Scleroderma & CREST Flashcards
What are the 3 types of Scleroderma? Describe them
Diffuse = widespread, rapidly progressive skin thickening proximal to elbows & knees, early visceral Dz Limited = restricted, non-progressive Dz, affects distal extremities (elbows/knees down) Overlap = diffuse or limited scleroderma with features of other connective tissue Dz
A pt presents because she is worried about her skin which feels thickened and hardened. She also mentions fatigue, stiff joints, some loss of strength, pain, and skin discoloration. What do you suspect? What lab should you order?
Progressive Systemic Scleroderma
ANA
A pt presents with thick, hardened skin, sclerodactyly, and fatigue. Labs show he is ANA +. Is this enough information to diagnose scleroderma?
Yes Any 2 of the following are diagnostic: 1. + ANA 2. Sclerodactyly 3. Raynaud's
A pt presents with thick, hard skin, sausage-shape fingers, Raynaud’s phenomenon, and mechanic’s hand. What limited complications are you worried about?
Limited Scleroderma
Pulmonary HTN
Interstitial Lung Dz (ILD)
A pt presents with Raynaud’s, sclerodactyly, fatigue, stiff joints, loss of strength, and pain. What diffuse complications are you worried about?
Diffuse Scleroderma
Renal crisis
Pulmonary complications
Cardiac Dz
A pt presents with sclerodactyly and Rauynaud’s. Labs show he is ANA +. What treatment should you recommend for this pt?
Scleroderma
Raynaud’s: keep hands warm, CCBs, PDE-5 inhibitors, topical nitrates
Dz Modifying Agents
What 5 sx make up CREST syndrome?
Calcinosis & acrolysis Raynaud's Esophageal Dysmotility Sclerodactyly Telangestasia
A pt presents with calcinosis, Raynaud’s, esophageal dysmotility, sclerodactyly, and telangestasia. What lab test can you order to diagnose their condition?
CREST
Autoantibodies
A pt presents with Raynaud’s, sclerodactyly, acrolysis, esophageal dysmotility, calcinosis, and telangestasia. What is their prognosis?
CREST
Good, > 70% 10 yr survival
