Dr. P Flashcards Preview

Organ Systems- Rheum > Dr. P > Flashcards

Flashcards in Dr. P Deck (97):
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

31

Which type of Raynauds is associated with other disease?

Type 2

32

Sjorgrens syndrome is divided into what forms? How common?

primary and secondary- Primary .1-.6%

33

What are the clinical hallmarks of Sjorgrens?

keratoconjunctivitis sicca/dry eyes, xerstomia/dry mouth, parotid swelling

34

What are extraglandular features of Sjorgrens?

fatigue, raynauds, polyarthralgias, ILD, neuropathy, purpura, renal tubular defects

35

_____ is the characteristic histo finding for Sjorgrens?

mononuclear cell infiltrate of lacrimal and salivary glands

36

How do you diagnose Sjorgrens?

dry eyes/mouth assessment, ANAs- antiRo/SS-a and anti-La/SS-b, labial salivary gland biopsy

37

What is the point of treated Sjorgens?

symptom management and control of extraglandular disease

38

Epidemiolgoy of Sj. Syn.

middle aged woman with increase autoimmunity in the fammily

39

What is the relationship between SjSyn and lymphoma?

44 times increase risk

40

Immunological features of Sj.Syn.

Hypergammaglobulinemia, FANA, RF, Anti-Ro and La

41

_____ are a heterogenous group of muscle diseases characterized by symmetric proximal muscle weakness and frequent involvement of other organs.

Myopathies

42

Myopathies are often accompanied by elevated levels of ________ and _____.

serum muscle enzymes and abnormal electromyograms

43

What does histology show with myopathies?

varying degrees of inflammation and muscle fiber degernation and generation

44

_____ are common therapies in myopathies.

Corticosteriods and cytotoxic drugs

45

What antibodies could be present in myopathies?

molecules involved in protein synthesis

46

What haplotype is found in a significant portion of IBM patients?

B8, DR3, dr52, DQ2

47

How is the cell mediated immune response different between Dm and Pm and Ibm?

DM: perivascular, Cd4, macs, dendritic cells
others are CD8 prominant

48

_____ results from chronic and recurrent activation of the immune system with production of Ab and other protein products contributing to inflammation and tissue damage.

SLE

49

What is typical epidemiology of SLE? What does this mean?

black female in child-bearing years- there may be a role for both hormone and sex-related factors

50

Describe what genetic analysis shows for SLE.

Complement def. confer the highest risk. Mutuations in TREX1 point to imparied regulation of nucleic acids. Most variants that increase SLE are related to innate and adaptive immune response.

51

Role of enviornmental factors with SLE

initation and flare ups

52

The discovery of _____ has showed importance of innate immunity in SLE.

TLR

53

Role of nucleic-acid immune complexes in SLE

serve as stimuli for TLR

54

Role of IFN in SLE

production of IFN as emerged as central mechanism of lupus

55

Additional pathogenic effectors of SLE

platelets, neutrophil, complement

56

Clinical manifestations and how to make a formal diagnosis of SLE

RASH PAIN O MD (renal, arthritis, serositis, hematologic, photosens., ANA, Immunologic, neurologic, oral ulcers, malar rash, discoid rash) 4/11

57

What are the general, constitutional symptoms of SLE

fever, fatigue, weight loss

58

____ is the most common subtype of CCLE.

discoid lupus

59

Malar region butterfly rash is ______.

ACLE

60

Is lupus arthritis symmetrical or not?

yes symmetrical

61

Describe lupus arthritis

symmetrical, deforming, non-erosive

62

What is Jaccoud's arthropathy?

SLE, lookes like RA, no erosions on radiographs

63

Describe SLE renal pthogenesis

significant morbitiy, 50% of SLE pt, coreelated with anti-DNA, immune complex can be deposited or form in situe

64

Anti-body directed cellular cytotoxicity is responsible for what in SLE?

hemolytic anemia, immune thrombocytopenia, vasculitis

65

Are SLE pregnancies considered high risk?

yes(fetal loss, small fetuss, neonatal lupus)

66

What is pediatric onset-SLE associated with?

severeity

67

Strategies to decrease impact of SLE comorbid infections.

