Scleroderma is a rare and chronic condition caused by the immune system mistakenly attacking healthy connective tissue. The body responds by overproducing a protein called collagen, resulting in the connective tissue tightening and hardening. The connective tissue provides structural integrity and strengthens tissues. It is mainly found under the skin, but is also an important component of different organs and blood vessels.
In localized scleroderma, the skin is usually the only tissue affected, but in the more serious systemic scleroderma, a variety of organs can be damaged. This can lead to a scleroderma patient developing a range of other conditions.
Pulmonary arterial hypertension (PAH)
Pulmonary arterial hypertension (PAH), or high pressure in the blood vessels of the lungs, is a common complication of systemic scleroderma, occurring in about 40 percent of patients. It is more common in patients with limited cutaneous scleroderma.
Scleroderma can also cause inflammation and scarring in the blood vessels of the lungs. The damage can trigger vasoconstriction, or the narrowing of the blood vessels, which makes it more difficult for blood to flow and increases blood pressure in the arteries.
PAH can be difficult to diagnose initially as common early symptoms, such as shortness of breath leading to an increasing inability to exercise, are often overlooked. Although there is no cure for PAH, there is a range of therapies approved to help manage the symptoms of the condition.
Kidney problems
Systemic scleroderma can often affect the kidneys and, in severe cases, may lead to renal crisis, which can result in kidney failure if left untreated.
Renal crisis may be difficult to identify as the early symptoms, such as an increase in blood pressure or the abnormal presence of protein in the urine, may be seen in all scleroderma patients. Renal crisis can come on suddenly and is normally associated with a rapid increase in blood pressure that can cause headaches, blurred vision, or even seizures.
Other symptoms of kidney involvement include nausea, vomiting, shortness of breath, a fast heartbeat, and chest pains.
Kidney involvement is usually associated with diffuse systemic scleroderma and is only rarely seen in limited scleroderma.
Muscle and joint problems
Muscle weakness, especially in the upper arms and thighs, can occur in scleroderma patients due to different causes, including damage from inflammation or muscle fibrosis (scarring), or wasting from malnutrition due to gastrointestinal problems. Muscle weakness can also occur due to other conditions, such as hyperthyroidism, or due to medications, such as corticosteroids.
Scleroderma can also cause arthritis-like symptoms from inflammation in the joints. This can cause pain, stiffness, swelling, warmth, and tenderness in the joints.
A common characteristic of systemic scleroderma is sclerodactyly. When fibrotic tissue builds up in the skin of the hands, the joints can stiffen, leading to an inability to bend or straighten the fingers.
Interstitial lung disease (ILD)
All types of systemic sclerosis can result in inflammation and scarring of the lung tissue causing interstitial lung disease (ILD). The scarring results in the lung tissue becoming stiffer and thicker, which restricts lung function and reduces the volume of air that can enter and leave the lungs. As a result, patients with scleroderma and ILD can be short of breath, and as the disease progresses, find simple daily tasks difficult to complete.
Scar tissue in the lungs may also restrict and damage the blood vessels of the lungs, leading to pulmonary hypertension.
Sjögren’s syndrome
Sjögren’s syndrome is another type of autoimmune condition that commonly occurs alongside systemic sclerosis. It affects fluid-producing glands, causing inflammation and impairing their function. Sjögren’s syndrome commonly leads to dry eyes and mouth as the saliva- and tear-producing glands are affected. Several treatments are available to manage the symptoms, including eye drops, saliva substitutes, or saliva stimulants.
Sexual dysfunction
About 80 percent of men with systemic scleroderma experience erectile dysfunction, due to damage to the blood vessels supplying the penis. In some cases, men with scleroderma may experience Peyronie disease, where inflammation and scar tissue formation as a result of scleroderma cause erections to be painful and severely curved.
Women with scleroderma can experience sexual discomfort, if they also have Sjögren’s syndrome, as this can affect the mucus-producing membrane of the vagina. This results in less lubrication when aroused, which can cause intercourse to be uncomfortable or painful.
Other conditions
There are potential associations between scleroderma and several other conditions, including:
- Erythromelalgia, where patients experience flare-ups of redness, heat, and a burning sensation in the hands, arms, legs, and face.
- Fibromyalgia, where patients experience a continuous pain throughout the body.
- Increased risk of pulmonary embolism, where blood clots block the blood vessels of the lungs.
- Other autoimmune conditions, such as lupus and rheumatoid arthritis.
- Vasculitis, which causes inflammation and damage to blood vessels, resulting in restricted blood flow to the affected tissues.
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