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Chapter: Medicine Study Notes : Musculo-Skeletal

Progressive Systemic Sclerosis (PSS) - Connective Tissue Diseases

Connective tissue disease with inflammation, vasculitis and fibrotic changes in skin and viscera

Progressive Systemic Sclerosis (PSS)

 

·        Connective tissue disease with inflammation, vasculitis and fibrotic changes in skin and viscera

·        Epidemiology: female = 3 * male.  Any age, but peak is 30 – 50 years

·        Pathology:

 

o   Small vessel damage + oedema ® collagen laid down ® fibrosis and contraction


o   Dilation of other vessels ® telangiectasia

·        Presentation:

o   Raynaud‟s (90%) may precede other signs by years


o   Then swelling of fingers and hands

 

o   Then skin gets tight, waxy and tethered (eg fingers – pointy fingers, forearms, face – no wrinkles, pointy nose)

 

o   Other: telangiectasia, nail bed spots, symmetrical polyarthritis


·        Types:

 

o   Diffuse/Generalised Progressive Systemic Sclerosis: widespread skin involvement with early visceral involvement ® kidney (proteinuria, sediment, maybe crisis ­­BP), polyarthritis, myopathy, lung fibrosis (¯expansion + ¯gas transfer ® SOB) and GI fibrosis

 

o   CREST Syndrome (probably very different disease entity to Diffuse): Calcinosis (subcutaneous calcium deposits on hands) + Raynaud‟s phenomenon + disordered oesophageal motility (heart burn and dysphagia) + sclerodactyly (Scleroderma of the hands) + telangiectasia. Centromeric

 

o   ANA


o   Limited Scleroderma: 

§  Tightening and fibrosis of the skin: proximal skin scleroderma (eg face – can they open their mouth wide, any wrinkles – if so then no involvement. Limited mouth opening = microstomia) or any 2 of sclerodactyly (can they make a fist, Prayer sign: can they oppose palmar MCP joints), digital pitting scars, pulp loss, bibasilar lung fibrosis. Late visceral involvement 

§  Scleroderma limited to the hands and maybe face („Limited Scleroderma‟) is probably a presenting symptom of CREST syndrome even if the other features aren‟t present

§  Morphoea (localised skin sclerosis) rarely, if ever, progresses to PSS 

 

·        Investigations:

o  FBC: normocytic anaemia, haemolytic anaemia 

o  ­ESR 

o  ANA positive in 75%. May have autonuclear autoantibodies in any of these three forms to: topoisomerase (Scl-70), RNA polymerases and centromeres. Anticentromere (ACA) in Limited and CREST. AntiScl-70 in diffuse. 

o  RF +ive in 30%

o  24 hour urine

o  Hand x-ray.  Can get distal phalanges resorption

o  Barium swallow and CT of lung

·        Treatment:

o  No cure

o  Education, support groups, etc

o  Raynaud‟s: warmth and vasodilators (Ca blocker)

o  Oesophageal mobility: omeprazole, cisapride, reflux prevention

o  Renal & Raynaud‟s: ACE inhibitors

o  Scleroderma: D-penicillamine (antifibrotic) or immunosuppressants (little efficacy from steroids) 

·        Prognosis: Limited disease has 70% 10-year survival; diffuse has 55% 10-year survival. Death from lung/renal effects

 

Mixed Connective Tissue Disease

 

·        Features of SLE, PSS and polymyositis

·        Anti-RNP (ribonuclear protein) +ive without other types of ANA

 

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Medicine Study Notes : Musculo-Skeletal : Progressive Systemic Sclerosis (PSS) - Connective Tissue Diseases |


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