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Pathology > Immunopathology III > Flashcards

Immunopathology III Flashcards

1
Q

What is Sjogren’s syndrome?

A

Autoimmune disease against Exocrine glands, primarily lacrimal and salivary glands

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2
Q

Who is usually affected with Sjogren’s?

A

Middle aged women

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3
Q

What is sicca syndrome? How

A

dry eye/mouth alone, without other involvement. $05 of Sjogren’s syndrome

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4
Q

What is the pathogenesis of Sjogren’s?

A

CD4+ T cells against glandular epithelial self Ag induced by a viral infection

Also Systemic B cell hyperactivity

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5
Q

What are the infections that are associated with Sjogren’s?

A

EBV, hep C

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6
Q

What are the specific ANA’s associated with Sjogren’s?

A

SS-A and SS-B

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7
Q

True of false: Rheumatoid factor is also seen with Sjogren’s?

A

yes, 75%

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8
Q

What causes the symptoms of Sjogren’s?

A

Attack by immune system, causing hyperplasia, fibrosis, and follicle formation

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9
Q

What is the predominate inflammatory cells in SJogren’s

A

Lymphocytes

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10
Q

What are the histological changes seenin Sjogren’s?

A

Ductal hyperplasia, inflammatory cells destroying glands

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11
Q

What are the symptoms of Sjogren’s?

A

Xerostomia
Keratoconjunctivitis
Nasal septal erosions

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12
Q

What is the lymph node characteristics of Sjogren’s?

A

Massively hyperplastics d/t hyperplasia

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13
Q

What lymphoma is commonly seen in Sjogren’s

A

B cell (Marginal zone) lymphoma

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14
Q

What are the systemic symptoms of Sjogren’s?

A

Vascultiis
renal problems
Skin problems
Perpheral neuropathy

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15
Q

What protein is associated with Sjogren’s?

A

SS-A-Ab

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16
Q

Which systemic symptoms are more prominant, SLE or sjogren’s ?

A

SLE

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17
Q

What is the cause of complications of Sjogren’s?

A

Infects major organs

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18
Q

What is systemic sclerosis?

A

Autoimmune disorder characterized by chronic inflammation, destruction of small vessels, and progressive tissue fibrosis

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19
Q

Who gets systemic sclerosis?

A

50-60 yo females

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20
Q

What are the major problem places in systemic sclerosis?

A

Heart, GI, or lung involvement

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21
Q

What are the triggers of systemic sclerosis?

A

T and B cells autoactivation driving fibrosis

Blood vessel narrow

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22
Q

What is the major cytokine in Systemic sclerosis that causes increased deposition of ECM?

A

TGF-beta

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23
Q

What is the major cytokine in Systemic sclerosis that causes increased deposition of ECM?

A

TGF-beta

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24
Q

What is CREST syndrome?

A

Limited variant of scleroderma

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25
Q

What is the anti ab seen in CREST syndrome?

A

Anti-centromere

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26
Q

What are the symptoms of CREST syndrome?

A 
Calcinosis
Raynaud's phenomenon
Esophageal dysmotility
Sclerodactyly
Telangiectasia
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27
Q

What is Raynaud’s syndrome seen in CREST?

A

vasospasms caused intermittent ischemia to digits

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28
Q

What is sclerodactyly seen in CREST syndrome?

A

Tapered digits

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29
Q

What is telangiectasia seen in CREST syndrome?

A

Purpuric lesions of the skin and mucous membranes

30
Q

What is the diffuse variant of Scleroderma?

A

Widespread involvement with rapid progression

31
Q

What is the Ab seen in diffuse scleroderma?

A

DNA topoisomerase I (anti-Scl-70)

32
Q

What are the early skin changes in scleroderma?

A

Edema Lymphocyte infiltrates

33
Q

What are the late skin changes with scleroderma?

A

Epidermal thinning
Dermal fibrosis
Subcutaneous calcifications

34
Q

What causes the autoamputation of digits seen in scleroderma?

A

Vascular fibrosis, leading to ischemia

35
Q

What are the early skin changes in systemic scleroderma?

A

Edema Lymphocyte infiltrates

36
Q

What is the mask face of systemic scleroderma?

