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ICC2 > Raynaud's phenomenon, scleroderma > Flashcards

Raynaud's phenomenon, scleroderma Flashcards

1
Q

what is Raynauds’s phenomenon ?

A

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

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2
Q

what is the pathogenesis of Raynaud’s phenomenon ?

A

imbalance of vasoconstrictor and vasodilator signals

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3
Q

what are the two types of raynnaud’s?

A

primary or secondary

secondary

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4
Q

what are the investigations required for differentiating between primary and secondary Raynaud’s?

A

History and physical examination
thermography
capillaroscopy
autoimmune profile -ANA

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5
Q

what are the three phases of Raynaud ?

A

pallor
deoxygenation, stagnant blood - so cyanosis
reactive hyperemia ( red)

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6
Q

why does Raynaud’s happen in the fingers ?

A

more sensitive to cold exposure

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7
Q

which type of raynaud’s is less severe ?

A

primary

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8
Q

what lab results would confirm that the patient has 2ry Raynaud’s and not 1ry ?

A

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

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9
Q

what are the features of primary raynaud’s?

A

fingers are affected with thumb sparing
symmetric manifestations
no trophic complications
nail fold capillaroscopy and serology are negative

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10
Q

what are the features of secondary raynaud’s?

A

signs and symptoms of underlying autoimmune disease
more prone to trophic complication
nail fold capillaroscopy and serology can be abnormal

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11
Q

what is the most common disease associated with secondary raynaud’s?

A

systemic sclerosis/scleroderma

SLE

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12
Q

what drugs could induce 2ry Raynauds?

A

ergo-alkaloids
B-blockers
bromocriptine
chemotherapeutics

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13
Q

what are the haematological conditions associated with 2ry raynauds syndrome ?

A

hyper-viscosity syndromes
polycythemia
leukemia
lymphoma

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14
Q

what are the endocrine associations with 2ry raynauds ?

A

hypothyroidism
pheochromocytoma
acromegaly

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15
Q

what are the differential diagnosis of Raynauds ?

A

vasculitis
antiphospholipid syndrome
Buerger’s disease
Macrovascular disease

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16
Q

what is the therapeutic approach for single short acute attack of raynauds ?

A

warmth, heating appliances, smoking cessation, vitamin c and E

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17
Q

what is the therapeutic approach for frequent long attacks that interfere with living in Raynauds ?

A

calcium channel blockers
angiotensin II receptor agonist
serotonin re-uptake antagonist

18
Q

what do we give in severe attacks of raynauds or in the prresence of ulcers ?

A

IV iloprost , perform sympathectomy

19
Q

what are the two types of systemic sclerosis ?

A

limited cutaneous systemic sclerosis (CREST syndrome)
diffuse cutaneous systemic sclerosis

diffuse cutaneous systemic sclerosis

20
Q

what differentiates limited SS from diffuse ?

A

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

21
Q

what is the associated pathology with systemic sclerosis ?

A

collagen fiber accumulation in the subcutaneous tissue which eventually leads to thinning of the skin

22
Q

what is the clinical presentation of scleroderma ?

A 
raynaud's phenomenon 
microstomata 
sclerodactyly 
calcinosis 
teleganctasia 
systemic manifestations in the diffuse type
23
Q

what are the scleroderma related antibodies ?

A

for diffuse cutaneous scleroderma :
anti-Scl 70
for limited cutaneous scleroderma :
anti-centromere

24
Q

which antibody is associated with pulmonary fibrosis in scleroderma ?

A

Scl-70

25
Q

what is the treatment for pulmonary fibrosis in scleroderma ?

A

N-acetylcysteine
mycophenolate mofetil or Azathioprine
IV cyclophophamide + oral steroids

26
Q

what can be used in refractory cases of pulmonary lung fibrosis in scleroderma ?

A

rituximab

27
Q

which autoantibody is associated with renal crisis in scleroderma patients ?

A

Anti-RNA polymerase 3

28
Q

what can we give in pulomary. hypertension in SSc?

A

phosphodiesterase 5 inhibitors
prostanoids
endothelin 1 receptor antagonists

29
Q

what is the presentation of renal crisis and what is the autoantibody associated with it ?

A

Anti-RNA polymerase Ab
malignant HTN
worsening renal functions and raised creatinine
MAHA on blood smear
oliguria anuria

30
Q

how do you treat renal crisis ?

A

control seizures
stop nephrotoxic drugs.
blood pressure control
review and initiate ACEE inhibitors
start iloprost

31
Q

what are the muscle problems associated with scleroderma ?

A

myositis

proximal muscle myopathy

32
Q

what is the treatment for myositis ?

A

steroids and methotrexate

33
Q

what antibody is associated with myositis and pulmonary fibrosis ?

A

PmScl or Jo1

34
Q

what is localized scleroderma ?

A

skin manifestation but without any internal organ affection

35
Q

what are the three types of localized scleroderma ?

A

morphoea
linear scleroderma
en coup de sabre

36
Q

what are the features of localized scleroderma morphoea ?

A

it can be either localized or generalized
localized affecting one or more skin lesion often on the truncal area
generalized - widespread skin nlesions

37
Q

what are the features of linear scleroderma ?

A

affection is often in childhood and the skin affection is usually following a dermatomal distribution and may lead to secondary growth defects

38
Q

what are the features of en coup de sabre ?

A

the most severe form of localized scleroderma where there is hemi-facial atrophy

39
Q

what drug can cause scleroderma ?

A

bleomycin

40
Q

what are the gastrointestinal manifestations associated with systemic sclerosis ?

A

GAVE - gastric antral vascular ectasia
oesophageal motility disorder
bowel hypomobility
malabsorption

ICC2 (42 decks)

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