Raynaud's phenomenon, scleroderma Flashcards
what is Raynauds’s phenomenon ?
exaggerated vasoconstriction reflex to a normal stimuli
associated by episodic attacks of well demarcated color changes with numbness and pain of the digits on exposure to cold
what is the pathogenesis of Raynaud’s phenomenon ?
imbalance of vasoconstrictor and vasodilator signals
what are the two types of raynnaud’s?
primary or secondary
secondary
what are the investigations required for differentiating between primary and secondary Raynaud’s?
History and physical examination
thermography
capillaroscopy
autoimmune profile -ANA
what are the three phases of Raynaud ?
pallor
deoxygenation, stagnant blood - so cyanosis
reactive hyperemia ( red)
why does Raynaud’s happen in the fingers ?
more sensitive to cold exposure
which type of raynaud’s is less severe ?
primary
what lab results would confirm that the patient has 2ry Raynaud’s and not 1ry ?
positive ANA in 2ry
occurs in older patients
signs and symptoms off underlying autoimmune disease
trophic changes seen on fingertips
abnormal nail fold capillaroscopy in 2ry type
what are the features of primary raynaud’s?
fingers are affected with thumb sparing
symmetric manifestations
no trophic complications
nail fold capillaroscopy and serology are negative
what are the features of secondary raynaud’s?
signs and symptoms of underlying autoimmune disease
more prone to trophic complication
nail fold capillaroscopy and serology can be abnormal
what is the most common disease associated with secondary raynaud’s?
systemic sclerosis/scleroderma
SLE
what drugs could induce 2ry Raynauds?
ergo-alkaloids
B-blockers
bromocriptine
chemotherapeutics
what are the haematological conditions associated with 2ry raynauds syndrome ?
hyper-viscosity syndromes
polycythemia
leukemia
lymphoma
what are the endocrine associations with 2ry raynauds ?
hypothyroidism
pheochromocytoma
acromegaly
what are the differential diagnosis of Raynauds ?
vasculitis
antiphospholipid syndrome
Buerger’s disease
Macrovascular disease
what is the therapeutic approach for single short acute attack of raynauds ?
warmth, heating appliances, smoking cessation, vitamin c and E
what is the therapeutic approach for frequent long attacks that interfere with living in Raynauds ?
calcium channel blockers
angiotensin II receptor agonist
serotonin re-uptake antagonist
what do we give in severe attacks of raynauds or in the prresence of ulcers ?
IV iloprost , perform sympathectomy
what are the two types of systemic sclerosis ?
limited cutaneous systemic sclerosis (CREST syndrome)
diffuse cutaneous systemic sclerosis
diffuse cutaneous systemic sclerosis
what differentiates limited SS from diffuse ?
1- limited cutaneous systemic sclerosis :
the distribution of skin fibrosis
hands and forearms
leg and foot
2- diffuse cutaneous systemic sclerosis :
wide spread skin involvement and early visceral involvement
what is the associated pathology with systemic sclerosis ?
collagen fiber accumulation in the subcutaneous tissue which eventually leads to thinning of the skin
what is the clinical presentation of scleroderma ?
raynaud's phenomenon microstomata sclerodactyly calcinosis teleganctasia systemic manifestations in the diffuse type
what are the scleroderma related antibodies ?
for diffuse cutaneous scleroderma :
anti-Scl 70
for limited cutaneous scleroderma :
anti-centromere
which antibody is associated with pulmonary fibrosis in scleroderma ?
Scl-70
what is the treatment for pulmonary fibrosis in scleroderma ?
N-acetylcysteine
mycophenolate mofetil or Azathioprine
IV cyclophophamide + oral steroids
what can be used in refractory cases of pulmonary lung fibrosis in scleroderma ?
rituximab
which autoantibody is associated with renal crisis in scleroderma patients ?
Anti-RNA polymerase 3
what can we give in pulomary. hypertension in SSc?
phosphodiesterase 5 inhibitors
prostanoids
endothelin 1 receptor antagonists
what is the presentation of renal crisis and what is the autoantibody associated with it ?
Anti-RNA polymerase Ab
malignant HTN
worsening renal functions and raised creatinine
MAHA on blood smear
oliguria anuria
how do you treat renal crisis ?
control seizures
stop nephrotoxic drugs.
blood pressure control
review and initiate ACEE inhibitors
start iloprost
what are the muscle problems associated with scleroderma ?
myositis
proximal muscle myopathy
what is the treatment for myositis ?
steroids and methotrexate
what antibody is associated with myositis and pulmonary fibrosis ?
PmScl or Jo1
what is localized scleroderma ?
skin manifestation but without any internal organ affection
what are the three types of localized scleroderma ?
morphoea
linear scleroderma
en coup de sabre
what are the features of localized scleroderma morphoea ?
it can be either localized or generalized
localized affecting one or more skin lesion often on the truncal area
generalized - widespread skin nlesions
what are the features of linear scleroderma ?
affection is often in childhood and the skin affection is usually following a dermatomal distribution and may lead to secondary growth defects
what are the features of en coup de sabre ?
the most severe form of localized scleroderma where there is hemi-facial atrophy
what drug can cause scleroderma ?
bleomycin
what are the gastrointestinal manifestations associated with systemic sclerosis ?
GAVE - gastric antral vascular ectasia
oesophageal motility disorder
bowel hypomobility
malabsorption
