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Flashcards in Dr. P Deck (97)
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1

Antiphosphoipis antibodies are a family of autoantibodies against _____.

Phospholipid binding plasma proteins, B2-gp-I

2

Clinical manifestations of anti-phophoslipid

asymptomatic to catastrophic, Arterial=stroke, venous=DVT, loss of preg post 10 wks,

3

What are catastrophic APS?

rare, mutltiple thrombosis of medium and small arteries over days

4

How do you make diagnosis of APS?

presence of characteristic clinical manifestations and persistently positive aPLs 12 wks apard

5

A common strategy to prevent fetal loss in aPL positive patients is ____

low dose aspirin and heparin

6

Primary thrombosis prevention in persistenly aPL positive pt?

risk-stratified: eliminate reversible thrombosis risk factor and prophylaxis during high risk periods

7

Clinical features suggesting APS

livedo reticularis, thrombocytopenia, myelopathy

8

Diffuse connective tissue diseases are usually associated with autoimmunity to what?

sliceosomal components (U-RNPs)
nucleosomal (nucelosomes, histones, DNA)
proteosomal (HC9, LMP2)

9

_____ of molecules often renders them more antigenic in overlap syndromes.

apoptotic modificaiton

10

The evolution of symptoms of overlap syndromes may be associated with ______.

mimcry and epitope spreading

11

What are undifferentiated connective tissue disease?

early stages of many autoimmune CT disease. 50% remain undifferentiated.

12

Important clues about the eventual direction of differentiation of UDCT can be obtained from ______

nail-fold capillary microscopy, auto-antibody profile, clinical manifestations

13

What is MCTD?

prototype- lupus, scleroderma, myositis--- takes years to develop

14

The most common presentation of MCTD is ____.

Raynauds

15

MCTD is most commonly associated with antibodies to_____. This means what?

u1-rnp...... predicts lack of severe renal and CNS involvement

16

What is the major cause of death of MCTD?

pulmonary hypertension

17

Nearly all pt with UCTD have ______.

raynaud's and unexplained synovitis

18

What auto-antibodies predict what from UCTD?

U1-rnp--> MCTD
DNA--> SLE
nucleora--> SS
Synthetase and Pm/Scl--> myositis overlap

19

Scleroderma overlap syndromes include scleroderma overlap syndromes:

calcinosis, raynauds, esophageal involvement, sclerodactyly, telangietasia, CREST, mysositis associated with sclerodactyly, and MCTD

20

_____ is often the first clinical feature of SSc overlap syndromes.

Raynauds

21

The finding of _______ on the nail capillary fold bed and pathologic autoantibodies are important clues about the development of overlap syndrome.

thickened and dilated capillaries

22

CREST has a common overlap with _____.

primary biliary cirrhosis

23

______ are the main causes of morbitiy of scleroderma overlap syndromes.

pulm fibrosis and HTN

24

Which is more common, mysositis overlap or classic PM or DM?

MOS

25

_______ are associated wth myositis, arthritis, ILD

amino-acyl tRNA synthetase ab

26

Order of appearance of bad things in ARS

arthritis and ILD first, then myositis

27

What AB in MOS tend to mean corticosteroid unresponsiveness?

ARS, nucleoporin

28

What ab in MOS mean corticosteroid responsivenss?

U1-RNP, PM/Scl, Ku

29

What ab in MOS mean malignancy?

155-kd and 140-kd proteins

30

What serious effects are associated with MCTD?

renal- 1/4 get membranous GN
CNS: rare, trigem neuropathy and senso-hearing loss