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Pathology 2 > 5.8 Autoimmune diseases > Flashcards

5.8 Autoimmune diseases Flashcards

1
Q

Presentation of Lupus

A

varried, you’ll see many different things like buterfly rash of malar eminences

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2
Q

Lupus is typically found in

A

Females – female preponderance 9:1 ratio at childbearing ages. Much lover in older pts or children. Presentation extremely variable. Classically young female with butterfly rash, multiple peripheral joint pain, no deformity, fever, photosensitivity, pleuritic chest pain

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3
Q

why is there photosensitivity in lupus

A

bc the rash becomes worse on sulight exposure

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4
Q

what is pleuretic chest pain

A

it is a sharp pain due to wrinkle in the pleura, ppl with MI or heart attack describe crushing pain, people with epigastric pain describe burning

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5
Q

what race is lupus more common in

A

more common and severe in black women

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6
Q

what kind of antibodies do you find in lupus

A

nuclear, ss-DNA, ds-DNA, histones, plasma protine, phospholipid, RBC, platelets, and other cells

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7
Q

Which Ab are cross reactive

A

Anti-PL ab will cross react

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8
Q

what problem dose Anti PL ab cause

A

Anti-pl will interfere with clotting. Counter intuitively it will increase PTT but what is really happening is that Anti-PL ab bind to platelets making them sticky making you prone to thrombosis. So anti-PL are actually prothrombotic. But you see a longer PTT bc the Anti-PL ab acutally interferes with the test itself.

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9
Q

Lupus and genetics

A

familial, 20% concordance in monozygotic twins. Non- affected twin often has same autoantibodies

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10
Q

Drugs that induce lupus

A

hydralizine, chelating agents, antihypertensives

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11
Q

Sex hormones affecting lupus

A

important bc of fluctuations of female predominance depending on age?

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12
Q

Lupus - type of hypersensitivity

A

Type II bc there are antibodies directed at plateltes clearing or lysing them, and Type III due to complex fromation and deposition especially in kidneys

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13
Q

Organs affected in lupus

A

many organs affected, small vessel vasculitis (type III) common mechanism. Renal manifestations are frequent cause of death

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14
Q

Genetic factors affecting Lupus

A

twins (20% concordance in monozygous twins), specific alleles in HLA-DQ locus, 6% of patients have inherited deficiencies in early complement components (C1q, C2, or C4), C4 KO mice get lupus like disease, NZBxNZW F1 mice have lupus and changes in over 20 loci

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15
Q

Environmental factors affecting Lupus

A

drugs, UV light, sex hormones

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16
Q

immunologic factors affecting lupus

A

large number of irregularities, polyclonal b cell activation, analysis of anti ds-DNA ab suggests specific autoreactive clones. CD4 cells the villain. Impaired clearing of apoptotic cells, Type II and III types of damage, Epitope spreading

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17
Q

sequence of events in lupus

A

defect in apoptotic body clearance –> exposure to sequestered ag (eg. DNA) –> genetic predisposition (MHCII polymorphisms) –> presentation of self ag –> generation of auto-ab

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18
Q

Why is it called systemic lupus erythematosis

A

bc almost all organsystems are affected _ renal are usually almost always present

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19
Q

Renal problems in lupus

A

glomerulonephritis – normal by light microsope, mesangial, focal proliferative, diffuse proliferative, or membranous

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20
Q

Skin problems in lupus

A

buterfly rash on face but may be on extremities; liquifactive degeneration of basal layers causing blistering or bulla formation, vasculitis with fibrinoid necrosis

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21
Q

Joint problems in lupus

A

non-erosive synovitis

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22
Q

CNS problems in lupus

A

vasculities, occlusion of small vessels from intimal proliferation

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23
Q

Heart problems in lupus

A

pericarditis, endocarditis, coronary artery disease – due to hypercoaguable state

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24
Q

Lung problems in lupus

A

pleurities

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25
Q

Spleen problems in lupus

A

enlargement, onion skinning of arterioles

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26
Q

what is always see in lupus patients

A

hematologic disorders

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27
Q

Diagnosis of Lupus (4/11) MD SOAP BRAIN

A

Malar rash, Discoid rash, Serositis, Oral ulcers (includes oral or nasopharyngeal ulcers), Arthritis, Photosensitivity, Blood–hematologic symptoms, Renal disorder, Antinuclear ab test positive, Immunologic disorder, Neurologic disorder

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28
Q

If you see a healthy woman with seizures and have ruled everything else out what should you think of

A

brain tumor or lupus

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29
Q

Problem with Anti PL

A

false positive on you VDRL. The VDRL test lets you know if you should test for presence of spirochetes. If you test positive on VDRL, but then test neg when you check for Ab to spirochetes you might have lupus.

