Anti Phospholipid Syndrome, Sjogren syndrome, Scleroderma Flashcards

1
Q

What is Anti phospholipid syndrome characterised by?

A

Thrombus (increased risk of blood clots)
Recurrent miscarriage

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2
Q

What Ab are found?

A

aPL Abs

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3
Q

It can be 1^ or 2^?

A

1^ = idiopathic
2^ to other diseases, especially SLE

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4
Q

Which gender is APS found in?

A

Females

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5
Q

Sx of APS?
Acronym

A

CLOTs
Coagulopathy
Livedo reticularis (purple discolouration of skin, lace like)
Obstetric Issues - MISCARRIAGES
Thrombocytopenia

+ increased risk arterial (Stroke, MI) + Venous thrombus (DVT)

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6
Q

Diagnosis of APS?

A

Sx + Lupus anticoagulant
Anti Cardiolipin Ab (Ig G/M) +ve
Anti B2 Glycoprotein-1 Ab

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7
Q

Treatment for APS?

A

1st line = Warfarin (long term if have had a thrombus)
Pregnant = Heparin + aspirin

Prophylaxic (not had thrombus) = Aspirin

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8
Q

What is sjogren syndrome?

A

DRY DRY DRY
Autoimmune exocrine dysfunction

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9
Q

Can be 1^ or 2^?

A

2^ to other autoimmune diseases (SLE)

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10
Q

Who is this syndrome mostly seen in? RF?

A

Females 40-50
fHx
HLA B8/DR3

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11
Q

Symptoms of Sjogrens?

A

Dry eyes (keratoconjunctivitis)
Mouth (xeostomia)
Vagina

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12
Q

Diagnosis of Sjogrens?

A

Serology - Anti RO + Anti LA Abs
ANA often +ve
Schirmer test = Induce tears + place filter paper under eyes, tears travel <10mm (should be 20+mm)

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13
Q

Treatment for Sjorgens?

A

Artificial tears, saliva, Lubricant for sexual activity

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14
Q

What is sometimes given (up to Dr)?

A

Hydroxychloroquine

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15
Q

What is scleroderma?

A

Systemic condition of which mc type is limited to cutaneous scleroderma (aka CREST)

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16
Q

What does crest stand for?

A

Calcinosis (Ca deposits in SC tissue)=renal failure

Raynauds - digit ischemia due to sudden vasospasm, often precipitated by cold, relieved by heat

Esophageal dysmotility/strictures = GI Sx

Sclerodactyly (local skin thickening or tightening on fingers/toes = movement restriction)

Telanglectasia (spider veins = risk of pul htn)

17
Q

Diagnosis of Scleroderma?

A

Anti Centromere Abs
(ACAs) - 70% cases
ANA often +ve

18
Q

Treatment for scleroderma?

A

No cure, treat Sx (eg. Raynauds = hand warmers, GI Sx = PPI)