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Musculoskeletal System > Pathology- Autoimmune Diseases > Flashcards

Pathology- Autoimmune Diseases Flashcards

1
Q

What are autoimmune disorders?

A

When the immune response is against self-antigens, and the body cannot differentiate “self” from “non-self”

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2
Q

What is systemic lupus erythematosus (SLE)?

A

multisystem inflammatory disease which involves the skin, joints, kidney and other organs.

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3
Q

What are the most important diagnostic antibodies (Ab)?

A

those against nuclear proteins (ANA). 2x (double stranded). Sm (anti Smith). Antihistone Ab.

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4
Q

What is the pattern of nuclear staining on a + ANA test for SLE?

A

homogenous or diffuse nuclear staining

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5
Q

What is a special pattern of staining that is more characteristic of SLE on a ANA test?

A

Rim pattern

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6
Q

What is the etiology of SLE?

A

unknown

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7
Q

What is most of the damage caused by in SLE?

A

vasculitis

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8
Q

Where in the body does SLE occur?

A

All over- immune complexes deposit in all tissues

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9
Q

What is the prevelence of SLE?

A

mainly women (9:1), especially in childbearing age, 2-3x high in blacks and hispanics.

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10
Q

What is the skin presentation in SLE?

A

erythematous rash in sun, “butterfly” rash on face is most characteristic.

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11
Q

What happens in the joints of 90% of SLE patients?

A

polyarthralgia

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12
Q

What is polyalthralgia?

A

inflammatory synovitis without joint destruction, often with pain (1st sign of SLE)

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13
Q

What happens in the kidneys of 75% of SLE patients?

A

glomerularnephritis

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14
Q

What Ab is mainly involved with glomerularnephritis in SLE?

A

IgG

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15
Q

What is the mesangial lupus glomerularnephritis?

A

Mildest form, where immune complexes and complement are found in the mesangium. prognosis is excellent

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16
Q

What is diffuse proliferative lupus nephritis?

A

most serious form of renal disease in SLE. epithelial crescents present. many pt’s progress to renal failure.

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17
Q

What might occur in the respiratory system of SLE pts?

A

Diffuculty breathing, wheezing and chest pain during physical activity might occur

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18
Q

What might occur in the CNS of SLE pts?

A

vasculitis might lead to hemmorage, memory loss, anxiwty and depression are neurological Sx of lupus

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19
Q

What might occur in the heart of SLE pts?

A

pericarditis is the most common finding, Libman-Sacks endocarditis with sterile vegitations.

20
Q

How can you differentiate drug-induced lupus from SLE?

A

no sex predominance, no CNS or renal involvement, Ab to histones

21
Q

What is chronic discord lupus (DLE)?

A

affects the skin but only in sub exposed patients, no organ system involvement

22
Q

What types of rash form on the skin of DLE pt’s?

A

circular rashes and papular/anular rings on trunk

23
Q

How can you differentiate DLE from SLE from lesions on the skin?

A

in DLE the areas without the rash will not have the + ANA test because DLE is not systemic

24
Q

What are some clinical features of Sjoren syntrome (SS)?

A

keratoconjunctivitis (dry eyes), xerostomia (dry mouf), possible parotid enlargement

25
Q

What population does SS affect?

A

women aged 30-65 (2nd most common connective tissue diesease after SLE)

26
Q

SS patients have a 40% increase risk of developing what type of tumors?

A

lympomas (esp on the skin)

27
Q

There is a widespread deposition of what molecules in Progressive Systemic Sclerosis (PSS)?

A

collagen

28
Q

What tissues are affected in PSS?

A

skin and organs (esp the kidney)

29
Q

What are some Ab’s specific for PSS?

A

nucleolar Ab to RNA polymerase, Ab to Scl-70

30
Q

What is the staining pattern of PSS?

A

nucleolar pattern

31
Q

What is the clinical presentation of PSS?

A

arteries, arterioles and capillaries are destroyed

32
Q

What happens in the kidneys of PSS?

A

fibrinoid necrosis in afferent arterioles

33
Q

What occurs in generalized PSS?

A

severe and progressive disease of the skin and organs

34
Q

What the the progression of PSS?

A

early Sx of Raynauds, followed by edema in fingers and hands including a “stone face,” and then organ involvement

35
Q

What are the color changes in Raynauds?

A

White –> Blue –> Red

36
Q

Why does the “stone face” occur in generalized PSS?

A

there is atrophy of the muscles in the temple, face and neck. these changes are called Mauskopf (mousehead).

37
Q

What occurs in diffuse cutaneous CREST?

A

Calcinosis, Raynauds, Esophageal dysmotility, Sclerodactyly, Telangiectasia

38
Q

What is the most common form of myopathies?

A

juvenile dermatomysitis

39
Q

What is the prevelence of myopathies?

A

3:1 prevelence in blacks vs whites, 2:1 female, may overlap other diseases like SLE, PSS, and SS.

40
Q

What is the clinical features of polymyositis?

A

chronic inflammatory muscle disease, affects adults 40-50’s, no cutaneous involvement

41
Q

What are the clinical features of dermatomyositis?

A

inflammatory involvment of the skin and muscles, difficulty standing or walking, liliac rash of eyelid, grotten lesions over knucles

42
Q

What % of dermatomyositis pt’s develip cancer?

A

20-25%

43
Q

What types of diseases comprise the “Mixed connective tissue disease”?

A

SLE, PSS, polymyositis/dermatomyositis

44
Q

Where is the Ab directed in Mixed connective tissue disease?

A

A ribonucleoprotein particle containing U1 ribonucleoprotein (including Jo-1 and PM-1)

45
Q

Where is the Ab NOT directed in Mixed connective tissue disease?

A

dsDNA

46
Q

What is the staining pattern in Mixed connective tissue disease?

A

Speckles pattern (they bind to the riubonucleoproteins)

Musculoskeletal System (8 decks)

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