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Robbins Pathology MOD1 (Chptr 1-6) > Chapter 6- SLE and autoimmune dieases > Flashcards

Chapter 6- SLE and autoimmune dieases Flashcards

1
Q

What condition regarding the esophagus is common in scleroderma

A

Dysphagia due to esophageal fibrosis, in almost 50% of patients

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2
Q

What are the characteristics of type 3 renal lesions aka focal lupus nephritis

A

Involvement of less that 50% of all glomeruli

-Red cell casts in the urine

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3
Q

What are the diagnostic antibodies in SLE

A

Antibodies to dsDNA and Smith (sm) antigens

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4
Q

What is polyarteritis nodosa characterized by

A

Necrotizing inflammation of the walls of blood vessels

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5
Q

Patients with high titers of SS-A are more likely to what

A

Early Sjogren syndrome onset, longer disease duration, and extraglandular manifestations, such as cutaneous vasculitis and nephritis

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6
Q

What is the percent of cardiac involvement in SLE

A

50% of pts

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7
Q

What are the characteristics of class 2 renal lesion aka mesangial proliferative lupus nephritis

A
  • masangial proliferation and accumulation of matrix

- Deposits of Igs and complement without involvement of glomerular capillaries

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8
Q

What are the most common causes of death in SLE patients

A

Renal failure and inter current infections

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9
Q

Specifically, what are the antibodies in SLE against

A

Antinuclear antibodies (ANA)

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10
Q

Which antibodies are commonly found in patients with Sjogren syndrome

A

Antibodies against two ribonucleoprotiens, SS-A (Ro) and SS-B (La)

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11
Q

What is the epidemiology of scleroderma

A

3:1 female to male ratio, with peak in 50-60

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12
Q

Which disease is sicca disorder associated with

A

Aka Sjogren syndrome, and is assocaited with RA

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13
Q

What percent of lung presentation is seen in SLE

A

50% of pts have pleuritis or pleural effusion

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14
Q

What is keratoconjunctivitis sicca

A

Dry eye

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15
Q

What is assocaited with scleroderma (systemic sclerosis) early in the course presentation

A

Vascular damage, especially microvascular

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16
Q

What is the changes seen in skin during scleroderma (systemic sclerosis)

A

Diffuse atrophy of skin, beginning in the fingers and distal regions

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17
Q

How does lupus antibodies affect clotting time

A

In Vitro, it will increase the PTT timing

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18
Q

What are the complications of mixed connective tissue disease

A

Pulmonary hypertension, interstitial lung disease, renal disease

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19
Q

What is the most prominent renal abnormality in scleroderma

A

Vascular lesion and interlobular arteries show thickening due to fibrosis

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20
Q

What is the percentage of scleroderma patients that have renal involvement

A

2/3

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21
Q

What is xerostoma

A

Dry mouth

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22
Q

What is the characteristic of joints in SLE

A

Nonerosive synovitis with little deformity (as opposed to RA)

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23
Q

Which HLA is more common to form a reaction following administration of hydralazine

A

HLA-DR4

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24
Q

Which HLA is more common to form a reaction following administration of Procainamide

A

HLA-DR6

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25
Q

What is the skin presentation in SLE

A 
Butterfly rash (50% of pts)
-Urticaria, bullae, maculopapular lesions, ulceration
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26
Q

What are possible effects of the changes to esophagus in scleroderma

A

GERD and subsequent Barrett’s metaplasia

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27
Q

What is libman sacks endocarditis

A

Aka valvular endocarditis

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28
Q

What is limited scleroderma

A

Skin involvement and confined to fingers, forearms, and face

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29
Q

What are the four categories of antinuclear antibodies (ANAs)

A

1) antiDNA
2) Antihistone
3) anti nonhistone proteins attached to RNA
4) Antinucleolar antigens

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30
Q

Patients with scleroderma and anti topoisomerase antibodies (anti-Scl-70) are more likely to develop which condition

A

Pulmonary fibrosis and peripheral vascular disease

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31
Q

What percentage of patients with scleroderma have ailment tract involvement

A

90%

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32
Q

How is scleroderma associated with malnutrition

A

Loss of microvilli in the small bowel

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33
Q

What is the etiology of Sjogren syndrome

A

Decrease in tears and salivary glands as a result of lymphocyte infiltration and fibrosis

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34
Q

What does the infiltrate in Sjogren Syndrome Primarily contain

A

CD4+ T cells

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35
Q

What are the characteristics of class 4 renal lesions aka diffuse lupus nephritis

