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Organ Systems- Rheum > Dr. P > Flashcards

Dr. P Flashcards

1
Q

Antiphosphoipis antibodies are a family of autoantibodies against _____.

A

Phospholipid binding plasma proteins, B2-gp-I

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2
Q

Clinical manifestations of anti-phophoslipid

A

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

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3
Q

What are catastrophic APS?

A

rare, mutltiple thrombosis of medium and small arteries over days

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4
Q

How do you make diagnosis of APS?

A

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

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5
Q

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

A

low dose aspirin and heparin

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6
Q

Primary thrombosis prevention in persistenly aPL positive pt?

A

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

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7
Q

Clinical features suggesting APS

A

livedo reticularis, thrombocytopenia, myelopathy

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8
Q

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

A

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

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9
Q

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

A

apoptotic modificaiton

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10
Q

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

A

mimcry and epitope spreading

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11
Q

What are undifferentiated connective tissue disease?

A

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

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12
Q

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

A

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

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13
Q

What is MCTD?

A

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

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14
Q

The most common presentation of MCTD is ____.

A

Raynauds

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15
Q

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

A

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

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16
Q

What is the major cause of death of MCTD?

A

pulmonary hypertension

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17
Q

Nearly all pt with UCTD have ______.

A

raynaud’s and unexplained synovitis

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18
Q

What auto-antibodies predict what from UCTD?

A

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

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19
Q

Scleroderma overlap syndromes include scleroderma overlap syndromes:

A

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

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20
Q

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

A

Raynauds

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21
Q

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

A

thickened and dilated capillaries

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22
Q

CREST has a common overlap with _____.

A

primary biliary cirrhosis

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23
Q

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

A

pulm fibrosis and HTN

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24
Q

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

A

MOS

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25
Q

_______ are associated wth myositis, arthritis, ILD

A

amino-acyl tRNA synthetase ab

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26
Q

Order of appearance of bad things in ARS

A

arthritis and ILD first, then myositis

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27
Q

What AB in MOS tend to mean corticosteroid unresponsiveness?

A

ARS, nucleoporin

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28
Q

What ab in MOS mean corticosteroid responsivenss?

A

U1-RNP, PM/Scl, Ku

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29
Q

What ab in MOS mean malignancy?

A

155-kd and 140-kd proteins

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30
Q

What serious effects are associated with MCTD?

A

renal- 1/4 get membranous GN

CNS: rare, trigem neuropathy and senso-hearing loss

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31
Q

Which type of Raynauds is associated with other disease?

A

Type 2

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32
Q

Sjorgrens syndrome is divided into what forms? How common?

A

primary and secondary- Primary .1-.6%

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33
Q

What are the clinical hallmarks of Sjorgrens?

A

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

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34
Q

What are extraglandular features of Sjorgrens?

A

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

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35
Q

_____ is the characteristic histo finding for Sjorgrens?

A

mononuclear cell infiltrate of lacrimal and salivary glands

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36
Q

How do you diagnose Sjorgrens?

A

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

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37
Q

What is the point of treated Sjorgens?

A

symptom management and control of extraglandular disease

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38
Q

Epidemiolgoy of Sj. Syn.

A

middle aged woman with increase autoimmunity in the fammily

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39
Q

What is the relationship between SjSyn and lymphoma?

A

44 times increase risk

40
Q

Immunological features of Sj.Syn.

A

Hypergammaglobulinemia, FANA, RF, Anti-Ro and La

41
Q

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

A

Myopathies

42
Q

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

A

serum muscle enzymes and abnormal electromyograms

43
Q

What does histology show with myopathies?

A

varying degrees of inflammation and muscle fiber degernation and generation

44
Q

_____ are common therapies in myopathies.

A

Corticosteriods and cytotoxic drugs

45
Q

What antibodies could be present in myopathies?

A

molecules involved in protein synthesis

46
Q

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

A

B8, DR3, dr52, DQ2

47
Q

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

A

DM: perivascular, Cd4, macs, dendritic cells

others are CD8 prominant

48
Q

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

A

SLE

49
Q

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

A

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

50
Q

Describe what genetic analysis shows for SLE.

A

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
Q

Role of enviornmental factors with SLE

A

initation and flare ups

52
Q

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

A

TLR

53
Q

Role of nucleic-acid immune complexes in SLE

A

serve as stimuli for TLR

54
Q

Role of IFN in SLE

A

production of IFN as emerged as central mechanism of lupus

55
Q

Additional pathogenic effectors of SLE

A

platelets, neutrophil, complement

56
Q

Clinical manifestations and how to make a formal diagnosis of SLE

A

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

57
Q

What are the general, constitutional symptoms of SLE

A

fever, fatigue, weight loss

58
Q

____ is the most common subtype of CCLE.

A

discoid lupus

59
Q

Malar region butterfly rash is ______.

A

ACLE

60
Q

Is lupus arthritis symmetrical or not?

A

yes symmetrical

61
Q

Describe lupus arthritis

A

symmetrical, deforming, non-erosive

62
Q

What is Jaccoud’s arthropathy?

A

SLE, lookes like RA, no erosions on radiographs

63
Q

Describe SLE renal pthogenesis

A

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

64
Q

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

A

hemolytic anemia, immune thrombocytopenia, vasculitis

65
Q

Are SLE pregnancies considered high risk?

A

yes(fetal loss, small fetuss, neonatal lupus)

66
Q

What is pediatric onset-SLE associated with?

A

severeity

67
Q

Strategies to decrease impact of SLE comorbid infections.

A
  1. educate phys/pat 2. immunize 3. minimize GC exposure 4. prompt therapy
68
Q

What is the target GC dose for SLE?

A

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

69
Q

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

A

anti-malarials

70
Q

_____ is effective in mild to moderate SLE including nephritis.

A

AZA

71
Q

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

A

CYC and GC

72
Q

What is mmf used for?

A

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

73
Q

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

A

t

74
Q

How do you control SLE skin manifestations?

A

prevent sun exposure, anti-malarials, topical GC

75
Q

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

A

mmf

76
Q

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

A

AZA or mmf

77
Q

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

A

anti-platelet, anti-coag

78
Q

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

A

GC +/- IS

79
Q

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

A

Scleroderma

80
Q

Is there marked pt-pt variability is Scleroderma?

A

yes

81
Q

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

A

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

82
Q

What does genetic analysis say about sleroderma?

A

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

83
Q

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

A

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

84
Q

What is fibrosis associated with in scleroderma?

A

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

85
Q

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

A

T

86
Q

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

A

Systemic sclerosis

87
Q

What is the difference between early limited and diffuse scleroderma

A

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
Q

Describe GI problems in systemic sclerosis

A

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
Q

Is lung disease a major cause of morbitidy in scleroderma?

A

yes

90
Q

Does pulmonary fibrosis occur in diffuse and limited sclerosis?

A

yes- variable in prognosis and severity

91
Q

______ has the worse prognosis with ILD in scleroderm.

A

nonwhites an anti-topo1 positive pt

92
Q

Tx for scleroderma drelated ILD

A

immunosuppresion

93
Q

What are risk factors for scleroderma lung

A

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

94
Q

The degree of lung fibrosis on ______ predicts outcom

A

HRCT

95
Q

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

A

in 5-10% OF PT

96
Q

What are risk factors for scleroderma renal crisis

A

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

97
Q

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

A

ACEis

Organ Systems- Rheum (10 decks)

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