Systemic Sclerosis Flashcards
Systemic sclerosis
Systemic scleroderma
Multi organ disease with diffuse sclerosis of connective tissue
Pathogenesis
Small vessel vasculopathy
Fibrosis
Immune activation
Genetic predisposition
Auto reactive T cells
Maybe transferred from fetus to mother
Classification of Systemic sclerosis
Acral systemic sclerosis Type 1 (hands and forearms)
Acral systemic sclerosis Type 2 (hands, arms and trunk)
Systemic sclerosis Type 3 (trunk, face)
CREST syndrome
Includes type 1 and 2 systemic sclerosis
Calcinosis
Raynaud’s phenomenon
Esophageal disease
Sclerodactyly
Telengiectasis
Clinical features of systemic sclerosis on skin
Hands
Sclerodactyly
Rat bite ulcer on fingertips
Loss of cuticle with telangiectasis
Calcinosis
Clinical features of skin on face
Microstomia with frenulum tightening
Reduced facial expression
Telangiectasis
Other clinical features of skin
Diffuse sclerosis of skin (restrict respiration motion)
Raynaud phenomenon
Confetti like hypopigmentation
Systemic findings on GIT
Sclerosis of lingual frenulum
Impaired esophageal motility
Systemic findings on lungs
Pulmonary fibrosis
Also skin sclerosis
Kidney systemic findings
Hypertension and
Nephrosclerosis
Systemic findings on heart
Myocardial fibrosis
Cor pulmonale
Pericardial effusion
Liver systemic findings
Biliary cirrhosis
Anti mitochondrial antibodies
Musculoskeletal findings
Arthralgia
Muscle pain
Muscle atrophy
Bones systemic findings
Acro Osteolysis
Teeth systemic findings
Widening of periodontal membrane
