Dr. P

Question 1

Antiphosphoipis antibodies are a family of autoantibodies against _____.

Answer 1

Phospholipid binding plasma proteins, B2-gp-I

Question 2

Clinical manifestations of anti-phophoslipid

Answer 2

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

Question 3

What are catastrophic APS?

Answer 3

rare, mutltiple thrombosis of medium and small arteries over days

Question 4

How do you make diagnosis of APS?

Answer 4

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

Question 5

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

Answer 5

low dose aspirin and heparin

Question 6

Primary thrombosis prevention in persistenly aPL positive pt?

Answer 6

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

Question 7

Clinical features suggesting APS

Answer 7

livedo reticularis, thrombocytopenia, myelopathy

Question 8

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

Answer 8

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

Question 9

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

Answer 9

apoptotic modificaiton

Question 10

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

Answer 10

mimcry and epitope spreading

Question 11

What are undifferentiated connective tissue disease?

Answer 11

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

Question 12

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

Answer 12

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

Question 13

What is MCTD?

Answer 13

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

Question 14

The most common presentation of MCTD is ____.

Answer 14

Raynauds

Question 15

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

Answer 15

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

Question 16

What is the major cause of death of MCTD?

Answer 16

pulmonary hypertension

Question 17

Nearly all pt with UCTD have ______.

Answer 17

raynaud’s and unexplained synovitis

Question 18

What auto-antibodies predict what from UCTD?

Answer 18

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

Question 19

Scleroderma overlap syndromes include scleroderma overlap syndromes:

Answer 19

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

Question 20

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

Answer 20

Raynauds

Question 21

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

Answer 21

thickened and dilated capillaries

Question 22

CREST has a common overlap with _____.

Answer 22

primary biliary cirrhosis

Question 23

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

Answer 23

pulm fibrosis and HTN

Question 24

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

Answer 24

MOS

Question 25

_______ are associated wth myositis, arthritis, ILD

Answer 25

amino-acyl tRNA synthetase ab

Question 26

Order of appearance of bad things in ARS

Answer 26

arthritis and ILD first, then myositis

Question 27

What AB in MOS tend to mean corticosteroid unresponsiveness?

Answer 27

ARS, nucleoporin

Question 28

What ab in MOS mean corticosteroid responsivenss?

Answer 28

U1-RNP, PM/Scl, Ku

Question 29

What ab in MOS mean malignancy?

Answer 29

155-kd and 140-kd proteins

Question 30

What serious effects are associated with MCTD?

Answer 30

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

Question 31

Which type of Raynauds is associated with other disease?

Answer 31

Type 2

Question 32

Sjorgrens syndrome is divided into what forms? How common?

Answer 32

primary and secondary- Primary .1-.6%

Question 33

What are the clinical hallmarks of Sjorgrens?

Answer 33

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

Question 34

What are extraglandular features of Sjorgrens?

Answer 34

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

Question 35

_____ is the characteristic histo finding for Sjorgrens?

Answer 35

mononuclear cell infiltrate of lacrimal and salivary glands

Question 36

How do you diagnose Sjorgrens?

Answer 36

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

Question 37

What is the point of treated Sjorgens?

Answer 37

symptom management and control of extraglandular disease

Question 38

Epidemiolgoy of Sj. Syn.

Answer 38

middle aged woman with increase autoimmunity in the fammily

Question 39

What is the relationship between SjSyn and lymphoma?

Answer 39

44 times increase risk

Question 40

Immunological features of Sj.Syn.

Answer 40

Hypergammaglobulinemia, FANA, RF, Anti-Ro and La

Question 41

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

Answer 41

Myopathies

Question 42

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

Answer 42

serum muscle enzymes and abnormal electromyograms

Question 43

What does histology show with myopathies?

Answer 43

varying degrees of inflammation and muscle fiber degernation and generation

Question 44

_____ are common therapies in myopathies.

Answer 44

Corticosteriods and cytotoxic drugs

Question 45

What antibodies could be present in myopathies?