A
  • most common and most severe
  • similar to 3, except more that 50% of glomeruli involved
  • May form the wire loop look aka vascular thickening
  • Proteinuria and hematoureia
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36
Q

What is the condition of chronic discoid lupus erythematosus

A

Skin manifestations mimic SLE, but no systemic presentations

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37
Q

What is noninfectious vasculitis

A

Conditions where vasculitis occurs due to something other than a direct infection of the vascular wall, such as an abcess

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38
Q

What percentage of Sjogren patients are likely to develop lymphoma

A

5%, which is 40 times higher than normal (due to development of a dominant B cell clone)

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39
Q

What gender and age range is Sjogren syndrome common

A

Women between the ages of 50 to 60

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40
Q

What is the likely antigen in Sjogren syndrome

A

Alpha-fodrin, a cytoskeletal protein

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41
Q

In patients with scleroderma, what percentage of patients have lung involvement and what are the two common complications

A

50%, Most commonly with pulmonary hypertension and interstitial fibrosis

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42
Q

Damage during SLE is generally due to what

A

Deposition of immune complexes and antibodies binding to various cells and tissues

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43
Q

Mixed connective tissue disease is characterized by high titer levels of what

A

Ribonucleoprotein particle containing U1 ribonucleoprotein

44
Q

Deficiency in which complement proteins may lead to SLE

A

C1q, C2, C4

45
Q

What is the genetic and antibody association with subacute cutaneous lupus erythematosus

A

Antibodies against SS-A antigen

HLA-DR3 genotype

46
Q

What portions of the heart are usually affected by SLE

A

Mitral and aortic valves, usually by leaflet thickening

47
Q

How does SLE result in hypocomplementemia

A

Flare ups cause the increase formation of immune complexes, which exhaust the amount of complement proteins

48
Q

What are the clinical presentations of chronic discoid lupus erythematosus

A

Skin plaques, edema, scaliness, skin atrophy surrounded by erythematosus border

49
Q

What is the primary cause of death in patients with scleroderma

A

Pulmonary disease

50
Q

Pulmonary fibrosis in scleroderma commonly causes which condition

A

Right side cardiac dysfunction, leading to arrhythmias and cardiac failure

51
Q

What presentation in bone marrow is indicative of SLE

A

LE bodies in the bone marrow

52
Q

What percentage if patients with scleroderma die of renal failure

A

50%

53
Q

What does speckled pattern staining indicate

A

Aka uniform or variable sized speckles. Indicates non-DNA nuclear components, such as Sm antigen, and SS-A and SS-B antigens

54
Q

What percentage of Lupus patients contain antiphospholipid antibodies

A

30-40%

55
Q

How common is SLE in women of child bearing age

A

1 in 700

56
Q

What are LE or hematoxylin bodies

A

Damaged cell nuclei are bound by ANA, lose their chromatin pattern and become homogeneous. Usually results in LE cells, which are neutrophils or macros with an engulfed nucleus of injured cell

57
Q

What is the level of dsDNA antibodies in chronic discoid lupus erythematosus

A

Rarely present

58
Q

What are the state of the fingers in late stages of scleroderma

A

They become claw like, tapered,with very limited motion in the joints, andthe face becomes a drawn mask

59
Q

What are patients with antiphospholipid syndrome more likely to develop

A

-Venous and Arterial thrombosis
-Miscarriages
Focal cerebral or ocular ischemia

60
Q

What race is scleroderma more common in

A

Black, especially black women

61
Q

What association does the parotid gland have with Sjogren syndrome

A

Enlarged in 50% of pts

62
Q

What are three drugs that can induce a SLE like attack

A

1) Hydralazine
2) Procainamide
3) D-penicillamine

63
Q

What class of renal lesion is most uncommon

A

1

64
Q

What are the race ratios of SLE

A

2 to 3 times higher for blacks and Hispanics compared to whites

65
Q

What is the ration of women no child bearing age to males

A

2 to 1 females to males

66
Q

What are the state of LN in Sjogren patients

A

Enlarged lymph nodes due to hyperplasia

67
Q

What percentage of scleroderma patients have Raynaud phenomenon

A

Virtually all cases

68
Q

What are the drugs that can induce a lupus like affect

A

1) hydralazine
2) Procainamide
3) Isoniazid
4) D-penicillamine
5) Anti-TNF

69
Q

What does a centromeric pattern indicate

A

Antibodies against centromeres, common in systemic sclerosis

70
Q

What is Mikulicz syndrome

A

Enlargement of the lacrimal or salivary glands due to any reason

71
Q

Which viruses are associated with Sjogren syndrome

A

Human T lymphoproliferative virus (HTLV), HIV, Hep C

72
Q

What are the two types of ANAs that are strongly associated with scleroderma

A

1) DNA topoisomerase (antiScl-70)

2) anticentromere

73
Q

What are the common SLE presentations in the spleen

A

-Splenomegaly, capsular thickening, follicular hyperplasia

74
Q

How are SLE flare ups usually treated

A

Corticosteroids or other immunosuppressive drugs

75
Q

Where in the ailment tract are patients with scleroderma most affected

A

Any portion will undergo fibrosis, but the esophagus is the primary location (forms a rubber hose like inflexibility.

