Systemic sclerosis, commonly known as scleroderma, is a rare but potentially devastating autoimmune disease. As osteopathic physicians, it's crucial for us to understand this condition to provide the best possible care for our patients. Let's dive into the world of systemic sclerosis and explore its complexities.
What is Systemic Sclerosis?
Systemic sclerosis is a triad of:
- Vasculopathy (blood vessel damage)
- Autoimmunity
- Fibrosis (scarring)
Think of it as a perfect storm in which the body's own immune mechanisms go haywire, attacking its own organ systems, leading to widespread symptoms.
The Hallmarks of Systemic Sclerosis
1. Sclerodactyly
Imagine the skin of your fingers thickening and becoming difficult to move, starting from the fingertips and moving proximally. That's sclerodactyly: the thickening of the skin on fingers. It is present in 95% of systemic sclerosis patients.
2. Raynaud's Phenomenon
Picture your fingers turning white, then blue, then red when cold or stressed. This is Raynaud's phenomenon, and it affects 90% of systemic sclerosis patients. It is usually adult onset, and can occur years before there are other outward systemic sclerosis symptoms. It's not just cold hands; it is a vascular response to cold temperatures or stress.
3. Positive ANA Test
Antinuclear antibodies (ANA) are markers of immune noise—although nonspecific, they tell us the body is creating antibodies in response to *something.* In systemic sclerosis, 95% of patients will have a positive ANA.
The Osteopathic Approach
As osteopathic physicians, we view the body as a unit. Systemic sclerosis affects not just the physical body, but also the mind and spirit. Patients often experience:
- Initial relief at diagnosis
- Fear of the unknown
- Depression and frustration
- Challenges in daily life and work
Our approach should be holistic, considering pharmacological treatments alongside complementary therapies like OMT, which has shown promise in improving hand function and reducing symptoms.
Systemic sclerosis is a complex challenge, but with early detection and a comprehensive, osteopathic approach, we can make a difference in our patients' lives. When in doubt, refer to a rheumatologist and/or scleroderma program specialist. Together, we can tackle this challenging disease with the goal of improving outcomes for our patients.
Learn More about Scleroderma
Through a grant partnership with the Friends of World Scleroderma Foundation (FWSF), ACOFP has posted a free, 0.5 credit CME course on scleroderma.
FWSF has a robust, free CME series hosted on the University of Michigan’s education center, which delves much deeper into scleroderma for primary care. Read more about the course and how to register.