1. educate phys/pat 2. immunize 3. minimize GC exposure 4. prompt therapy

68

What is the target GC dose for SLE?

.25 mg/kg every other day for 2-3 months

69

_____ are effective for skin and mucocuatneous manifestations of SLE. Their ues has been associated with reduced organ damage.

anti-malarials

70

_____ is effective in mild to moderate SLE including nephritis.

AZA

71

What is the standard of care for severe SLE with major organ involvement?

CYC and GC

72

What is mmf used for?

equally efficient and better tox profile than CYC for moderartely severe PLN

73

T or F, Calcineurin inhibitors and rituximab are used in refractory lupus nephritis.

t

74

How do you control SLE skin manifestations?

prevent sun exposure, anti-malarials, topical GC

75

In moderately severe PLN, ______ may be preferred as induction regiment when gonadal tox is concern.

mmf

76

What may be used in maintenace therapy after induction with severe lupus

AZA or mmf

77

______ are necesary in pt with APS to prevent recurrent events.

anti-platelet, anti-coag

78

What is used for neurpsychiatric events of SLE though to be due to inflammatory process?

GC +/- IS

79

___ has a complex pathogenesis and protean clinical manifestations reflecting the underlying early immune dysregulation and microangiopathy as well as a systemic fibrosis.

Scleroderma

80

Is there marked pt-pt variability is Scleroderma?

yes

81

In scleroderma, vascular lesions in ___ blood vessels occur early and progress to _____.

small, obliterative vasculopathy that causes tissue hypoxia, oxidative stress, and vascular complications

82

What does genetic analysis say about sleroderma?

role of HLA and other immunoregulatory genes that are also associated with lupus

83

____, ____, and _____ is prominent in scleroderma but roles undetermined as primary factory.

immune dysregulation manifested by ab, evidence of innate immune activation, interferon signature

84

What is fibrosis associated with in scleroderma?

sustained mesenchymal cella ctivation by grwoth factors, cytokines, chemokines, hypoxia, ROS, abberaant reactivation of developmental pathways

85

T or F Systemic sclerosis is a mutlisystem connective tissue disease affecting the skin and internal organs.

T

86

____ disease process is characterized by chronic inflammatoin with variable degrees of collagen accumulation in affected tissues and obliterative vasculopathy of peripheral and visceral vasculature.

Systemic sclerosis

87

What is the difference between early limited and diffuse scleroderma

Limited: Raynauds only for years. general symptoms are rare, minmal arthralgia, puffy FINGERS, limited skin thickening, anti-centromere
Diffuse: raynauds delayed, acute onset of lots of symtpoms, lots of arthtralgias including carpal tunnel, swollen HANDS, early diffuse skin, anti-scl70, anti-RNA pol3

88

Describe GI problems in systemic sclerosis

manifestations of gut dysmotility are universally present in scleroderma and can affect anywhere in tract, though upper is more common and can be severe. Dysfunciton and failure of lwoer GI is associated with poor prognosis

89

Is lung disease a major cause of morbitidy in scleroderma?

yes

90

Does pulmonary fibrosis occur in diffuse and limited sclerosis?

yes- variable in prognosis and severity

91

______ has the worse prognosis with ILD in scleroderm.

nonwhites an anti-topo1 positive pt

92

Tx for scleroderma drelated ILD

immunosuppresion

93

What are risk factors for scleroderma lung

late onset of slceroderma, limimted phenotype, lots of telangtasia, positive anti-centromere ab

94

The degree of lung fibrosis on ______ predicts outcom

HRCT

95

Scleroderma renal crisis in a life threatening condition that occurs ____.

in 5-10% OF PT

96

What are risk factors for scleroderma renal crisis

early diffuse skin disease, use of corticosteroids, anti-RNApol3

97

What can be done to control and maybe even reverse scleroderma renal crisis?

ACEis