Systemic Sclerosis Flashcards
what are characteristics
vasomotor disturbances (raynauds), fibrosis and subsequent atrophy of the skin and subcutaneous tissue
what causes the skin and internal organ changes
excessive collagen deposition
what causes death in these patients
renal and lung changes
what percentage of deaths does pulmonary hypertension lead to
12%
what is the presentation (cutaneous)
Raynauds (early, uncommon to not be present)
what three phases does cutaneous involvement have
- oedematous
- indurative
- atrophic (skin becomes thickened and tight)
what two groups are the features divided into
major and minor
what is included in major
centrally located skin sclerosis affecting arms, face and neck
what is included in minor
sclerodacyly and atrophy of fingertips and bilateral lung fibrosis
how is a diagnosis made
patient has 1 major and 2 minor features
when the skin of the face is affected what is seen
pinching of nose (beaking)
tightening of skin around the mouth
telangiectasia
what is calcinosis
subcutaneous deposits of calcium seen in digits
what organ involvement can be seen
pulmonary hypertension, pulmonary fibrosis, accelerated hypertension leading to renal crisis,
gut involvement can lead to dysphagia, malabsorption and bacterial overgrowth of small bowel
inflammatory arthritis and myositis
what is the classification
limited and diffuse form
what is involved in limited
skin involved is confined to face, hands, forearm and feet
organ involvement occurs later
what antibody is associated with limited
anti-centromere
what is involved in diffuse
skin changes develop more rapidly and involve whole trunk
early significant organ involvement
what antibody is associated with diffuse
anti-Scl-70
what are investigations
anti-centromere and anti-Scl-70 auto-antibodies, organ screening including pulmonary function tests, echo and renal function
what is the management dependent on
specific issues
how is Raynauds/digital ulcers managed
calcium channel blockers, iloprost, bosentan
how is renal involvement managed
ACE inhibitors
how is GI involvement managed
PPI for reflux
how is interstitial lung disease managed
immunosuppression (usually cyclophosphamide)
