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Musculoskeletal Exam 2 > Pathology - Autoimmune Diseases > Flashcards

Pathology - Autoimmune Diseases Flashcards

1
Q

What is autoimmune disease a prototype of?

A

Hypersensitivity

  • Type II (cytotoxic) - organ specific
  • Type III (immune complex) - SLE, RA
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2
Q

What is Systemic Lupus Erythematosus (SLE) and what does it involve?

A

Multisystem inflammatory disease which characteristically involves:

1) skin
2) Joints
3) Kidney
4) Other organ systems

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3
Q

In SLE what is the most important diagnostic Ab?

A
  • Ab against nuclear proteins (ANA; is generic)
  • 2x (double stranded)
  • Sm (anti Smith) - and Antihistone Ab
    - PRESENCE OF BOTH ARE Dx OF SLE
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4
Q

What is very characteristic of SLE in an ANA test?

A
  • “rim” pattern.
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5
Q

What is most damage caused by in SLE?

What sex is SLE more prevalent in?

A

Vasculitis; immune complexes deposit in essentially all tissues, –> all organs can be involved. Type III hypersensitivity.
- women 9:1, 2x-3x higher in blacks and hispanics, most cases occur in 20s and 30s

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6
Q

What are the clinical manifestations on the skin?

What is the defining histological characteristic of dermal and epidermal border of skin anywhere?

A
  • Erythematous rash in sun - exposed sites ( malar, mid facial “butterfly” rash being most characteristic. Lupus purpura also diffuse (raised rash)
  • will see immunoglobulin/ complement deposits AKA “lupus band”
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7
Q

What are the characteristic of joint disease due to lupus?

A
  • > 90% SLE patients have polyarthralgia
    • -> inflammatory synovitis w/o joint destruction ( as in RA)
      - ->Usually first sign
      - ->bilateral pain at same time
      - ->joints look inflammed and warm
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8
Q

What are is characteristic of Renal disease in Lupus?

What are the 4 main types of renal disease in lupus?

A
  • 75% have glomerularnephritis, IgG mainly involved
  • 4 main types of Glomerularnephritis:
    1) Mesangial lupus GN - Mildest form
    • immune complexes and complement found in mesangium
      2) Focal proliferative lupus nephritis
      3) Diffuse proliferative lupus nephritis***- MOST SEVERE of renal disease. Epithelial crescents present (hallmark of serious renal disease). May patients progress–>renal failure
      4) Membranous lupus nephritis
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9
Q

What are the respiratory symptoms of SLE?

A
  • Pleural effusions
  • Upper airway involvement
  • Pneumonitis
  • Progressive interstitial fibrosis
  • Pulmonary hypertension
  • According to the Mayo Clinic, difficulty with breathing, wheezing and chest pain during physical activity are respiratory symptoms of lupus.**
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10
Q

What are the CNS implications in SLE?

A
  • Life threatening complication
    • ->vasculitis leading to hemorrhage infarction
  • Memory loss, anxiety, and depression are neurological symptoms of lupus that may become sever if not treated
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11
Q

What are the Cardiac disease implications in SLE?

A
  • Pericarditis most common finding***
  • Libman-Sacks endocarditis***
    • ->sterile vegetative growths on valve leaflets (not clinically significant- cause they’re not large and rarely vegetate)
  • 33% of patients have and increase in concentrations of antiphospholipid Ab which leads to thromboembolisms
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12
Q

What drugs could cause Drug-induced Lupus?

What is are 3 differences betwen SLE and drug-induced lupus?

A
  • Procainamide (arrhythmias) temporary
  • Hydralazine ( hypertension)
  • Isoniazid (TB)
  • 1) no sex predominance
    2) Unlike SLE, no CNS nor RENAL involvement
    3) Ab to histones ( giving (+) ANA test)
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13
Q

What are differences between SLE and Chronic Discoid Lupus (DLE)?

A
  • DLE which affect eh skin ( but only in sun exposed region) and no organ system involvement.
  • The most common symptom in DLE is a circular rash.
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14
Q

What is the main distinguishing factor of Subacute Cutaneous Lupus?

A
  • Papular and anular rings on trunk which are aggravated by sunlight.
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15
Q

What are the main clinical manifestations of Sjogrens syndrome?

A
  • Keratoconjunctivitis (dry eyes)
  • xerostomia ( dry mouth)
  • Both the above are associated with primary SS.
  • Sicca (keratoconjunctivitis and xerostomia)
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16
Q

What sex/age group is Sjogrens most common in?

A
  • Women 30-65 yrs
17
Q

What other glandular issues might be relevent in Sjogrens?

A
  • immune cells attack and destroy the exocrine glands that produce tears and saliva -therefore bilateral parotid gland enlargement might occur.
18
Q

What is the relevance of Skin lymphoma with Sjogrens?

A

40x increase risk of skin lymphoma***, chronic thyroiditis, interstitial nephritis.

19
Q

What is Scleroderma (Progressive Systemic Sclerosis)?

