Scleroderma: Causes, Survival Rates, and Treatment Options

Scleroderma

Hello, doctor.
I would like to ask: 1.
What is scleroderma? 2.
Is the survival rate high? What are the chances of a cure? 3.
What are the causes of the disease? Is it hereditary, or are there other factors? Thank you for taking the time to answer.
I really appreciate it.

kk, 20~29 year old female. Ask Date: 2001/08/02

Dr. Zheng Lizhen reply Dermatology

Scleroderma is a rare autoimmune disease with an unknown etiology, possibly related to genetic factors.
The skin becomes hardened and tight due to fibrosis, resembling leather.
It can be classified into: 1.
Localized scleroderma: affecting only a few localized lesions.
2.
Systemic scleroderma: which is further divided into (1) limited to the skin only, and (2) diffuse systemic involvement: where, in addition to the skin, blood vessels and internal organs such as the heart, lungs, kidneys, esophagus, gastrointestinal tract, and muscles may also exhibit similar changes.
Symptoms include cyanosis or erythema of the extremities due to cold, pulmonary hypertension, difficulty swallowing, diarrhea, changes in skin color, and fingertip ulcers, among others.
Current treatment options include anti-inflammatory medications, immunosuppressants, penicillamine, colchicine, calcitonin, vitamin A derivatives, interferons, and ultraviolet light therapy.
Additionally, it is important to maintain warmth to regulate body temperature, engage in physical rehabilitation, and pay attention to oral hygiene (as the mouth may not open fully).
The localized and limited systemic types are generally less life-threatening but may cause limitations in limb mobility; approximately half of patients experience relief within months or years, with some even achieving remission.
The diffuse systemic type is more likely to be fatal, depending on the affected areas and severity, with variations observed among different countries and ethnic groups.
The seven-year survival rate is approximately 70%, and generally, patients with kidney involvement are at higher risk of mortality.
However, with advancements in medical care, the one-year survival rate for severe kidney involvement has significantly improved from 25% to 75%.

Reply Date: 2001/08/02

More Info

Scleroderma, also known as systemic sclerosis, is a complex autoimmune disease characterized by the hardening and tightening of the skin and connective tissues. It can affect various parts of the body, including the skin, blood vessels, and internal organs. Understanding this condition involves exploring its causes, survival rates, and treatment options.

1. What is Scleroderma?
Scleroderma is primarily classified into two types: localized scleroderma and systemic scleroderma. Localized scleroderma typically affects only the skin and may resolve on its own, while systemic scleroderma can involve multiple organ systems, including the lungs, heart, kidneys, and gastrointestinal tract. The hallmark of scleroderma is the excessive production of collagen, leading to fibrosis (thickening and scarring) of the skin and internal organs.

2. Survival Rates and Treatment Options
The survival rate for individuals with scleroderma varies significantly based on the type and severity of the disease. According to various studies, the overall 5-year survival rate for systemic scleroderma can range from 70% to 90%, but this can be lower for those with severe organ involvement, particularly pulmonary complications. Advances in treatment have improved outcomes, but there is currently no cure for scleroderma.
Treatment options focus on managing symptoms and preventing complications. Common approaches include:
- Medications: Immunosuppressants (like methotrexate and mycophenolate mofetil) can help control the immune response. Other medications may include corticosteroids to reduce inflammation and vasodilators to improve blood flow.

- Physical Therapy: This can help maintain mobility and function, especially in cases where skin tightening affects movement.

- Lifestyle Modifications: Patients are often advised to avoid smoking, manage stress, and maintain a healthy diet to support overall health.

3. Causes and Genetic Factors
The exact cause of scleroderma remains unclear, but it is believed to involve a combination of genetic, environmental, and immunological factors. While there is evidence suggesting a genetic predisposition, scleroderma is not directly inherited. Certain environmental triggers, such as exposure to silica dust or certain viral infections, may also play a role in the onset of the disease.

Research indicates that women are significantly more likely to develop scleroderma than men, particularly during their reproductive years. This gender disparity suggests that hormonal factors may also influence the disease's development.

Conclusion
In summary, scleroderma is a multifaceted autoimmune disease with varying degrees of severity and impact on survival. While treatment options are available to manage symptoms and improve quality of life, ongoing research is essential to better understand the underlying causes and develop more effective therapies. If you or someone you know is dealing with scleroderma, it is crucial to work closely with a healthcare provider to tailor a treatment plan that addresses individual needs and concerns. Regular follow-ups and monitoring for potential complications are also vital for managing this complex condition.

Similar Q&A

Understanding Scleroderma: Treatment Options and Life Expectancy Insights

Is scleroderma curable? On average, how many years does it take from early detection to death? Should one avoid taking certain herbal medicines that boost immunity? Thank you, doctor!

Dr. Zeng Guosen reply Internal Medicine
Hello! Scleroderma can be categorized into two main types: idiopathic autoimmune disorders and those triggered by medications or chemicals. Except for a few cases where the cause can be identified and treated, most cases can only be managed and not cured. Generally, if the condit...

Understanding Localized Scleroderma: Treatment Options and Side Effects

Hello doctor, in recent years I have developed a large area of scleroderma on my back. After visiting the hospital, I was diagnosed with localized scleroderma. The doctor prescribed me Pimecrolimus 0.1%. I would like to know how this medication helps me manage my condition, as we...

Dr. Wu Yuxin reply Dermatology
In the early stages, corticosteroid ointments or topical immunomodulators (such as pimecrolimus) are commonly used to reduce inflammation, or vitamin D3 ointments may be used to soften the skin.

Potential Treatments for Skin Regeneration in Scleroderma Patients

Dr. Jian: A young girl was diagnosed with scleroderma over six years ago at the age of ten. Throughout this long journey of illness, her physical symptoms have stagnated in the contracture phase. Although there has been no further deterioration, it remains quite inconvenient and ...

Dr. Jian Yingxiu reply Rare Disease
Please consult a physician from the Rehabilitation Medicine, Rheumatology, or Dermatology departments. Thank you.

Managing Scleroderma: What to Do for Affected Fingers

What should I do if my fingers have scleroderma?

Dr. Zeng Guosen reply Internal Medicine
Hello! There are many reasons for skin thickening and hardening, including occupational factors, chemical agents, endocrine diseases, connective tissue diseases, and malignant diseases. As for the user mentioning scleroderma in the fingers, it is difficult to provide a clear answ...