2.4 Autoimmune Disorders Part 2 Flashcards

1
Q

What are the components of the RASH OR PAIN mnemonic for SLE?

A
  • Rash (malar)
  • Arthritis
  • Soft tissue/ serositis
  • Heme DO
  • Oral/ nasopharyngeal ulcers
  • Renal dz
  • Photosensitivity
  • ANA
  • Immunosuppressants
  • Neurological DO
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2
Q

Who is the classic SLE patient?

A

Middle aged AA /hispanic female

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3
Q

Is the female:male ratio with SLE more or less pronounced at extremes of age?

A

Less

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4
Q

What is the general pathophysiology of SLE?

A

Antibodies directed against host nuclear material, leading to the activation of C3, C4, and CH50 complement

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5
Q

Which three components of complement are classically depleted with SLE?

A

CH50
C3
C4

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6
Q

What type of hypersensitivity rxn is SLE?

A

III

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7
Q

What is CH50?

A

Test that measures complement C1-C9 levels and ability for complement to lyse RBCs

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8
Q

What are the three classic antibodies that are seen in SLE?

A

ANA
Anti-smith (snRNP)
Anti-dsDNA

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9
Q

What is the classic antibody that is elevated with drug induced SLE?

A

Anti-histone

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10
Q

What is the pathophysiology behind ANA?

A

As cells are damaged, nuclear material is released, exposing B cells to nuclear material, and activating the adaptive immune system.
-Ag/ab complexes are then phagocytosed by APCs, and further activate the immune system

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11
Q

Patients with what deficiency have an increased risk of developing SLE?

A

C1q, C2, C4 deficiency

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12
Q

What are the components of C3 convertase?

A

C1, C4, C2

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13
Q

What is the major function of C3b?

A

Removal of ag/ab complex

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14
Q

What is the function of Cr1 on RBCs?

A

Binds C3b, and takes the bound ag/ab complex to the spleen for removal

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15
Q

A deficiency in what complement components predisposes patients to SLE?

A

C1q
C2
C4

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16
Q

When in particular are malar butterfly rashes present?

A

Upon exposure to sunlight

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17
Q

What are the characteristics of the discoid rash with SLE?

A

Circular rash with erythema and plaques

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18
Q

What are the different forms of serositis that can occur with SLE patients?

A

Pericarditis

Pleuritis

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19
Q

What are the neurological s/sx of SLE?

A

Psychosis or Seizures

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20
Q

What is the most severe form of kidney damage that can occur with SLE?

A

Diffuse proliferative glomerulonephritis

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21
Q

What are some of the hematological disorders that can occur with SLE? (3) What type of hypersensitivity reaction does this represent?

A

Anemia
Thrombocytopenia
Leukopenia

Type II

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22
Q

What is the specific heart abnormality that can occur with SLE? What is this?

A

Libman-Sacks endocarditis

-Wart-like vegetations on both side of a valve

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23
Q

What is the role of anti-dsDNA in the prognosis of SLE?

A

Follows with the course of disease, and is used to predict renal involvement

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24
Q

What, generally, are antiphospholipid antibodies?

A

Autoantibodies against proteins bound to phospholipids

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25
Q

What are the three major antiphospholipid antibodies?

A
  • Anticardiolipin
  • Lupus anticoagulant
  • Anti-beta2-glycoprotein I
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26
Q

Which antibody commonly found in SLE will produce a false positive VDRL and RPR test? Why?

A

Anticardiolipin

-lyses of cells 2/2 treponema pallidum infx releases cardiolipin

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27
Q

Which antibody commonly found in SLE will produce a falsely elevated PTT?

A

Lupus anticoagulant

28
Q

What is the antiphospholipid antibody syndrome associated with SLE?

A
  • Antiphospholipid antibody + a hypercoagulable state

- Leads to DVTs, hepatic vein, placental and cerebral thromboses

29
Q

What is the treatment for APA syndrome?

A

Lifelong anticoagulation

30
Q

What happens to PTT with APA syndrome? What is odd about this?

A

Falsely elevated, despite being a hypercoagulable state

31
Q

What are the top three causes of death in SLE (not in order)?

A
  • CV dz
  • Infx
  • Renal dz
32
Q

What are the three major drugs that cause SLE?

A

Hydralazine
Procainamide
Isoniazid

33
Q

What is the anti-arrhythmic drug that classically causes SLE?

A

Procainamide

34
Q

What is the anti-TB drug that classically causes SLE?

