Systemic Sclerosis Flashcards
pathogenesis
excessive collagen deposition - skin and internal organ changes vasomotor disturbances (raynauds) fibrosis subsequent atrophy of the skin and subcutaneous tissue 3 phases of cutaneous symptoms - odematous - indurative - atrophic
prognosis
renal and lung changes responsible for death pulmonary hypertension leads to 12% of systemic sclerosis-related deaths
How does it present?
Raynaud’s phenomenon skin becomes tight and thickened skin of the face affected - pinching of the skin of the nose (beaking) - tightening of the skin around the mouthtelangiectasia calcinosis - subcutaneous deposits of calcium may be seen in the digits major features - centrally located skin sclerosis (arm, face, neck)minor features - sclerodactyly and atrophy of the fingertips and bilateral lung fibrosis
what is the presentation of organ involvement in systemic sclerosis?
lung - pulmonary hypertension, pulmonary fibrosis accelerated hypertension - leading to renal crisis gut involvement - dysphagia, malabsorption, bacterial overgrowth of the small bowel inflammatory arthritis and myositis
how is it classified?
Limited (aka CREST)- skin involved confined to face, hands, forearms and feet - organ involvement occur later - anti-centromereDiffuse - skin changes develop more rapidly, can involve the trunk - early significant organ involvement - anti-Scl-70
how is it investigated?
immunology regular organ screening- pulmonary function test - echo - monitor renal function
how is it diagnosed?
patient needs 1 major feature and 2 minor features major features - centrally located skin sclerosis - arms, face, neckminor features - sclerodactyly and atrophy of the fingertips - bilateral lung fibrosis
how is it managed?
Tailored to specific issues:
Raynauds/digital ulcers - CCBs (calcium channel blockers) Iloprost, Bosentan
Renal involvement - ACEi
GI involvement - PPI for reflux
ILD - immunosuppression - usually cyclophoysphamide
