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SCLERODERMA/SYSTEMIC SCLEROSIS (CREST (limited ssc) (Raynaud's…
SCLERODERMA/SYSTEMIC SCLEROSIS
overproduction of connective tissue
diffuse
limited cutaneous
less severe
affects skin:
face :neutral_face:
forearms
legs
limited to hands and face
Raynaud's phenomenon
this can be the first symptoms
MX:
nifedipine
can also
consider
IV prostacyclin infusion
when to suspect
underlying connective tissue disorde
r? :checkered_flag:
40yo at onset
unilateral
rashes
RA
recurrent miscarriages (SLE)
calcinosis
CREST
(limited ssc)
calcinosis
Raynaud's phenomenon
oEsophageal dysmotility
sclerodactyly
telangiectasia
Ix: ECG - RV strain
restrictive pattern fibrosis
Mx: steroids
immunosuppression (cyclophosphamide) for organ involvement
Dx: :checkered_flag:
must have RP and ANA :heavy_plus_sign: for systemic sclerosis
for scleroderma does not have to have RP :heavy_plus_sign:
Features:
SKIN
LOCALISED -
SCLERODERMA
morphoea
itchy
trunk and legs - most affected
guttate morphea
variant in neck, shoulders and anterior chest
SYSTEMIC SLCERODERMA
early stage
thinning epidermis
non pitting oedema
taut skin
skin creases disappear
late stage
contractures
fibrosis
ulcers
:silhouettes:
systemic features
:silhouettes:
MSK - arthritis
GI - oEsophagus hypomotility, reflux
small bowel - malabsorption :arrow_right: TPN :question:
anaemia - due to vascular lesions in GI mucosa
:red_flag:lungs -
major cause of death in systemic sclerosis
:arrow_right: fibrosis/ pulmonary HTN
:<3:
fibrosis, MI, endocarditis
RENAL
renal hypertensive crisis :arrow_right: treat with ACEI and CCB
:red_flag: avoid corticosteroids as they can precipitate the crisis
LIVER
PBC
fibrosis
: DLCO abnormal
pulmonary
HTN
:
DLCO low + normal lung volumes
W>M, 40-50yo
if not skin affected :arrow_right: termed :
systemic sclerosis sine scleroderma
anti-scl70
:arrow_right:
SYSTEMIC SCLEROSIS
anticentromere antibody
limited disease systemic sclerosis
anti-topoisomerase & anti-RNA polymerase antibodies
diffuse SSC
