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Flashcards in Systemic sclerosis Deck (13):

Systemic sclerosis

-Scleroderma: thickened hardened skin (may be diffuse or limited)
-Localized (morphoea and linear scleroderma) and systemic scleoderma (limited systemic sclerosis, diffuse systemic sclerosis, systemic sclerosis w/o scleroderma)
-Diffuse scleroderma is the same as systemic sclerosis, except there is skin involvement as well


Limited systemic scleroderma 1

-Limited to hands, distal forearms, face, chest (visceral involvement delayed) usually symmetrical
-Assciated w/ pulmonary HTN
-CREST are main defining Sx
-Calcinosis: usually not painless/dangerous
-Raynauds: change in color of skin due to vasospasm (white-> blue-> red)


Limited systemic scleroderma 2

-Esophageal dysfunction: dysfunction of the lower esophageal sphincter, causes esophageal reflux
-Sclerodactyly: deformities due to tightening/fibrosis of soft tissues of the feet/hands (contractures, ulcerations, ischemia)
-Telangiectasia: small dilated blood vessels
-More slowly progressing than diffuse, limited is associated w/ anti-centromere


Diffuse systemic sclerosis

-Diffuse skin thickening: face, neck, chest, hands, arms, legs
-Significant early extra-cutaneous manifestations
-Major face involvement: mask like facies, thinned and/or retracted lips, decreased oral aperture, tight skin w/ waxy texture
-Stages of skin involvement: edema, induration, atrophy
-Dermis gets very thick w/ collagen, salt and pepper appearance (advanced changes of sclerodactyly)
-Rapidly progressing, associated w/ anti-topoisomerace (SCL70)


Systemic sclerosis sine scleroderma

-Systemic sclerosis w/o significant skin involvement
-Extra-cutaneous features: musculoskeletal, GI, pulmonary, CV, renal
-MSK: symmetric polyarticular arthritis, tendon rubs (squeaky tendons, muscle atrophy and/or myositis, associated w/ more severe disease)


Systemic sclerosis sine scleroderma: GI involvement

-Entire extent of GI tract affected
-Esophagus: dilation, impaired mobility, lower esophageal sphincter dysfunction, esophageal reflux w/ mucosal ulcerations
-Stomach: delayed gastric emptying, watermelon stomach (gastric antral vascular ectasia)
-Small intestine: atony w/ obstruction, stasis w/ malabsorption
-Large intestine: pseudo-diverticula, infarction


Systemic sclerosis sine scleroderma: pathophysiology of GI Sx

-Atrophy of smooth muscle and replacement w/ collagen (fibrosis)
-Atrophy and fibrosis of submucosa and mucosa
-Degenerative and inflammatory changes in blood vessels


Systemic sclerosis sine scleroderma: pulmonary involvement

-Pulmonary fibrosis
-Hyperplasia and sclerosis of pulmonary artery-> pulmonary HTN-> heart failure
-Superimposed infection
-Microaspirations (exacerbated by GERD)
-Lung CA


Systemic sclerosis sine scleroderma: CV involvement

-Myocardial fibrosis
-Conduction deficits
-Pulm HTN-> heart failure


Systemic sclerosis sine scleroderma: renal involvement

-Malignant HTN plus renal insufficiency can lead to scleroderma renal crisis
-Precipitated by steroid use, results in intimal hyperplasia and fibrinoid necrosis of medium size renal blood vessels (vascular issue due to fibrosis)
-Rx is ACE inhibitor, since angiotensin/renin is over activated you must block the converting nz for angiotensin (ACE)


Localized scleroderma: morphoea

-Spectrum of diseases mainly affecting the skin
-Plaque-like lesion, most common
-Lesions usually oval and small, on 1 area of body
-Early phase of morphoea: erythematous
-Late phase of morphoea: sclerotic, white


Localized scleroderma: linear scleroderma

-Most common type in children
-Longitudinal, line-band lesions usually on limbs
-Can lead to joint contractures and muscle atrophy


Pathogenesis of systemic sclerosis

-Not understood completely
-Key elements: immune activation, vascular damage, fibrosis
-There is an increased number of activated fibroblasts in affected areas
-There are usually ANAs against topoisomerase (leading to diffuse) or centromeres (leading to limited)
-But these two are mutually exclusive
-Imbalance of cytokines due to/causing inflammation