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Published on: June 16, 2020
Lower extremity ulcers in systemic sclerosis: features and response to therapy
Victoria K Shanmugam1, Patricia Price, Christopher E Attinger
1Division of Rheumatology, Immunology and Allergy, Georgetown University Hospital, 3800 Reservoir Road, N.W., Hington, DC 20007, USA.
Nondigital lower extremity ulcers affect 4.0% of scleroderma patients. Screening for antiphospholipid antibodies and genetic prothrombotic states is recommended for these patients.
Area of Science:
- Rheumatology
- Dermatology
- Vascular Medicine
Background:
- Nondigital lower extremity ulcers are a challenging complication of scleroderma.
- These ulcers contribute significantly to patient morbidity.
Purpose of the Study:
- To determine the prevalence of nondigital lower extremity ulcers in scleroderma patients.
- To investigate associations between these ulcers and autoantibodies or genetic prothrombotic states.
Main Methods:
- A cohort of 249 scleroderma patients was evaluated.
- Prevalence, clinical characteristics, autoantibody status, and genetic prothrombotic profiles were assessed.
Main Results:
- The prevalence of active ulcers was 4.0% (10 out of 249 patients).
- Diffuse scleroderma patients developed ulcers earlier than limited scleroderma patients (4.05 vs. 22.83 years).
- 70% of ulcer patients had positive genetic prothrombotic screens, and 50% had positive antiphospholipid antibodies.
Conclusions:
- Fibrin occlusive vasculopathy was present in all biopsied ulcer patients.
- Increased prevalence of antiphospholipid antibodies and genetic prothrombotic states suggests a link to ulcer development.
- Screening scleroderma patients with lower extremity ulcers for these conditions is advised.
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Position the patient at a thirty- to forty-five-degree angle or in a semi-fowler's position. Look for the highest point of pulsation in the internal jugular vein and measure the vertical distance to the angle of Loius or sternal angle. A normal JVP is 3-4 cm above the...