A
  • PSS characterized by widespread collagen deposition (type III- granulation tissue)
  • Could be activated via TGF-Beta
20
Q

What organs are involved in Scleroderma?

A
  • Skin
  • internal organs
  • lungs
  • GI tract
  • heart
  • Kidney - vasculature becomes very narrow and decreases blood flow and therefore increases renin release leading to hypertension.
21
Q

What is the sex/age prevalence of Scleroderma?
What Ab are specific for Scleroderma?
What factor is commonly found?
What do the antibodies target?

A
  • 4:1 women vs. men; 25-50 yrs
  • nucleolar Ab to RNA polymerase. Ab to Scl-70 (most common)***
  • RA factor
  • collagen I, IV ( renal basement membrane)
22
Q

What pattern of immunofloresece is suggestive of PSS?

A
  • nucleolar pattern of staining in which the bright fluorescence is seen within the nucleoli of the Hep2 cells.
23
Q

What are some symptoms of Scleroderma?

A
  • Skin edema and collagen deposits
  • arteries, arterioles and capillary destruction (increased vascular permeability) ***
  • fibrosis with thickening –>Telangiectasia ***
24
Q

How does Scleroderma affect the kidneys?

A

Kidneys involved ini more than 50%

-fibrinoid necrosis common in afferent arterioles (ischemia with increased plasma renin–>hypertension)

25
Q

What 2 variants do patients with PSS present with?

A
  1. generalized progressive systemic

2. diffuse cutaneous (“Crest”)

26
Q

What are the main features of generalized progressive systemic sclerosis?

A
  • Severe and progressive disease of the skin and organs. Early symptoms present with Raynaud’s (intermittent episodes of ischemia in the fingers)
  • Next follows edema of fingers and hands, and thickening and “tightening” of skin
    • -> patients present with “stone” face with restricted motion of the mouth
  • then organ involvement
27
Q

What are the main symptoms of Raynaud’s?

A
  • When the fingers become cold-sensitive –>due to vasoconstriction
  • Patients fingers go from white (lack of blood flow) –>blue as vessels dilate to keep blood in tissue–>red as blood finally returns
28
Q

What are the outward symptoms of Scleroderma?

A
  • Stone Face - drawn pursed lips, shiny skin over cheeks and forehead, atrophy of muscles in the temple, face, and neck. AKA “Mauskopf”
  • hypo- and hyperpigmentation and alopecia are also symptoms
29
Q

What are the main features of diffuse cutaneous form of Scleroderma?

A

CREST

  • Calcinosis (calcium deposits on skin)
  • Raynaud phenomenon
  • Esophageal dysmotility (acid reflux and decrease in motility of esophagus)
  • Sclerodactyly (thickening and tightening of skin on the fingers and hands)
  • Telangiectasia (dilation of capillaries causing red marks on surface of skin)

*** Only 2 of the five symptoms of CREST syndrome is necessary to be diagnosed with the disease

30
Q

What are the main symptoms of Polymyositis/Dermatomyositis?

A
  • Chronic autoimmune diseases of muscle, most common form is juvenile dermatomyositis, 3:1 prevalence in blacks vs. whites, 2:1 female
31
Q

What diseases might Polymyositis/Dermatomyositis be mixed with

A

SLE, PSS, mixed connective tissue disease, and Sjogren syndrome

32
Q

What are the clinical features of Polymyositis?

A
  • Usually affects adults 40-50’s ***
  • No cutaneous involvement ***
  • Chronic inflammatory muscle disease
  • Symmetric proximal muscle involved
33
Q

What are the symptoms of long-standing, uncontrolled polymyositis?

A
  • Muscle atrophy, decreased ROM,
34
Q

What are the main features of dermatomyositis?

A
  • Inflammatory involvement of skin and muscles.
  • Bilateral symmetric proximal muscle weakness first to occur
  • difficulty standing or climbing stairs
  • Fine distal muscle weakness occurs latter
35
Q

What is the skin issue that occur with dermatomyositis?

A
  • Lilac rash (heliotrope)** on the upper eyelid with periorbital edema
  • Grotton lesions** -erythematous patches over knuckles
36
Q

What percentage go on to develop cancer?

What is involved in the juvenile form?

A

20-25%

- GI tract involvement (pain)

37
Q

For mixed connective tissue disease what are the combined features?

A

-SLE, Sleroderma (PSS), polymyositis/dermatomyositis

38
Q

What are the clinical features of mixed connective tissue disease?

A
  • Ab direct to ribonucleoprotein particle, containing U1 ribonucleoprotein***
  • Not directed at double stranded DNA***
  • Nuclear proteins include Jo-1 and PM-1***
  • respond well to glucocorticoid Tx
  • Etiology and pathogenesis unknown
    NO RIM PATTERN
39
Q

What type of immunofloresent pattern is seen in mixed connective tissue disease?

A
  • “speckled” pattern of staining which is more characteristic of the presence of autoantibodies to extractable nuclear antigens, particularly ribonucleoprotein . Not specific

Musculoskeletal Exam 2 (8 decks)

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