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30
Q

in lupus, diffuse nuclear ab can be directed towards

A

chromatin, histons, and ds-DNA

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31
Q

in lupus, rim or peripheral ab can be directed toards

A

ds-DNA

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32
Q

in lupus, speckled ab can be directed towards

A

non-DNA components RNP, SS-A, SS-B

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33
Q

in lupus, nucleolar pattern ab can be directed towards

A

RNA proteins. Usually systemic sclerosis

34
Q

slide 59

A

v. important chart

35
Q

many autoimmne disease like SLE, systemic sclerosis, limited scleroderma, sjogren syndrome, and inflammatory myopathies have

A

ANA - anti nulcear ab –> sensitive test not specific —lots of false positives —lupus is the highest though

36
Q

in 40-60% of lupus pts you find

A

anti ds-DNA ab –> specific but you get lots of false negatives. If they have the ab, they pretty much have lupus, but if they don’t have ab, they might still have lupus

37
Q

In 20-30% of lupus pts you find

A

anti smith ab

38
Q

What diseases do you see Ro/L (SS-A/B RNPs)

A

Sjorgren > SLE

39
Q

What disease do you see anti Histidyl-t-RNA synthetase (Jo-1)

A

inflammatory myopathies – 25%

40
Q

What disease do you see anti centromere ab

A

Limited scleroderma > Systemic sclerosis

41
Q

What disease do you see anti DNA topoisomerase ab

A

Systemic slerosis > Limited Sleroderma

42
Q

What disease do you see anti U1RNP ab

A

SLE >Systemic sclerosis> Limited Scleroderma

43
Q

clinical course of lupus

A

variable and unpredictable, ranging from severe to mild, treatemtn with steroids and immunosuppression yeilds long term remissions with occasional flare-ups, prone to infection

44
Q

what is the survival rate of lupus

A

90% 5 yr survival, 80% 10yr survival

45
Q

what is the most common cause of death in lupus

A

renal failure – coronary vasculuar disease is becoming a more important cause of death

46
Q

Renal manifestations of Lupus

A

With light microscopy up to 70% of pts show changes, but with EM and immunofluoresecne 100% of pts show changes –> diffuse proliferative, focal proliferative, mesangial GN, membranous, **wire loop lesions with extensive deposits

47
Q

what do you see in end stage glomerulonephritis

A

lots of scarred glomeruli, will present as no more pee, inc creatinine

48
Q

where do you see skin changes histologically in lupus

A

the dermal epidermal border – formation of blisters and bulli

49
Q

what do you see in the spleen histologically in lupus

A

onion skinning around the arteriole

50
Q

Libman-Sacks endocarditis in lupus

A

anti PL makes person hypercoaguable and you can get deposits of thrombi in endocardium of the heart on the underside of the leaflets, these are sterile and not due to any infectious agents, they have a bead like appearance, they aren not usually that symptomatic but on the left side they can break off and cause stroke

51
Q

chronic discoid lupus

A

confined to skin lesions, some develop systemic disease later, only 35% positive for ANA rare anti ds-DNA, same immunofluorescnece findings as SLE

52
Q

subacute cutaneous lupus

A

skin involvement with mild systemic disorder

53
Q

Drug induced lupus

A

hydralazine, procainamide, isoniazid, D-penicillamine (chelating agent) induces SLE and disease stops on drug withdrawal

54
Q

What are you worried about in lupus

A

the hypercoagualbe state theat you’re in due to the Anti PL(remember will give a flase + VDRL) bc if you have clotting and thrombosis when you’re pregnant that can lead to damage to placenta and abortion of the baby

