From the Guidelines
Scleroderma findings typically include a combination of skin, vascular, and internal organ manifestations, such as skin thickening, Raynaud's phenomenon, digital ulcers, and interstitial lung disease, which are crucial to identify for timely management and prevention of complications. The clinical presentation of scleroderma can vary widely among patients, but common features include 1:
- Skin thickening and hardening, especially of the fingers and face
- Raynaud's phenomenon, characterized by color changes in fingers and toes in response to cold
- Telangiectasias, which are small dilated blood vessels visible on the skin
- Calcinosis, or calcium deposits under the skin
- Digital ulcers, which can be painful and prone to infection Internal organ involvement may present with a range of symptoms, including 1:
- Dysphagia and reflux due to esophageal dysfunction
- Shortness of breath from interstitial lung disease or pulmonary hypertension
- Cardiac abnormalities, such as arrhythmias and heart failure
- Renal crisis, characterized by hypertension and kidney failure Laboratory findings often include positive antinuclear antibodies (ANA), with specific autoantibodies like anti-Scl-70 or anti-centromere antibodies, which can help guide diagnosis and treatment 1. Nailfold capillaroscopy is also a useful diagnostic tool, typically showing dilated and distorted capillaries in patients with scleroderma 1. These findings reflect the underlying pathophysiology of scleroderma, which involves excessive collagen production, vascular damage, and autoimmune dysregulation, highlighting the importance of early recognition and prompt treatment to manage symptoms and prevent complications 1.
From the Research
Scleroderma Findings
- Scleroderma renal crisis (SRC) is a life-threatening complication that affects 2-15% of patients with systemic sclerosis (SSc) 2.
- SRC is characterized by an acute onset of severe hypertension, acute kidney injury, and thrombotic microangiopathy in about 50% of patients 2.
- The prognosis of SRC has improved with the introduction of angiotensin-converting-enzyme inhibitors for rapid blood pressure control, but patient survival is still a concern, with 70-82% survival at 1 year and 50-60% at 5 years despite dialysis support 2.
- Early diagnosis and initiation of treatment with angiotensin-converting enzyme inhibitors (ACE-I) are crucial for a better prognosis, and there is no role for using ACE-I prophylactically in high-risk patients 3.
- Scleroderma renal crisis can occur in combination with other complications, such as pulmonary hypertension, and patients with SRC who develop pulmonary hypertension are at increased risk of mortality 4.
Clinical Presentations
- Systemic sclerosis can present with various clinical manifestations, including renal involvement, pulmonary hypertension, and skin fibrosis 5.
- Renal involvement in systemic sclerosis can range from scleroderma renal crisis to other forms of renal disease, such as vasculitis and proteinuria 6.
- Patients with systemic sclerosis are at risk of developing multiple organ involvement, and early detection and risk stratification are essential for optimal management 5.
Management and Treatment
- The management of scleroderma renal crisis involves the use of angiotensin-converting enzyme inhibitors to control blood pressure, and additional therapeutic agents may be necessary for patients who are refractory to ACE-I 3.
- Renal transplantation may be considered in selected patients with scleroderma renal crisis, especially those on long-term dialysis 3.
- A holistic approach is required when choosing the optimal therapeutic strategy for patients with systemic sclerosis, balancing the side-effect profile with efficacy and tailoring the treatment according to the goals of care of the patient 5.