What organs are affected by scleroderma? An expert guide

September 25, 2025 •

4 min read

According to the National Scleroderma Foundation, approximately 300,000 people in the United States have scleroderma. This autoimmune condition goes far beyond skin-deep, and understanding what organs are affected by scleroderma is critical for both patients and healthcare providers. The disease's systemic nature means it can cause widespread and sometimes life-threatening complications.

Quick Summary

Scleroderma, particularly its systemic form, can cause fibrosis and damage to major internal organs, including the lungs, heart, kidneys, and entire gastrointestinal tract, leading to impaired function and serious complications.

Key Points

Lungs: Scleroderma can cause Interstitial Lung Disease (scarring) and Pulmonary Arterial Hypertension (high blood pressure in lung arteries), which are major causes of mortality.
Heart: The heart muscle can develop fibrosis, leading to irregular heartbeats, heart failure, and inflammation of the heart's protective sac.
Kidneys: A life-threatening complication called scleroderma renal crisis can cause a rapid onset of severe hypertension and kidney failure, predominantly in those with diffuse scleroderma.
Gastrointestinal Tract: The entire GI tract can be affected, causing difficulty swallowing, acid reflux, bloating, and issues with nutrient absorption.
Blood Vessels and Connective Tissues: Excess collagen leads to thickened skin and damaged blood vessels throughout the body, contributing to symptoms like Raynaud's phenomenon and joint stiffness.

Scleroderma's systemic impact on internal systems

Scleroderma, or systemic sclerosis, is an autoimmune disease where the immune system attacks healthy tissue, causing an overproduction of collagen. This excess collagen builds up, leading to fibrosis (scarring) and hardening of tissues throughout the body. While the skin is the most visible target, the real danger lies in how this process affects internal organs. The severity and speed of organ involvement can vary dramatically depending on the specific subtype of scleroderma, with diffuse cutaneous systemic sclerosis often progressing more rapidly than the limited form. Early and accurate diagnosis of organ involvement is vital for managing symptoms and preventing irreversible damage.

The devastating effects on the lungs

Lung complications are among the most serious and common issues for those with systemic scleroderma, often becoming the leading cause of death in these patients. The disease can manifest in two primary ways:

Interstitial Lung Disease (ILD): Affecting about 60% of patients, ILD causes scarring of the lung tissue, known as pulmonary fibrosis. This scarring stiffens the lungs, making it difficult for them to expand and absorb oxygen. Symptoms include shortness of breath, a persistent dry cough, and fatigue.
Pulmonary Arterial Hypertension (PAH): This condition causes high blood pressure in the arteries that carry blood from the heart to the lungs. The thickening of the vessel walls restricts blood flow, forcing the heart to work harder. PAH can lead to shortness of breath, chest pain, and heart failure. Early screening via regular echocardiograms is essential for detection.

Cardiovascular complications

Scleroderma's impact on the heart can significantly affect quality of life and longevity. The disease can cause fibrosis of the heart muscle and surrounding tissues. Common heart-related issues include:

Myocardial fibrosis: Scarring of the heart muscle can impair its ability to pump blood effectively, leading to heart failure.
Arrhythmias: Damage to the heart's conduction system can cause irregular heartbeats, which may be detected with an electrocardiogram.
Pericarditis: Inflammation of the sac surrounding the heart, known as the pericardium, can cause chest pain.
Cardiac Raynaud's phenomenon: Similar to the cold-induced constriction in fingers, coronary blood vessels can spasm, potentially leading to chest pain.

Gastrointestinal (GI) tract involvement

From the esophagus to the colon, scleroderma can affect the entire digestive system due to fibrosis and nerve damage. Over 90% of scleroderma patients experience some form of GI issues.

Esophagus: The lower esophageal sphincter can weaken, leading to severe acid reflux and heartburn. Damage to the esophagus's muscles can make swallowing difficult (dysphagia).
Stomach: Gastroparesis, or delayed stomach emptying, can cause nausea, bloating, and early satiety.
Intestines: Altered motility can lead to small intestinal bacterial overgrowth (SIBO), causing bloating, diarrhea, and poor nutrient absorption. Scarring can also cause chronic constipation or pseudo-obstruction.