Answer 45

molecules involved in protein synthesis

Question 46

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

Answer 46

B8, DR3, dr52, DQ2

Question 47

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

Answer 47

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

Question 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.

Answer 48

SLE

Question 49

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

Answer 49

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

Question 50

Describe what genetic analysis shows for SLE.

Answer 50

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.

Question 51

Role of enviornmental factors with SLE

Answer 51

initation and flare ups

Question 52

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

Answer 52

TLR

Question 53

Role of nucleic-acid immune complexes in SLE

Answer 53

serve as stimuli for TLR

Question 54

Role of IFN in SLE

Answer 54

production of IFN as emerged as central mechanism of lupus

Question 55

Additional pathogenic effectors of SLE

Answer 55

platelets, neutrophil, complement

Question 56

Clinical manifestations and how to make a formal diagnosis of SLE

Answer 56

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

Question 57

What are the general, constitutional symptoms of SLE

Answer 57

fever, fatigue, weight loss

Question 58

____ is the most common subtype of CCLE.

Answer 58

discoid lupus

Question 59

Malar region butterfly rash is ______.

Answer 59

ACLE

Question 60

Is lupus arthritis symmetrical or not?

Answer 60

yes symmetrical

Question 61

Describe lupus arthritis

Answer 61

symmetrical, deforming, non-erosive

Question 62

What is Jaccoud's arthropathy?

Answer 62

SLE, lookes like RA, no erosions on radiographs

Question 63

Describe SLE renal pthogenesis

Answer 63

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

Question 64

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

Answer 64

hemolytic anemia, immune thrombocytopenia, vasculitis

Question 65

Are SLE pregnancies considered high risk?

Answer 65

yes(fetal loss, small fetuss, neonatal lupus)

Question 66

What is pediatric onset-SLE associated with?

Answer 66

severeity

Question 67

Strategies to decrease impact of SLE comorbid infections.

Answer 67

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

Question 68

What is the target GC dose for SLE?

Answer 68

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

Question 69

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

Answer 69

anti-malarials

Question 70

_____ is effective in mild to moderate SLE including nephritis.

Answer 70

AZA

Question 71

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

Answer 71

CYC and GC

Question 72

What is mmf used for?

Answer 72

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

Question 73

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

Answer 73

t

Question 74

How do you control SLE skin manifestations?

Answer 74

prevent sun exposure, anti-malarials, topical GC

Question 75

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

Answer 75

mmf

Question 76

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

Answer 76

AZA or mmf

Question 77

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

Answer 77

anti-platelet, anti-coag

Question 78

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

Answer 78

GC +/- IS

Question 79

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

Answer 79

Scleroderma

Question 80

Is there marked pt-pt variability is Scleroderma?

Answer 80

yes

Question 81

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

Answer 81

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

Question 82

What does genetic analysis say about sleroderma?

Answer 82

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

Question 83

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

Answer 83

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

Question 84

What is fibrosis associated with in scleroderma?

Answer 84

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

Question 85

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

Answer 85

T

Question 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.

Answer 86

Systemic sclerosis

Question 87

What is the difference between early limited and diffuse scleroderma

Answer 87

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

Question 88

Describe GI problems in systemic sclerosis

Answer 88

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

Question 89

Is lung disease a major cause of morbitidy in scleroderma?

Answer 89

yes

Question 90

Does pulmonary fibrosis occur in diffuse and limited sclerosis?

Answer 90

yes- variable in prognosis and severity

Question 91

______ has the worse prognosis with ILD in scleroderm.

Answer 91

nonwhites an anti-topo1 positive pt

Question 92

Tx for scleroderma drelated ILD

Answer 92

immunosuppresion

Question 93

What are risk factors for scleroderma lung

Answer 93

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

Question 94

The degree of lung fibrosis on ______ predicts outcom

Answer 94

HRCT

Question 95

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

Answer 95

in 5-10% OF PT

Question 96

What are risk factors for scleroderma renal crisis

Answer 96

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

Question 97

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

Answer 97

ACEis