A

Isoniazid

35
Q

What organs are rarely involved with drug induced SLE, as compared to normal SLE?

A

CNS

Renal

36
Q

True or false: removal of the drug that causes SLE usually results in remission

A

True

37
Q

What is the treatment for SLE?

A
  • Avoid sunlight
  • Glucocorticoids
  • NSAIDs
  • Hydroxychloroquine
38
Q

What are the nephrotic and nephrotic syndromes most commonly encounters with SLE?

A

Nephritic = diffuse proliferative glomerulonephritis

Nephrotic = Membranous nephropathy

39
Q

What, generally, is Sjogren’s syndrome?

A

Autoimmune destruction of lacrimal and salivary glands

40
Q

What type of hypersensitivity reaction is Sjogren’s syndrome?

A

IV (lymphocyte mediated with fibrosis)

41
Q

What is the classical presentation of Sjogren’s syndrome?

A
  • Xerophthalmia
  • Xerostomia
  • Bilateral parotid enlargement
42
Q

What are the antibodies that are found with Sjogren’s syndrome? What are these against?

A

SS-A (Ro)
SS-B (La)

Against ribonuclear proteins

43
Q

Can’t chew a cracker, or dirt in eyes = ?

A

Sjogren’s

44
Q

What is the most common autoimmune disease associated with Sjogren’s?

A

RA

45
Q

True or false: rheumatoid factor can be elevated in Sjogren’s syndrome, regardless if the patient actually has RA or not

A

True

46
Q

What is the major issue associated with Anti-SSA and SS-B?

A

Can cross the placenta and cause congenital complete heart block

47
Q

What is the lymphocytic sialadenitis that is found with Sjogren’s syndrome?

A

Lymphocytes attacking the salivary glands of the mouth / lip / parotid

48
Q

What are the three major criteria that are used to diagnose Sjogren’s syndrome?

A
  • Xerophthalmia
  • ANA, SSA/SS-B/ RF +
  • Lymphocytic sialadenitis
49
Q

What hematological malignancy are pts with Sjogren’s syndrome more susceptible to? How does this usually present?

A
  • B cell lymphoma

- Enlargement of the parotid gland late in the disease course

50
Q

Pt with Sjogren’s syndrome + unilateral parotid gland enlargement = ?

A

B cell lymphoma

51
Q

Who is usually affected with systemic sclerosis?

A

Middle aged females

52
Q

What, generally, is systemic sclerosis?

A

Sclerosis of the skin and visceral organs

53
Q

What is the pathophysiology behind systemic sclerosis?

A

Fibroblast activation leads to deposition of collagen

54
Q

What is the theory behind the development of systemic sclerosis?

A

Endothelial damage leads to an immune reaction against collagen, causing release of TGF-beta and PDGF, leading to sclerosis and continued inflammation

55
Q

What are the two major cytokines that activate fibroblasts?

A

TGF-beta

PDGF

56
Q

What are the components of limited sclerosis? (Formerly known as CREST syndrome)?

A
  • Calcinosis
  • Raynaud’s phenomenon
  • Esophageal dysmotility
  • Sclerodactyly
  • Telangiectasias
57
Q

True or false: there is early visceral involvement with CREST syndrome?

A

False–late involvement

58
Q

What is the antibody that is found in CREST syndrome?

A

Anti-centromere antibody

59
Q

What are the associated symptoms from esophageal involvement of CREST?

A

GERD and decreased peristalsis

60
Q

Is visceral involvement with diffuse systemic sclerosis early or late in onset?

A

Early

61
Q

What are the GI sequelae of diffuse systemic sclerosis?

A

GERD
Esophageal dysmotility
Malabsorption

62
Q

What are the pulmonary sequelae of diffuse systemic sclerosis?

A

Interstitial fibrosis and pHTN

63
Q

What are the renal sequelae of diffuse systemic sclerosis? What is the treatment for this?

A

Scleroderma renal crises–acute onset of renal failure and HTN

ACEIs

64
Q

What are the cardiac sequelae of diffuse systemic sclerosis?

A

HTN

Pericarditis

65
Q

What is the antibody that if found with systemic sclerosis?

A

Anti-DNA topoisomerase I abs

66
Q

What are the features of mixed CT disease?

A

Mixed features of SLE, systemic sclerosis, and polymyositis

67
Q

What are the antibodies found in MCTD?

A
  • ANA

- Anti-U1-RNP