systemic scleroderma

Systemic scleroderma, also known as systemic sclerosis, is a chronic autoimmune disease that affects the skin and internal organs [1]. It is characterized by the deposition of collagen in the skin and other tissues, leading to thickening and hardening of the skin, as well as involvement of various internal organs such as the lungs, heart, kidneys, and gastrointestinal tract.

Symptoms:

• Skin thickening and tightening, often accompanied by a decrease in skin elasticity
• Raynaud's phenomenon (discoloration of fingers and toes in response to cold or stress)
• Limited mobility and stiffness due to skin tightness
• Fatigue and weakness
• Shortness of breath and coughing due to lung involvement
• Swallowing difficulties and heartburn due to esophageal involvement

Causes:

The exact cause of systemic scleroderma is unknown, but it is

Systemic Scleroderma Signs and Symptoms

Systemic scleroderma, also known as systemic sclerosis, is a chronic autoimmune disease that affects the skin and internal organs. The symptoms of systemic scleroderma can vary from person to person, but here are some common signs and symptoms:

• Raynaud's phenomenon: This is often one of the earliest signs of systemic scleroderma. It causes the blood vessels in your fingers and toes to contract in response to stress or cold temperatures, leading to discoloration (white, red, and/or blue) [11][13].
• Skin thickening: Systemic scleroderma can cause skin thickening on various parts of the body, including the fingers, hands, face, lower arms, and shins. The affected skin may appear lighter or darker than usual [12][14].
• Finger stiffness and tightness: As systemic scleroderma progresses, the skin on your fingers and toes can become stiff and tight, leading to limited mobility [3][6].
• Digestive system problems: Some people with systemic scleroderma may experience digestive system issues, such as difficulty swallowing (dysphagia), heartburn, diarrhea, constipation, or a bloated feeling after eating [2][8].
• Muscle and joint pain: Systemic scleroderma can cause muscle and joint pain, which can be widespread or localized to specific areas [6].
• Dilated blood vessels: In some cases, systemic scleroderma can lead to dilated blood vessels that can be seen through the skin [6].
• Kidney involvement: Systemic scleroderma can affect the kidneys, leading to mild or serious complications, including high blood pressure (hypertension), protein in the urine, and blood test abnormalities. In severe cases, it can lead to renal crisis, a life-threatening complication [10].

It's essential to note that not everyone with systemic scleroderma will experience all of these symptoms, and their severity can vary from person to person.

References: [1] - Symptoms [2] - When scleroderma affects internal organs [3] - Systemic sclerosis symptoms [4] - Early Symptoms of Scleroderma [5] - Systemic scleroderma, also called systemic sclerosis [6] - Other manifestations of systemic scleroderma include muscle and joint pain, skin tightening, and dilated blood vessels [7] - Kidney or renal involvement in systemic scleroderma may be mild or very serious in nature [8] - The symptoms of systemic scleroderma may include: Skin thickening on your fingers, hands, face, lower arms, and shins [9] - What are the early signs and symptoms of systemic sclerosis? [10] - Systemic scleroderma can lead to pulmonary fibrosis (increased tissue in the lung that can cause breathing difficulties) [11] - Diffuse cutaneous systemic scleroderma. ... Raynaud’s phenomenon is one of the earliest signs of SS. [12] - The symptoms of systemic scleroderma may include: Skin thickening on your fingers, hands, face, lower arms, and shins [13] - What are the early signs and symptoms of systemic sclerosis? Often, the first symptom of systemic sclerosis (systemic scleroderma) is Raynaud’s phenomenon. [14] - Systemic scleroderma, also called systemic sclerosis, affects many parts of the body. This is the more serious type of scleroderma and can damage your blood vessels and internal organs, such as the heart, lungs, and kidneys.

Diagnosing Systemic Scleroderma: A Comprehensive Overview

Systemic scleroderma, also known as scleroderma, is a complex autoimmune disease that affects various parts of the body. Diagnosing this condition can be challenging, and healthcare providers often use a combination of tests to determine the presence and extent of the disease.

Tests Used in Diagnosis

• Electromyography/Nerve Conduction Velocity Testing: This test is used when muscle involvement is suspected. Abnormal results may warrant further testing, such as a muscle biopsy.
• **High-Resolution CT (HR

Treatment Options for Systemic Scleroderma

Systemic scleroderma, also known as diffuse scleroderma, is a chronic autoimmune disease that affects the skin and internal organs. While there is no cure for this condition, various medications can help manage symptoms and slow down disease progression.

Medications Used in Treatment

Several medications have been demonstrated to be effective in treating systemic scleroderma:

• Immunosuppressants: These medications can help reduce inflammation and slow down disease progression. Examples include:
  • Cyclophosphamide [5, 13]
  • Methotrexate [14]
  • Prednisone [7, 14]
• Anti-inflammatory agents: These medications can help reduce inflammation and manage symptoms.
  • Nintedanib [6, 9]
  • Tocilizumab [6, 9]
• Immunomodulators: These medications can help modify the immune system's response to the disease.
  • Mycophenolate mofetil (Cellcept) and mycophenolate sodium (Myfortic) [8]

Treatment Goals

The primary goal of treatment is to manage symptoms, slow down disease progression, and improve quality of life. Treatment plans are tailored to individual patients based on their specific needs, type of scleroderma, age, and overall health.

Important Considerations

It's essential to note that no current conventional treatment can stop or reverse the overall course of systemic scleroderma [13]. Regular monitoring for internal organ complications is also crucial for patients with systemic scleroderma [4].

References:

[1] Roofeh D, Lescoat A, Khanna D. Emerging drugs for the treatment of scleroderma: a review of recent phase 2 and 3 trials. Expert Opin Emerg Drugs 2020;1–12.

[2] Roofeh D, Khanna D. Management of systemic sclerosis: the first five years. Curr Opin Rheumatol 2020;32:228–237.

[3] Ferri FF. Scleroderma (Systemic sclerosis). In: Ferri's Clinical Advisor 2024. Elsevier; 2024.

[4] The key to feeling better is to tailor the scleroderma treatment to meet the specific needs, taking into account the type of scleroderma, age, and overall health.

[5] Cyclophosphamide has been demonstrated in well-designed scientific studies either to slow down the progression of specific existing symptoms or to reduce the development of new symptoms.

[6] Nintedanib and tocilizumab are approved by the US Food and Drug Administration to slow the rate of decline in pulmonary function in adults with interstitial lung disease, which is a common complication of systemic scleroderma.

[7] Prednisone can help reduce inflammation and manage symptoms.

[8] Mycophenolate mofetil (Cellcept) and mycophenolate sodium (Myfortic) may be especially helpful for people with skin or lung involvement.

[9] Nintedanib and tocilizumab have been shown to slow down disease progression in patients with systemic scleroderma.

[10] Autologous haematopoietic stem cell transplantation can improve survival in patients with early diffuse cutaneous systemic sclerosis.

[11] Treatment of systemic sclerosis (SSc) is organ-based or aimed at disease modification.