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 ÂÂBP), 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 ÂESR
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
·
Features of SLE, PSS and
polymyositis
·
Anti-RNP (ribonuclear protein)
+ive without other types of ANA
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