Lupus, the Heart and Lungs

This site is intended for healthcare professionals as a useful source of information on the diagnosis, treatment and support of patients with lupus and related connective tissue diseases.

The heart

The heart can be affected in a variety of different ways in lupus involving the pericardium, the myocardium, the endocardium and the coronary arteries, as below:-
• Pericarditis is the most common heart abnormality in lupus, presenting in up to 45% of patients.
• Pericardial effusions can present with symptoms of sub-sternal pain and Breathlessness.
• Myocarditis is less common, presenting in approximately 10% of patients Clinical symptoms of bradycardia and/or tachycardia, breathlessness and palpitations are common sometimes with signs of congestive heart failure.

There is evidence of increasing risk factors for accelerated atherosclerosis in lupus that may predispose to atherosclerosis plaque formation. Young women with lupus are more susceptible, especially those from Afro-Caribbean and African backgrounds. Close control of cholesterol on a yearly basis is essential and treatment with statins is often needed especially in patients with disease. The increased incidence of atherosclerosis in some lupus patients can be considered in part to be related to steroid use that may in turn lead to obesity, hypertension, glucose intolerance and hypercholesterolemia. Ideal target ranges are seen below:-

Important lifestyle advice includes attention to modifiable risk factors such as:
• Eating a healthy balanced diet, one that is low in sugar and fat and high in fibre, with plenty of vegetables and whole-grain foods.
• Controlling weight and blood pressure.
• Regular daily exercise.
• Smoking should be avoided and stopped as soon as possible as this significantlycontributes to the risks of heart disease and cancers, especially lung cancers, and overall health will improve.

Hydroxychloroquine has been shown to provide cardio-protection so should be considered where appropriate. If there is clinical suspicion of active lupus leading to inflammatory response, then immunosuppressives should be considered to improve outcomes.

The lungs

Lupus can cause a number of acute and chronic lung diseases that include:-
• Pleural disease: Up to 60% present with pleuritic chest pain, some associated with pleural effusion. This can be complicated by infection which should be treated quickly to prevent complications developing.
• Pulmonary embolism: Sudden onset pleuritic chest pain with dyspnoea should be investigated for existence of pulmonary emboli. Thromboembolic events are increased in those who carry the antiphospholipid antibodies and those diagnosed with antiphospholipid syndrome. Patients should be
rapidly assessed and anticoagulated pending further investigations such as CT-PA scanning.
• Acute pneumonitis: This has been reported in up to 9% of lupus patients with symptoms of fever, pleuritic chest pain, dyspnoea and cough. Chest x-ray may show inner zone alveolar infiltrates, elevated diaphragm or pleural effusion.
• Acute alveolar haemorrhage: this can present in a similar way to acute pneumonitis and may represent the same spectrum of disease. This is rare and can be life threatening.
• Interstitial lung Disease: this can be sub-acute as a bronchiolitis obliterans or chronic active inflammation. When ‘ground glass’ focal alveolar opacities are noted, treatment with steroids and immunosuppression can be effective.
• ‘Shrinking lung’ was first described in 1965, and possibly relates to respiratory muscle weakness (predominately the diaphragm) caused by an unknown myopathic process. This remains a rare presentation but like the other manifestations responds to immunosuppression if treated promptly

Lupus and Pulmonary Hypertension

Pulmonary hypertension is defined as an elevated mean pulmonary arterial pressure > or = 25mmHg at rest by right heart catheterisation, and can be primary or secondary to other lung conditions such as interstitial lung disease or pulmonary embolism. Due to lack of reliable research data and the prognostic implications, a disease entry “PH on exercise” cannot be defined and should not be used. Whilst pulmonary hypertension (PH) is seen more frequently as a secondary complication of scleroderma it can be seen in lupus especially in those with antiphospholipid syndrome. The gold standard treatment includes referral to a specialist pulmonary hypertension designated national centre where examination of both right and left heart is assessed by cardiac catheterisation, in combination with pulmonary assessment to ensure the best outcome.

The prognosis for people with lupus and pulmonary hypertension is much better than in other connective tissue diseases like scleroderma. In lupus, there can be an inflammatory component that responds to steroids and immunosuppression.
Treatment options for pulmonary hypertension have improved with specialist management from designated national centres who often run satellite clinics in peripheral areas. Current treatment options include endothelial-receptor antagonists, phosphodiesterase type-5 inhibitors (sildenafil), diuretics, anticoagulants and epoprostenol (given subcutaneously or intravenously via a pump) for its potent vasodilator effects. Specialist treatment options have, and continue to, significantly improve outcomes.