Dr. Shane Shapera
Program Director of the Interstitial Lung Disease Clinic, Toronto General Hospital
Advances in medical treatments provide hope for patients with scarring of the lungs caused by scleroderma.
While the cause of scleroderma is unknown, it’s believed that a genetic predisposition combined with a stressful event, such as an infection, triggers your immune system to attack itself, says Dr. Shane Shapera, Program Director of the Interstitial Lung Disease Clinic at Toronto General Hospital.
Scleroderma most often affects women between the ages of 30 and 50. Symptoms vary depending on what area of the body the disease affects, such as the skin, muscles, blood vessels, or internal organs. Symptoms can include hardening of the skin, painful joints, heartburn, nausea, and fatigue.
When scleroderma affects the lungs, it causes pulmonary hypertension, leading to shortness of breath, fatigue, and chest pain, or pulmonary fibrosis, in which the lungs become scarred and breathing becomes increasingly difficult.
“The scarring is progressive and so patients with scleroderma will get worse,” says Dr. Shapera. “We think that about half of patients with scleroderma will get pulmonary fibrosis, which is one of the leading causes of death in people with scleroderma.”
There’s no cure for pulmonary fibrosis and treatments seek to relieve the symptoms. In 2019, Health Canada approved the first treatment shown to slow the speed of lung scarring. It’s expensive and remains difficult to access, but as the Canadian Agency for Drugs and Technologies in Health (CADTH) has recommended that provincial health plans cover treatment costs for patients with progressive fibrotic interstitial lung disease (ILD) and scleroderma, Dr. Shapera is optimistic that the Government of Ontario will start to cover the drug under its provincial drug plan.
Experts and professionals in the space are advocating strongly for provincial coverage as soon as possible. “We can’t wait much longer. This treatment shifts the paradigm in how we treat patients with pulmonary fibrosis,” says Dr. Shapera. “It offers hope of slowing down the disease.”
This article was made possible with support from a leading research-based pharmaceutical company.