55
Q

Sjorgren Syndrome

A

50-60 y/o females, dry eyes (keratoconjunctivites, sicca syn), dry mouth (xerostomia), immunologically mediated destruction of lacremal and salivary glands, can occur as an isolated disorder but commonly secondary in other AI disorders such as lupus and rheimatoid arthritis

56
Q

Histologically what do you see in Sjorgren Syndrome

A

lymphocytic infiltration of glands (CD4, Bcells, and plasma cells), over time dominant clone of B cells emerges

57
Q

what kind of Abs do you see in sjorgren syndrome

A

several ANAs and RNP ab but SS-A and SS-B positive in 90% of pts (also 30-50% of lupus), many rheumatoid factor positive

58
Q

what cells are defective in Sjorgren

A

CD4s

59
Q

high titers to SS-A predict what in Sjorgren

A

extraglandular involvment including nose, respiratory system

60
Q

what secondary effects do you see in Sjorgren

A

ulcerated cornea, cracks and fissures in mouth

61
Q

what cancer is there a high incidence of in Sjorgren

A

B-cell lymphomas

62
Q

why do you do a lip biopsy for sjorgren

A

bc the lips have minor salivary glands for you to look at and see if affected

63
Q

Systemic Sclerosis (Scleroderma)

A

diffuse and localized forms of interstitial fibrosis in skin and other organs

64
Q

CREST syndrome (localized) in Systemic Sclerosis stands for

A

calcinosis, Raynaud’s, esophageal dysmotility, sclerodactyly, telangiectasia

65
Q

Systemic sclerosis is seen in

A

females 50-60

66
Q

what kind of malfunctions are see in systemic sclerosis

A

GI, myocardial, and renal malfunctions

67
Q

What kind of Renal involvement is related to Systemic sclerosis

A

renal involvement is vascular (not glomerular) due to vasculites that will cause an infarct and reanl failure leading to death in 50% of ts. Also inductino of malignant hypertension.

68
Q

What other systems are affected in Systemic sclerosis

A

Pulmonary vessel involvement puts SS in differential for pulmonary hypertension, GI tract affected in 90% especially esophagus. Loss of villi in small bowel can induce malasorption.

69
Q

What kind of antibodies are seen in Systemic slerosis

A

Anti-topoisomerase and centromere ab

70
Q

What happens to the skin in Systemic sclerosis

A

skin gets stretched, and the skin becomes attenuated and shiny, and there is a loss of wrinkles

71
Q

What can happen to the mouth in Scleroderma

A

cheilosis at the corners of the mouth from riboflavin deficiency as a result of the malabsorbtion that can occur with scleroderma

72
Q

Why is peristalsis affected in Systemic sclerosis

A

normally the submucosa should be thin, but in this condition there it is thick with lots of connective tissue interfering with peristalsis. There is also loss of villi affecting absorption

73
Q

Dermatomyositis

A

lilac discoloration of eyelids and periorbital edema, slow onset muscle weakness, bilateral, symmetrical, proximal, climbing steps affected before digital fine motor control, increased risk of cancer —> bilateral conditions tend to be systemic, attack large muscle first

74
Q

Age of onset of Dermatomyositis

A

any age

75
Q

in Dermatomyositis what is happening on a deeper level

A

perivascular and perimysial inflammation with some atrophic fibers. Quantitatively, dramatic decrease in number of capillaries –> vessels are being killed in the muscle

76
Q

Polymyositis

A

very similar to dermatomyositis but no skin involvement, occurs mainly in adults, killing of muscle fibers by CD8 lymphosytes, necrotic regenerating fibers, puter type 4 hypersensitivity, thends to be in big muslces first so strength is a problem

77
Q

Inclusion body Myositis

A

begins in distal muslces, affects pts over 50, CD8 cells present but antiinflammatory therapy doesn’t work, inclusion bodies in muscle diagnostic, some stain with congo red i.e. amyloid positive, fine motor control affected first, steroids don’t work here

78
Q

What ab do you see in myopathies

A

anti histidyl RNA syntetase ab

79
Q

Mixed connective Tissue Disease

A

pts have symptoms of SLE, polymoyosits, and systemic sclerosis, very little renal disease and good responses to steroids, bc of overlap of disease many question if this is a separate disease

80
Q

In mixed connective tissue disease what do you find high titers of

A

RNP particles containing RNP U1

Pathology 2 (15 decks)

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