Scleroderma and Lung Disease

Kirsten Lassey, Clinical Nurse Manager, Respiratory Services

Kirsten Lassey, Specialist Nurse, Lung Disease, Hutt Hospital, shared her knowledge with us at the Scleroderma Seminar on 18th May this year.

80% of people with Scleroderma have some lung involvement.

Pulmonary Arterial Hypertension (PAH) and Interstitial Lung Disease (ILD) are the most common problems.
Interstitial Lung Disease is pulmonary fibrosis causing scarring, stiffness of lung tissue, less flexibility, and leading to shortness of breath.

Pulmonary Arterial Hypertension makes it hard to pump blood, resulting in sustained high blood pressure in the small arteries. It builds up slowly with the first symptoms being an increasing dry cough and shortness of breath.

Both diagnosis and treatment have improved in the past 10 – 15 years. Kirsten recommended to be judicious when looking up Google as many articles were not up to date; their prognosis was too grim for today’s treatments.


There is no Gold Standard, with not all tests available in all DHBs. All patients should be screened at diagnosis of scleroderma (not ILD and PAH). Stethoscope, clinical questioning, and ability to walk upstairs are common starting points.

Spirometry tests and other Pulmonary Function. Spirometry measures how much air can be breathed in/out and how quickly. Other pulmonary function tests will reveal if there is reduced diffusion oxygen into the blood.

BNP is a blood test for chemicals that are released by the body when the heart muscle is stretched

6 minute walk tests heart rate and oxygen levels on exertion. This is a good test without being invasive.

HRCT scans for pulmonary fibrosis.

ECHO test when PAH is suspected, but tends to give lots of false negative results. There are very long waiting lists for this test in some hospitals.

Right heart catheter can give a specific diagnosis of PAH but is very intrusive.

Tests should be annually in the first years when fibrosis is more likely to be rearing its head, then every couple of years. Patients should advocate for screening if shortness of breath is becoming a greater issue.

Treatment for ILD:

There is not much treatment. Prednisone which has many side effects, may be used. Oxygen if needed. Low oxygen levels do not correlate with breathlessness though. Oxygen is given to increase the oxygen reaching the vital organs: liver, kidney, brain, heart will all work better.

Treatment for PAH

Drugs have increased, with more being available.

First Line of Drugs:

Diltiazem, a calcium channel blocker has good outcomes but only works for a small minority.

Warfarin, but clinicians are more cautious now because of risk from bleeding.

Sildenafil (Viagra) is a first line drug available because of Pharmac funding.

Many respond well to this drug. It dilates the arteries leading to lower blood pressure for PAH.

Second line of drugs:

Bosentan can help to reduce narrowing of arteries.

Iloprost, a nebuliser that opens up arteries, ideally taken 7-9 times a day as it won’t stay in the system. It takes 4 -5 minutes to work, but it’s not taken at night, for obvious reasons.

Self-Management of Lung Disease:

Don’t stop drugs suddenly as there can be a rapid rebound effect. Be aware that any sudden and extreme change in diet can affect warfarin; e.g. Sudden increase in broccoli can impact on warfarin. Build a good relationship with your G.P. and Pharmacists so that they are aware of your needs, and stock supplies as needed.

Google does not show that different patients react differently to drugs. Research is only now beginning to understand why.

Exercise is really important; ‘Use it or Lose it.’ Better to have consistency and “Pace Yourself”so you are exercising continually – Not 1 day exercise and the next day you are unable to do anything as you are shattered.

Pulmonary Rehabilitation Programmes run by Physiotherapists and/or nurses are available at DHBs. These are 9 weeks of twice weekly exercise and education programmes usually focussed on COPD & emphysema patients; sometimes PAH patients are included.

Wear protective masks when working with pollutants as lungs are more susceptible. Pregnancy is particularly difficult to manage in PAH, a high percentage of pregnant women with PAH do not survive pregnancy, and some drugs can affect the unborn baby.

Manage breathlessness by pacing yourself so your breathlessness is around 4 out of 10. Consistent activity is better than ‘boom and bust.’

In cold weather do circuits inside the local mall. Breathe through your nose or wear a scarf to protect your airways from cold weather. Choose an exercise you enjoy as you’re more likely to continue with it. Lung Disease tends to be progressive so track your breathlessness over 6 months to see how it progresses.

Try drugs to see what your journey is like. How you respond to your first drug suggests how the disease will progress. Scleroderma based pulmonary hypertension has less positive outcomes than other forms of PAH.