Scleroderma or systemic sclerosis is a multi-system disorder that involves the lungs as part of its spectrum of disease. The systemic form of the disease is classified into two types. Firstly, limited cutaneous systemic sclerosis (LcSSc) involves the face and skin of the hands and lower arms. The second pattern of disease is diffuse cutaneous systemic sclerosis (DcSSc) which involves the skin both above and below the elbows and the rest of the body. This classification is important, as there are particular causes of breathlessness that we need to be aware of in these two subsets.

What are the causes of breathlessness in scleroderma? To address this question we need to consider causes of breathlessness that occur in anyone (with or without scleroderma) and causes of breathlessness in only those with scleroderma.

Common causes of breathlessness in the population

Asthma Wheeze, cough Peak flow estimation

Angina Chest pains, breathless on exertion ECG, CXR

Anaemia Tiredness, no cough Full Blood Count

Infection Fevers, sputum Blood cultures, CXR

Fluid on lungs Tiredness, swollen ankles CXR

Kidney malfunction Ankle swelling Blood tests

Causes of breathlessness in Limited Cutaneous Systemic Sclerosis

Anaemia Tiredness, no cough Full Blood Count

Infection Fevers, sputum Blood cultures, CXR

Pulmonary hypertension Very few. Worse on exertion Echo

Clot on lungs Acute breathlessness, chest pain ECG, CT scan

Causes of breathlessness in Diffuse Cutaneous Systemic Sclerosis

Lung fibrosis Cough, sputum CXR, CT scan

Aspiration pneumonia Fevers, sputum Blood cultures, CXR Myositis Weakness on breathing in and out Muscle enzymes

Pulmonary hypertension Very few. Worse on exertion Echo

The treatment of the above causes involves making an accurate diagnosis. This is usually done by blood tests and investigations such as an ECG, CXR, echo or a CT scan of the chest. Most of the above diagnoses can be substantiated by these tests. However, pulmonary hypertension occurring in the context of LcSSc requires further tests after it has been suspected on echo. To do this we usually go on to do a cardiac catheter test so that we can confirm the raised pressures on the right side of the heart before starting specific treatments to combat this.

At the Royal Free Hospital we have a special clinic treating people with pulmonary hypertension, which we have developed over the last 4 years. We also closely liase with the lung specialists at the Royal Brompton Hospital. Treatments include oxygen at home, thinning the blood with a drug called warfarin, calcium channel blockers, and Iloprost. The latter can be given to patients by using a drip that inserts into the chest called a Hickman Line and then the drug is infused at a very low rate continuously. Other routes of treatment with iloprost for pulmonary hypertension include continuous infusion via a needle and a device that allows Iloprost to be breathed in (like a nebulizer for asthma).

The choice of which delivery system to use depends on the severity of the pulmonary hypertension (i.e. the degree of elevation of the pressures and whether the heart is coping or not). Also, it is important to know which type of system the patient can tolerate and cope with at home. This is decided between the doctor, the patient and the pulmonary hypertension nurse who is integral to supervising any of the treatments for this condition.

Newer treatments are becoming available for this condition- one includes a drug called Bosentan which acts by settling inflammation within the lining of the blood vessels that link the right side of the heart with the lungs (the pulmonary arteries). This underlines the importance of regular breathing and heart tests in clinic so that this condition together with all the above conditions that cause breathlessness in a SSc patient can be picked up early so that we can start prompt treatments.