Systemic sclerosis (SSc)

목차

1. Definition
2. Pathology
3. Clinical feature
4. Laboratory Features
5. Diagnosis
6. Treatment

본문내용

Definition Connective tissue disorder of unknown etiology Diffuse cutaneous form Thickening of skin(scleroderma) Distinctive involvement of multiple internal organs
Definition
Pathology Skin Dermal expansion and obliteration of hair follicle, sweat gland
Pulmonary arterial obliterative vasculopathy Early interstitial lung disease. Diffuse fibrosis of the alveolar septae and a chronic inflammatory cell infiltrate Pathology Lung
Clinical feature DcSSc Prominent early internal organ involvement LcSSc Long-standing Raynaud’s phenomenon Indolent skin Limited internal organ involvement → better prognosis
Clinical feature
Raynaud’s phenomenon Clinical feature Pallor Cyanosis Erythema Nail bed : widened and irregular loops, dilated lumen
Transverse creases on the dorsum of the fingers disappear. Clinical feature Skin features Skin thickening Hallmark of SSc Symmetric and bilateral Fingers → Distal →Proximal Involved skin: firm, coarse, and thickened

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