Limited systemic sclerosis vs Diffuse systemic sclerosis
How to tell Limited systemic sclerosis and Diffuse systemic sclerosis apart when a board question makes them look alike.
Limited disease is distal CREST with anticentromere and pulmonary hypertension; diffuse disease is proximal skin and early lung/renal involvement.
Side by side
Limited systemic sclerosis causes distal skin thickening and CREST features: calcinosis, Raynaud, esophageal dysmotility, sclerodactyly, telangiectasia, with pulmonary hypertension risk.
Diffuse systemic sclerosis causes widespread proximal skin thickening, early visceral disease, interstitial lung disease, renal crisis, and more rapid progression.
Autoimmune vascular injury and fibroblast activation cause fibrosis mainly in distal skin and selected organs in limited disease.
More extensive fibroblast activation and vasculopathy drive widespread skin and early internal organ fibrosis in diffuse disease.
Anticentromere antibodies support limited systemic sclerosis and pulmonary arterial hypertension risk.
Anti-topoisomerase I supports diffuse disease and interstitial lung disease; anti-RNA polymerase III supports renal crisis risk.
The trap
Both cause Raynaud and skin thickening, so distribution, antibody, and visceral timing separate them.
Watch it explained
Test it nowThe repair move
For systemic sclerosis, map skin distribution first, then match antibody and organ risk.
Try a free question
CoreA 54-year-old woman has 3 years of Raynaud phenomenon, sclerodactyly limited to fingers and distal forearms, telangiectasias on the face and hands, calcinosis on the elbows, and dysphagia for solids. Labs: anti-centromere antibody positive, anti-Scl-70 negative. Echocardiogram shows estimated pulmonary artery systolic pressure of 52 mm Hg. Which diagnosis is most likely?
Show answer and explanation
Answer: A. Limited systemic sclerosis (CREST). Limited systemic sclerosis (CREST) features distal skin involvement, Calcinosis, Raynaud, Esophageal dysmotility, Sclerodactyly, and Telangiectasias, with anti-centromere antibodies and a higher risk of pulmonary arterial hypertension.
Why the other choices are tempting
- B. Diffuse systemic sclerosis has widespread proximal skin involvement, anti-topoisomerase I/anti-Scl-70 positivity, and early visceral disease.
- C. MCTD has anti-U1 RNP and overlap features.
- D. Primary Raynaud disease has isolated Raynaud without sclerodermatous skin changes, autoantibodies, or organ involvement.
- E. Eosinophilic fasciitis has groove sign and peripheral eosinophilia, not anti-centromere positivity.
Flashcards
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What antibody supports limited systemic sclerosis?
Anticentromere antibody supports limited systemic sclerosis.
What antibody supports diffuse systemic sclerosis?
Anti-topoisomerase I supports diffuse systemic sclerosis.
Quick answers
What is the fastest way to tell Limited systemic sclerosis from Diffuse systemic sclerosis?
Limited disease is distal CREST with anticentromere and pulmonary hypertension; diffuse disease is proximal skin and early lung/renal involvement.
What is the key clue for Limited systemic sclerosis?
Limited systemic sclerosis causes distal skin thickening and CREST features: calcinosis, Raynaud, esophageal dysmotility, sclerodactyly, telangiectasia, with pulmonary hypertension risk.
What is the key clue for Diffuse systemic sclerosis?
Diffuse systemic sclerosis causes widespread proximal skin thickening, early visceral disease, interstitial lung disease, renal crisis, and more rapid progression.
What is the common trap with Limited systemic sclerosis vs Diffuse systemic sclerosis?
Both cause Raynaud and skin thickening, so distribution, antibody, and visceral timing separate them.