cardiovascular risk in RA, as in Exercise testing provides evidence of other conditions, can be assessed on the ischaemia, but may be impossible or Several processes operating alone or in basis of history, blood pressure, lipids and difficult due to physical disability. A tandem may lead to myocardial dysfunc- ECG. A range of other investigations is useful alternative is nuclear perfusion tion in RA. Heart failure may be one of also available to identify specific cardiac imaging under pharmacological stress. the main causes of increased cardio- pathologies, assess their effects and allow This may show ischaemia, whether due vascular mortality in RA, particularly targeted treatment. These should be used to epicardial or small vessel abnormali- 1 in men . Diastolic LV dysfunction on judiciously, and they require collabora- ties, and inform the need for further Echo-Doppler, found in 30–40% of RA tion between rheumatologists and invasive investigation 18,19. Coronary 16 patients without overt heart disease , cardiologists. angiography will reveal epicardial is thought to be an early sign of IHD or ECG and chest X-ray are useful, but disease, but can neither differentiate heart failure and has adverse prognostic significance. Restriction due to amyloid can lead to diastolic heart failure; in the Table 2. Differential diagnostic features between constrictive pericarditis and past it was found in 10–20% of rheuma- restrictive cardiomyopathy. toid hearts, but is now rare. Pancarditis and small vessel vasculitis can lead to Diagnostic feature Constrictive Restrictive pericarditis cardiomyopathy systolic pump failure 17, while pulmonary fibrosis can cause right ventricular S3 Gallop Absent May be present failure. Overall, however, heart failure in Pericardial knock May be present Absent RA, as in the general population, is more Palpable systolic apical impulseAbsent May be present likely to be the result of atherosclerotic Pulsus paradoxus May be present May be present disease. Symptoms of myocardial dys- Pericardial calcification Present 50% Absent function such as dyspnoea are unusual in CT scan, MRI, RA, possibly due to reduced physical echocardiography Thickened pericardiumNormal pericardium activity, while signs are non-specific. Equal RV and LV Patients suddenly developing overt heart diastolic filling pressures Usually present LV>RV failure should be investigated for

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