A

Fibrosis causing pulled appearance of skin

37
Q

What causes the GI changes, such as LES dysfunction, seen in systemic scleroderma?

A

Fibrosis or the muscularis

38
Q

What causes the GI changes, such as loss of villi, seen in systemic scleroderma?

A

Fibrosis of the muscularis

39
Q

What are the MS early and late changes seen in systemic Scleroderma?

A 
Early = nondestructive hyperplasia and inflammation
Late = Fibrosis of synovial and periarticular CT
40
Q

What percent of Systemic scleroderma have myositis with lymphocytic infiltrate?

A

10%

41
Q

What are the renal changes in systemic sclerosis?

A

Thickening of interlobular arteries via concentration of intimal cells

42
Q

What are the renal changes in systemic sclerosis? What can be a consequence of this?

A

Thickening of interlobular arteries via concentration of intimal cells

HTN may develop

43
Q

What are the pulmonary changes seen in systemic scleroderma?

A

Alveolar fibrosis, pulmonary HTN

44
Q

What are the pulmonary changes seen in systemic scleroderma?

A

Alveolar fibrosis (restrictive disease), pulmonary HTN

45
Q

What are the cardiac changes seen in systemic scleroderma?

A

Pericarditis with effusion
Perivascular lymphoid infiltrates
Arteriolar thickening

46
Q

What type of lung and heart problems develop with systemic scleroderma?

A

Restrictive cardiomyopathy,

47
Q

What type of lung and heart problems develop with systemic scleroderma?

A

Restrictive lungs disease/cardiomyopathy,

48
Q

What are the causes of death in systemic sclerosis?

A

Renal
Cardiac
Pulmonary
GI dysfunction/failure

49
Q

What is RA?

A

Systemic, autoimmune inflammatory disorder

50
Q

What are the parts of the body affected by RA?

A

Joints, skin, vessels, heart, lungs, soft tissue

51
Q

What is the antigenic trigger for scleroderma or RA?

A

Unknown

52
Q

What is the gene associated with RA? What is the protein that this codes for?

A

HLA-DRB1, coding for PTPN22

53
Q

What is the MOA behind RA?

A

Autoimmune rxn by CD4 T cells, causing production of cytokines, and activation of macrophages, B cells

54
Q

What is rheumatoid factor?

A

IgM auto-Ab to Fc portion of autologous IgG (antigen + IgG, antibody = IgM)

55
Q

What is the peptide that has been associated with RA?

A

Citrullinated peptides

56
Q

What type of hypersensitivity is RA?

A

Type III, II

57
Q

What is the MOA behind RA?

A

CD4 T cells cause production of cytokines/activation of macrophages, B cells in joints

Immune complex deposition in joints

58
Q

What is deposited on synovial surfaces in RA?

A

Fibrin and granulation tissue, forming a pannus

59
Q

Where are rheumatoid nodules found?

A

Pressure points (elbows, occiput, lumbosacrum)

60
Q

What causes the vasculitis seen in RA?

A

RF-IgG complexes

61
Q

What are the histological characteristics of a rheumatoid nodule?

A

Area of fibrous tissues, with necrosis in the center

62
Q

What are the clinical symptoms of RA?

A

highly variable–malaise, pain, swelling etc

63
Q

What are the usual causes of death with RA?

A

Amyloidosis

Drug therapy complications

64
Q

What is Juvenile idiopathic arthritis?

A

Large joint oligoarthritis

65
Q

What are the systemic features of Juvenile idiopathic arthritis?

A

Pericarditis
myocarditis
Pulmonary fibrosis
Glomerulonephritis

66
Q

Is there ANA in juvenile idiopathic arthritis? RF?

A

ANA +

RF -

67
Q

Juvenile idiopathic arthritis + febrile illness, rash, hepatosplenomegaly, leukocytosis = ?

A

Still’s disease

68
Q

What is mixed CT disease?

A

Mixed SLE and systemic sclerosis at the same time

69
Q

What is the Ab seen in mixed CT disease?

A

Anti-U1RNP) to ribonucleoprotein

70
Q

What makes mixed CT disease clinical distinctive

A

minimal renal disease, and good response to steroids

71
Q

What is the complication of mixed CT disease?

A

Progression to SLE or SS

Pathology (32 decks)

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