Lupus Site - a guide for lupus patients and their families

Symptoms and signs

The main symptoms and signs of heart or lung involvement are chest pain (sometimes on deep breathing-in), shortness of breath, cough, and ankle swelling. The commonest manifestations of lupus in the heart and lungs are in the linings of these organs, the pleura and pericardium.

Pleurisy

Pleurisy is common in lupus - estimates vary between 30 and 60% of all patients at some time suffering from inflammation of the lining of the chest. The symptom is pain - often a 'catching' pain on taking a deep breath. More severe forms of inflammation and pleurisy produce fluid, so-called pleural effusions. Any fluid in the chest takes useful breathing space away, therefore causing shortness of breath. Pleurisy can be detected clinically and on chest X-ray. Fortunately, it usually responds rapidly to a short course of steroids.

The lungs

Other lung conditions are less common than involvement of the linings of the lungs (pleura). In a small number of patients a scarring occurs in the lungs, so-called fibrosis. This can be detected both clinically or on chest X-ray or scan. It produces shortness of breath, especially on exercise. Another rare manifestation of lupus in the lung is the so-called 'shrinking lung' where on chest X-ray the lung areas look smaller than normal. These are all signs of active disease and require treatment.

The heart - Pericarditis

The pericardium or tissue membrane surrounding the heart is identical to the pleura surrounding the lungs and is also frequently inflamed. Classically, the pain is in the centre of the chest at the front and sometimes mistaken by patient and doctor for a heart attack. It can be detected clinically or on chest X-ray or echocardiogram. Fortunately, it rarely causes damage to the underlying heart and responds to steroids.

Heart valve disease

A small number of lupus patients develop heart valve disease. Many of these patients were, in the old days, wrongly diagnosed as rheumatic fever, but we now know that this was not the case. There is a very strong association with the presence of antiphospholipid antibodies ('sticky blood') and this will be discussed in another fact sheet. Leaky heart valves result in shortness of breath and require expert cardiological advice. Ultimately some patients require valve surgery. Fortunately this is exceptionally rare.

Heart attacks

It was discovered some years ago that a number of lupus patients who have seemingly recovered from their lupus still suffer from coronary disease and have an increased chance of a heart attack. The reasons for this are not clearly known although considerable research work is now going on to look at possible risks. Certainly, steroids have been implicated although they are not the major culprit. Some patients with lupus have persistently raised cholesterol and other lipids. Modern treatment is very effective in reducing these. Most recently antiphospholipid antibodies have been implicated in the clotting process in coronary arteries as well as in other arteries and it may well be that taking regular blood-thinning medicines such as low-dose aspirin will be shown to reduce this risk.

Conclusions

The heart and lungs are not the most commonly affected organs in lupus but pericarditis and pleurisy are often extremely distressing. Fortunately they respond well to steroid treatment. More emphasis is now being placed on looking at risk factors for coronary artery disease - risk factors such as abnormal clotting profiles and abnormal cholesterol levels.