systemic sclerosis

Pulmonary arterial hypertension

High blood pressure in the lung arteries (pulmonary arterial hypertension) is a serious complication and a major cause of harm in systemic sclerosis.

Raynaud phenomenon

Raynaud phenomenon, episodes of color change and reduced blood flow in the fingers and toes triggered by cold or stress, is usually the earliest sign of systemic sclerosis.

Pulmonary fibrosis

interstitial lung disease, scarring (fibrosis) of the lung tissue, is the most common cause of death in systemic sclerosis and affects up to 30 percent of people with the condition.

Abnormal pulmonary interstitial morphology

lung scarring in systemic sclerosis is more common in people with the diffuse skin form of the disease or with anti-topoisomerase 1 (Scl-70) antibodies.

Dyspnea

shortness of breath (dyspnoea) and cough are the most common first signs of lung scarring in systemic sclerosis.

Digital ulcer

Painful sores at the fingertips (digital ulcers) can develop because of the reduced blood flow caused by the disease's blood-vessel damage.

Cutaneous sclerotic plaque

systemic sclerosis is an immune-mediated connective tissue disease whose defining feature is progressive hardening and thickening (fibrosis) of the skin.

Sclerodactyly

Tightening and hardening of the skin of the fingers (sclerodactyly) is a hallmark skin change, often following early puffy swelling of the fingers.

Thickened skin

Hardening and thickening of the skin, caused by excess collagen, is the defining feature of systemic sclerosis and gives the disease its name.

Acral ulceration

Reduced blood flow to the fingers can cause painful sores at the fingertips (digital ulcers) in systemic sclerosis.

Anti-centromere antibody positivity

Anticentromere antibodies in the blood are associated with the limited form of systemic sclerosis (CREST) and help confirm the diagnosis and predict its pattern.

Anti-RNA-polymerase-III-autoantibody positivity

the pattern of autoantibodies found in the blood in systemic sclerosis can predict which organs are likely to be involved and how the disease may progress.

Antinuclear antibody positivity

in systemic sclerosis, autoantibodies in the blood are important markers that help predict the clinical pattern of disease and how it may progress.

Anti-topoisomerase I antibody positivity

Anti-topoisomerase I (Scl-70) antibodies are associated with diffuse systemic sclerosis and a higher risk of lung fibrosis.

Gastroesophageal reflux

the esophagus is the most commonly affected part of the gut in systemic sclerosis, with esophageal problems such as reflux affecting about 90 percent of people; intestinal involvement affects 40 to 70 percent.

Bowel incontinence

loss of bowel control (fecal incontinence) is a common symptom of systemic sclerosis.

Mycophenolate mofetil

Mycophenolate mofetil is an immune-suppressing medicine used as a backbone treatment for the lung scarring (interstitial lung disease) that can occur in systemic sclerosis.

Used to help with: Systemic sclerosis.

nintedanib

nintedanib, an antifibrotic medicine, is recommended in current European treatment guidelines for systemic sclerosis, including for lung fibrosis (interstitial lung disease).

Used to help with: Systemic sclerosis.

mycophenolate

for systemic sclerosis-associated lung scarring (interstitial lung disease), an American Thoracic Society guideline recommends mycophenolate.

Used to help with: Systemic sclerosis.

angiotensin-converting enzyme inhibitor

angiotensin-converting enzyme (ACE) inhibitors are the mainstay treatment for scleroderma renal crisis, a life-threatening kidney complication of systemic sclerosis, and have substantially improved kidney outcomes.

Used to help with: Systemic sclerosis.

rituximab

rituximab is among the targeted therapies newly recommended in European guidelines for key fibrotic features of systemic sclerosis, including skin fibrosis.

Used to help with: Systemic sclerosis.