Antiscleroderma-70 Antibodies

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Scl-70 antibody is seen in 20% of people with scleroderma (also known as systemic sclerosis), and in some people with CREST syndrome (calcinosis, Raynaud, esophageal dysfunction, sclerodactyly, telangiectasia).

These syndromes are also associated with a high frequency of speckled pattern immunofluorescent antinuclear antibody tests. Scl-70 may identify a subset of scleroderma patients with severe skin, joint, and lung disease. In addition, the presence of Scl-70 in Raynaud phenomenon may indicate a poor prognosis.

Negative results do not necessarily rule out the presence of SSc. If clinical suspicion remains, consider further testing for centromere, RNA polymerase III and U3-RNP, PM/Scl, or Th/To antibodies.

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Progressive Systemic Sclerosis (PSS), is a chronic systemic rheumatic disease that can effect many organ systems including the skin, the GI tract, the heart, lungs and kidneys. It occurs more commonly in females and its onset most frequently in the ages from 20 to 50 years. Sera (=plural of serum) from people with PSS contain several autoantibodies to different nuclear antigens including Scl-70, Sm and centromere antigens. Antibody to Scl-70 (topoisomerase I) is present in around 20% of patients with scleroderma, particularly those patients with a more progressive form of the disease. Scl-70 is difficult to detect by immunofluorescent studies where it gives fine speckles within the nucleus. Scl-70 antigen is present in the ENAscreen. Samples positive for the ENAscreen but negative for Ro, La, RNP, Sm or the centromere antigen will automatically be tested for Scl-70. However, since many Scl-70 sera are also positive for other ENAs (Ro52or RNP) it is best to specifically request Scl70 or give scleroderma as clinical details where appropriate.

What does it mean if your Antiscleroderma-70 Antibodies result is too high?

Understanding Elevated Scl-70 Antibodies: A Comprehensive Guide

Quick Summary

An elevated Antiscleroderma-70 (Scl-70) antibody level may indicate systemic sclerosis (scleroderma) or other autoimmune conditions. This guide explains your test results, potential implications, and next steps for proper medical care.

Primary Concern: Possible systemic sclerosis or related autoimmune condition

What Does an Elevated Antiscleroderma-70 Antibody Result Mean?

The presence of Scl-70 antibodies is most commonly associated with systemic sclerosis, a chronic autoimmune disease characterized by the abnormal growth of connective tissue. Systemic sclerosis can affect the skin and internal organs, leading to a range of symptoms and potential complications. While the Scl-70 antibody is most commonly linked to this condition, an elevated level does not necessarily confirm a diagnosis of systemic sclerosis. Instead, it suggests that your body may be experiencing an autoimmune response, and further evaluation is typically needed.

Possible Diagnoses

→ Systemic Sclerosis (Scleroderma): Scl-70 antibodies are specifically associated with diffuse cutaneous systemic sclerosis, a more severe form of scleroderma that can involve widespread skin thickening as well as organ involvement (such as the lungs, kidneys, and heart).

→ Other Autoimmune Conditions: Although less common, Scl-70 antibodies can sometimes be present in other autoimmune diseases or mixed connective tissue diseases, which share some symptoms with systemic sclerosis but may have a different prognosis and treatment approach.

Symptoms of Systemic Sclerosis

If systemic sclerosis is suspected, the symptoms can vary widely, but may include:

- Skin thickening and hardening (usually starting in the fingers, hands, and face)

- Joint pain and stiffness

- Raynaud's phenomenon (a condition where the fingers and toes turn white or blue in response to cold or stress)

- Gastrointestinal issues, such as acid reflux and difficulty swallowing

- Shortness of breath (if the lungs are involved)

- Kidney problems or elevated blood pressure (if the kidneys are affected)

The severity and combination of symptoms can vary from person to person, and some people with systemic sclerosis have only limited skin involvement and fewer systemic symptoms.

Treatment Options

While there is no cure for systemic sclerosis, treatment focuses on managing symptoms, slowing disease progression, and improving quality of life. Treatment plans are typically personalized based on the organs involved and the severity of symptoms.

Medications:

- Immunosuppressants

- Corticosteroids (used cautiously)

- Vasodilators for Raynaud's phenomenon

- Antifibrotic agents for lung involvement

Physical and Occupational Therapy:

- Stretching and mobility exercises

- Joint protection techniques

- Adaptive equipment training

Lifestyle Modifications:

- Avoiding cold exposure and stress

- Dietary modifications for gastrointestinal symptoms

- Regular exercise as tolerated

- Smoking cessation if applicable

Next Steps

Immediate Actions

  1. Schedule an appointment with a rheumatologist
  2. Document any current symptoms
  3. Prepare questions for your healthcare provider

Additional Testing May Include

- Comprehensive blood work

- Chest X-ray or CT scan

- Pulmonary function tests

- Skin biopsy if needed

Support Resources

Professional Organizations:

- Scleroderma Foundation: www.scleroderma.org

- National Organization for Rare Disorders (NORD): www.rarediseases.org

- Arthritis Foundation: www.arthritis.org

Support Groups

- Local scleroderma support groups (check with Scleroderma Foundation)

- Online communities and forums

- Hospital-based support programs

References:

- American College of Rheumatology Guidelines for Systemic Sclerosis

- UpToDate: "Clinical manifestations and diagnosis of systemic sclerosis (scleroderma) in adults"

- Rheumatology Network: "Diagnosis and Management of Systemic Sclerosis"

- Laboratory Corporation of America (LabCorp) Reference Ranges

Note: This guide is for informational purposes only and should not replace professional medical advice. Always consult with your healthcare provider for personalized medical guidance.

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