76
Q

Which HLA alleles are common in patients with antibodies to SS-B or SS-A

A

HLA-DQA1 and DQB2

77
Q

In patients with Sjogren and high titers of SS-A are more likely to have which conditions

A

-synovitis, diffuse pulmonary fibrosis, and peripheral neuropathy

78
Q

What is the involvement of TLR in activation of autoreactive B cells

A

Self reactive B cells also are activated by TLR to pathogenic DNA/RNA. This serves as the second signal to activate and produce autoantibodies

79
Q

What have the HLA-DQ allele been linked to

A

Antibodies against dsDNA, anti-Sm, antiphospholipid

80
Q

What does rim or peripheral staining patterns indicate

A

Antibodies to dsDNA and some envelope proteins

81
Q

What is essential for the diagnosis of Sjogren syndrome

A

Biopsy of the upper lip to examine minor salivary glands

82
Q

What is Sjogren syndrome characterized by

A

Dry eyes and dry mouth via destructions of lacrimal and salivary glands

83
Q

What is CREST syndrome

A

Calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia

84
Q

Patients with scleroderma and anticentromere antibodies are more likely to develop which condition

A

CREST syndrome

85
Q

Mixed connective tissue disease presents with which clinical findings

A

Synovitis of the fingers, Raynaud, mild myositis

86
Q

What is the most widely used method for detecting ANAs

A

Indirect immunoflourescence

87
Q

What does nucleolar pattern staining indicate

A

Antibodies against RNA, most common in MS

88
Q

What are the general mechanisms of tissue injury

A

1) Deposition of immune complexes
2) Autoantibodies against RBCs, WBCs, and platelets
3) Antiphospholipid antibody syndrome

89
Q

What is diffuse scleroderma

A

Widespread skin involvement, rapid progression, and early visceral involvement

90
Q

What are the characteristics of class 6 renal lesions aka advanced sclerosing lupus nephritis

A

More than 90% of glomeruli, and represents end stage renal diease
-occasionally tubulointerstitial lesions

91
Q

What percent of Sjogren syndrome patients have Rh factor

A

75%

92
Q

What is the ratio of women in child bearing age to men for SLE

A

9 to 1 female to male ratio

93
Q

What are the components recognized by antiphospholipid antibodies

A

1) Prothrombin
2) Annexin V
3) B2 Gp1
4) Protein S and C

94
Q

What class of renal lesions is most common

A

Type 4

95
Q

What is the significance of CD4+ cells involvement in SLE

A

It means that there is T cell dependant antigen, so it must be protein

96
Q

How is subacute cutaneous lupus erythematosus differentiated from chronic discoid

A

Rash is more widespread, superficial, and no scarring

97
Q

What is the earliest form of histological finding of Sjogren syndrome

A

Periductal and perivascular lymphocytic infiltrate

98
Q

What is the significance of Type 1 interferons in the blood of SLE patients

A

It means that antigenic nucleic acids are almost acting as molecular mimicry of their viral counterparts, leading to activation of B cells

99
Q

In drug enduced erythematosus, what antibody is common, and which is more rare

A

Rare to find dsDNA antibodies, but very common to find histone antibodies

100
Q

What are the characteristics of class 1 renal lesions aka minimal mesangial lupus nephritis

A

Immune complex deposition in the mesangium by electron microscopy, but no light microscopy damage

101
Q

What is systemic sclerosis characterized by

A

1) Chronic inflammation
2) Widespread damage to blood vessels
3) progressive interstitial and perivascular fibrosis in skin

Overall, systemic fibrosis

102
Q

What is the fundamental defect in patients with SLE

A

Failure of the mechanism that maintains self tolerance

103
Q

What doe homogenous or diffuse nuclear staining usually reflect

A

Antibodies to chromatin, histones, dsDNA

104
Q

Scleroderma is common with which condition

A

CREST syndrome

105
Q

What is Raynaud phenomenon

A

Vasoconstriction of Arteries and arterioles in extremities

Robbins Pathology MOD1 (Chptr 1-6) (10 decks)

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