1、SSc 101Julia RhiannonJune 2013julia.rhiannonva.govoutline Intro and pathogenesis Classification dcSSc diffuse cutaneous systemic sclerosisorgan involvement with 2 casesRodnan skin scoring lcSSc limited cutaneous systemic sclerosis consider digital sympathectomy if successful Early Skin Changes in Di
2、ffuse SSc Initially skin may be edematous Hands only early May have CTS Edema resolves; skin then indurated Extremities, face, trunk may be involved Advancing skin involvement often pruriticLate Skin Changes As disease progresses, skin becomes atrophic Ulcers may develop at pressure points or with t
3、rauma Disappearance of finger tips due to resorption or gangrene Secondary joint contractures Other skin changes OtherThickened and tetheredTelangiectasiaCalcinosisNarrowed oral apertureCase 2 44 y/o male with known dcSSc admitted to MICU for BP 210/150 creatinine 1.5 mg/dl. UA with +2 protein Smear
4、 with schistocytesNext step is to give:1. plasma exchange2. IV Solu-Medrol 1 mild; 2 moderate; 3 severe thickening summation of 17 body areasFaceUpper armAnteriorchestForearmHandFingersThighLegFootUpper armAbdomenForearmHandFingersThighLegFootUninvolvedMild thickeningModerate thickeningSevere thicke
5、ning0123Clinical Assessment of Skin Thickening-Modified Rodnan Skin Score (MRSS)Clinical Meaningfulness of Change in MRSS Increase predicts worsening of SSc Stability predicts reduced new internal involvement Improvement predicts improved 5 and 10 year survival High scores (20) predict renal crisis (OR 6.64) High scores (20) predict mortality (OR 3.39) Improved score correlates with HAQ-DI, hemoglobin
