Dr Richard Steele: Overview of treatment for systemic sclerosis

It’s not great to have scleroderma and its associated ailments but thank your stars you are living now and not a couple of hundred years ago.

Dr Richard SteeleFor then, Dr Richard Steel says, doctors probably killed more patients than they successfully treated.

Dr Steele is a clinical immunologist in Wellington and says modern medicine bases itself on clinical trials and high quality evidence.

Doctors and medical researchers grade the quality of evidence according to how rigorously they gathered it, ranging from level 1: high quality randomised trials across many studies down to 5: expert opinion.

Treatment for scleroderma is based on this evidence.

Scleroderma’s problem is that it’s relatively rare so it’s difficult to recruit enough people for an authoritative trial. Scleroderma also manifests itself in so many different ways, Dr Steele says, so how do you know that treatments will work for all sufferers equally?

He says doctors have only a moderate understanding of the treatment because the disease attacks in so many different ways and can start slowly and lessen equally slowly. It’s difficult in a trial to measure the efficacy of a treatment. But through it all, the evidence-based approach is paramount.

Trials for treatment are more and more becoming multi-national so there are greater numbers of patients to test. However, a cure is a long way off, he says, because of the slow progress to understand it coupled with its rarity.

Treatment day-to-day for patients with scleroderma starts with doctors evaluating the possible symptoms:

  • Raynaud’s.
  • skin sores,
  • organ involvement,
  • overlap with other auto-immune diseases and
  • a hunt for the tell-tale auto anti-body profile associated with some sub-groups of systemic sclerosis.

Because scleroderma varies in its effects so much, doctors tailor their treatment to the individual.

It begins with an inflammatory period, which lasts for several years and after that Dr Steele says it becomes more difficult to treat. So he says doctors focus of early treatments on the inflammatory part of the condition.

“If we can treat that aggressively and effectively then maybe we get less of the fibrosis and the other more permanent problems associated with systemic sclerosis,” he says. Early treatment often depends on the GP recognising what their patient has and referring them to a specialist or the patient being assertive enough to have the possibility of scleroderma examined.

Dr Steele ran through the various ways that scleroderma manifests itself and the types of drugs and treatments brought to bear against it. He explained cases in which a range of immunosuppressives are used to treat the disease from its outset and as it progresses.

He covered Raynaud’s, which he says is not just about hands and feet but about core body temperature. A life in the tropics should fix the temperature but that’s not possible so patients should always dress warmly, never smoke and try to avoid stress.

As if to illustrate the variety of ways in which scleroderma attacks the body, Dr Steele ranged over potential effects on the heart, lungs, kidneys and even erectile dysfunction. He canvassed the treatment, types of drugs and severity with which it can affect a patient.

We may not live with the seat-of-the-pants medicine of 200 years ago but the breadth and scope of scleroderma is still a big challenge for modern medicine. A cure eludes it but Dr Steele says significant progress has been made with a greater variety of treatments and better scientific evidence to guide us.