scleroderma

progressive systemic sclerosis (PSS)

hypertrophy then atrophy of collagen fibers
4-6th decade, M:F = 1:3
bones
- punctate soft tissue ca++ (finger tips, shoulder, hips)
- acro-osteolysis (63%)
- intercarpal joint space narrowing (late)
chest
- evident in 10-25%
- pulmonary fibrosis with diffuse reticulate infiltrate
- predominantly in lower lungs
GI
- esophageal dilation and aperistalsis (>50%)
- hiatus hernia + GE reflux + esophagitis + distal esophageal stricture
- gastroparesis
- dilation and dysmotility of small bowel
- pseudosacculation and dysmotility of colon

Yong H. Hahn, MD - 2 February 1995
Last updated: 1 October 2013