Kidney concerns and scleroderma renal crisis

Kidney involvement is a less common but extremely serious complication, particularly for those with diffuse scleroderma. Scleroderma renal crisis (SRC) is a life-threatening emergency characterized by a sudden and rapid onset of severe hypertension and acute kidney failure.

Cause: It results from damage to the small blood vessels in the kidneys, leading to narrowing and reduced blood flow.
Early detection: Frequent blood pressure monitoring is crucial, especially in high-risk patients. The development of aggressive high blood pressure is the key warning sign.
Treatment: The advent of angiotensin-converting enzyme (ACE) inhibitors has dramatically improved outcomes, but prompt treatment is essential to preserve kidney function and save lives.

Other affected organs

Beyond the major systems, scleroderma can cause issues in other parts of the body:

Muscles and joints: Inflammation and fibrosis can lead to joint pain, stiffness, and weakness in the muscles.
Blood vessels: The disease damages small blood vessels throughout the body, contributing to Raynaud's phenomenon and other complications.
Teeth: Tightening of the facial skin can restrict the mouth's opening, making dental hygiene difficult and increasing the risk of dental decay.

Comparison of systemic scleroderma subtypes


Feature	Limited Cutaneous Systemic Sclerosis (lcSSc)	Diffuse Cutaneous Systemic Sclerosis (dcSSc)
Skin Involvement	Thickening on hands, forearms, feet, legs, and face. Progression is typically slow.	Rapid, widespread thickening affecting the trunk and proximal limbs, in addition to hands and feet.
Internal Organs	Often presents much later in the disease course, if at all. PAH is a key concern.	Can involve multiple internal organs early and often progresses rapidly. Higher risk of SRC and severe ILD.
Associated Syndrome	Formerly known as CREST syndrome (Calcinosis, Raynaud's, Esophageal dysmotility, Sclerodactyly, Telangiectasias).	Higher risk of renal crisis and rapid-onset lung disease.
Prognosis	Generally has a better long-term prognosis than dcSSc, though serious complications like PAH still pose a risk.	The more severe form, often with more rapid progression and a higher risk of serious organ complications.

The importance of a comprehensive treatment plan

Given the potential for widespread organ damage, managing scleroderma requires a multi-faceted and personalized approach. Treatment plans often include immunosuppressive therapies, medications to address specific organ complications, and regular monitoring. Early detection of internal organ involvement through regular screenings is key to mitigating risk and improving outcomes. For further information and support, consider visiting the Scleroderma Research Foundation to learn about the latest research and patient resources.

Conclusion

Scleroderma is a complex and potentially severe disease that extends far beyond its visible effects on the skin. By affecting vital organs such as the lungs, heart, kidneys, and digestive system, it poses significant health risks. A proactive, collaborative approach involving rheumatologists and other specialists is essential for early detection, symptom management, and improved quality of life for individuals living with this condition.

Frequently Asked Questions

Localized scleroderma primarily affects the skin and underlying tissues, while systemic scleroderma (or systemic sclerosis) is more severe because it can also impact major internal organs such as the heart, lungs, and kidneys.

Scleroderma can affect the lungs in two main ways: by causing interstitial lung disease (scarring of lung tissue) and pulmonary arterial hypertension (high blood pressure in the arteries of the lungs), both of which can impair breathing.

Yes, scleroderma can lead to various heart problems, including scarring of the heart muscle (fibrosis), irregular heartbeats, and inflammation of the sac around the heart. These issues can contribute to heart failure.

Scleroderma renal crisis is a serious and potentially fatal complication that occurs in some people with scleroderma, typically presenting as a sudden and severe increase in blood pressure and rapid kidney failure.

Yes, scleroderma often affects the digestive system, from the esophagus to the intestines. This can result in symptoms such as acid reflux, difficulty swallowing, bloating, constipation, and nutrient malabsorption.

While some scarring caused by scleroderma may not be reversible, timely and aggressive treatment can help manage symptoms, slow down the progression of the disease, and prevent further organ damage. Early detection is crucial for better outcomes.

Yes, diffuse cutaneous systemic scleroderma is more likely to involve internal organs and often progresses more rapidly than limited cutaneous systemic sclerosis. Limited forms can still cause serious complications like pulmonary arterial hypertension.