Scleroderma: shedding light on this rare autoimmune disease


Scleroderma results in the tightening of the skin, connective tissue and internal organs. (Wikipedia pic)

PETALING JAYA: Systemic sclerosis, more commonly known as scleroderma, is a rare autoimmune disorder that affects the skin, connective tissue and internal organs.

It is a disease mostly unheard of in Malaysia, but doctors from Hospital Canselor Tuanku Muhriz UKM are hoping to create greater awareness through the Malaysian Scleroderma Association (MSA).

Its president, Dr Mohd Shahrir Mohamed Said, hopes more people with the condition will join the patient-centric organisation, which plans to help them buy expensive medications.

Shahrir tells FMT that scleroderma happens when a patient’s immune system attacks the body’s tissues. This results in an overproduction of collagen, the protein that forms the basis of connective tissue.

Symptoms include the hardening and tightening of the skin, restricted ability to move, and a small mouth.

Dr Shahrir hopes that those with the disease will join the association. (Malaysian Scleroderma Association pic)

“The patient’s skin will become so tight to the point that they are barely able to move their mouth and fingers.

“It worsens because the build-up of collagen thickens lung tissue and causes fibrosis or scarring, making the transport of oxygen in the bloodstream more difficult,” says Shahrir, who is a senior consultant physician and rheumatologist at the hospital.

Scleroderma is less known compared with related autoimmune diseases such as rheumatoid arthritis and lupus, as patients tend to be shunned by society due to their physical appearance.

Detecting scleroderma

There is currently no cure for scleroderma, while diagnosing it can be tricky because not all patients have facial skin tightening. And even when they do, it occurs in a span of four to five months, he says.

The arm of a scleroderma patient with skin lesions. (Wikipedia pic)

The first symptoms take place in the patient’s fingers and toes when they get cold. Known as Raynaud’s Phenomenon, the digits feel numb, prickly and frigid.

This is followed by difficulty swallowing and shortness of breath.

“Patients with early signs of scleroderma are referred to us, so we can move fast before the heart is affected. Once the patient has tightening in the lungs, this weakens the heart and we are unable to combat the situation,” Shahrir says.

However, by the time most patients with scleroderma are admitted, they are usually ill from a lung infection and have an increased likelihood of getting cancer, especially in the lungs, he explains.

MSA infographic showing the clinical manifestations of scleroderma.

Shahrir adds that he and his colleagues set up the MSA last year, and the pandemic has made their objectives of increasing its member base and educating the public more urgent.

“We are here to help scleroderma patients and make the government and country aware that this disease is out there,” he says.

There are currently seven doctors from Hospital Canselor Tuanku Muhriz UKM heading the association – four respiratory physicians, a cardiologist, a nephrologist, and Shahrir himself.

For more information on the Malaysian Scleroderma Association, visit their Facebook page or email them at [email protected].