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Immunopathy III Flashcards

1
Q

SLE incidence

A
  • predominantly affects female (9F:1M ratio
  • 1/700 females affected
  • 1/245 and more severely in black females
  • occurs from menarche-menopause (typically in 2nd or 3rd decade
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2
Q

SLE multifactorial etiology

A
  • Genetic = HLA-DQ locus
  • Environment = UV radiation, viruses, drugs, hormones
  • Immune response:
  • -> Self-reactive helper T cells escape tolerance and drive autoantibody production by B cells; defects in elimination of self-reactive B cells peripherally and in bone marrow
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3
Q

lupus autoantibodies

A
  • Hallmark of disease = autoantibodies called antinuclear antibodies (ANAs), reflect loss of tolerance, act as mediators of cell/tissue injury
  • ANAs are directed against 4 categories of nuclear Ag: DNA, histones, proteins bound to RNA, nucleolar Ag
  • immunofluorescent staining patterns suggest underlying disease and level of disease activity
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4
Q

SLE Effects

A
  • any organ may be involved
  • Type III mechanism observed in visceral lesion (DNA - anti-DNA immune complexes), especially in kidneys
  • Type II mechanism occurs against blood cells in which Abs opsonize red and white cells and platelets promoting phagocytosis and lysis
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5
Q

SLE effect on Joints

A
  • polyarthralgia of hands, knees, ankles is a common manifestation in 90% of cases
  • -> arthritis is a clniical criterion
  • Mononuclear inflammatory synovitis occurs but without joint destruction
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6
Q

SLE effects on skin

A
  • Erythematous rash may be found on the face, trunk, or extremities and is often exacerbated by exposure to ultraviolet light (photosensitivity); the malar or butterfly rash across the cheeks and nasal bridge is a clinical criterion
  • skin may also show vesicles, macules, papules and ulcers
  • Histologically, basal layer degeneration occurs due to deposition of immunoglobulin (IgG) and complement at the dermal-epidermal junction
  • -> perivascular lymphocytic infiltrates and immune complex vasculitis
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7
Q

SLE effects on Blood vessels

A
  • Circulating immune complex (ANAs-nuclear proteins) deposit in vascular beds, activate complement –> acute, damaging inflammatory reaction (Type III)
  • Acute vasculitis with fibrinoid necrosis of arteries/arterioles may occur in any tissue
  • Chronic lupus shows fibrous thickening of vessels in a layered, onion-skin fashion, often seen in the spleen
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8
Q

SLE effects on Kidneys

A
  • Major cause of morbidity and mortality, affecting 50-70% of patients
  • lupus nephritis is the prototype of immune complex glomerulonephritis (GN)
  • mechanism of injury is immune complex deposition in glomeruli, basement membranes, larger blood vessels; also thombosis due to antiphospholipid Ab
  • tubulointerstitial changes also occur with immune complex deposits in tubular basement membranes
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9
Q

Lupus Nephritis

A
  • prototype of immune complex glomerulonephritis (GN)
  • glomular changes:
  • -> includes frank necrosis, are prominent due to immune complex induced inflammation with proliferation of endothelila, epithelial and mesangial cells
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10
Q

what are the 5 patterns of GN

A
  • normal = no discernible morphologic abnormality
  • mesangial GN
  • focal proliferative GN
  • duffiuse proliferative GN
  • membranous GN
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11
Q

SLE effects on serosal membranes

A
  • 50% of cases demonstrate inflammation of the serous membranes or serositis, often with effusion, principally involving the pleurae and pericardium
  • in the acute form, exudation of fibrin occurs
  • chronically, proliferation of fibrous tissue –> thickened membranes, adhesion of lung lobes and of visceral and parietal pleurae
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12
Q

SLE effects of Heart

A
  • Fibrinous pericarditis with effusion in 25-50% of cases
  • myocarditis may reflect severity of disease process in other organs and is characterized by interstitial lymphocytic infiltrates and fibrinoid necrosis of small vessels
  • Nonbacterial endocarditis, called libman-Sacks endocarditis = exhibits warty lesions occuring onany valve and on either surface of the affected valve
  • accelerated coronary artery disease due to steroid therapy or immune damage.
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13
Q

infective endocarditis (IE)

A
  • large, irregular DESTRUCTION masses on valve cusps can extend to chordae
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14
Q

Rheumatic heart disease (RHD)

A
  • small, warty vegetations along lines of closure of valve leaflets
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15
Q

nonbacterial thrombotic endocarditis (NBTE)

A
  • small, blad vegetations at the line of closure
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16
Q

libman-sacks endocarditis (LSE)

A
  • small-medium sized vegetations on either/both sides of valve leaflets
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17
Q

SLE effects on lungs

A
  • pleuritis with effusion occurs in 50% of cases
  • pneumonitis from deposition of immune complexes in alveolar septa may occur –> alveolar damage, edema and hemorrhage
  • chronic, progressive lupus may cause interstitial fibrosis
  • pulmonary vascular involvement with fibrosis may cause pulmonary hypertension
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18
Q

SLE effects on CNS

A
  • involvement is common and neurologic disease is a clinical criterion
  • varying manifestations: focal neurologic deficit, seizures, neuropsychiatric symptoms (psychosis)
  • histopathology mild compared to symptom
  • small vessel thickening by intimal proliferation leads to ischemia, microinfarcts
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19
Q

describe the clinical course of SLE

A
  • variable: ranges from mild dermatologic or joint symptoms to life threatening organ failure and cytopenias
  • depends on organ involved and extent of damage
  • 90% live 5 years
  • 80% live 10 year
  • COD: renal failure, infection (immunosuppressive therapy), CNS disease, Coronary artery disease (CAD)
20
Q

describe drug induced form of SLE

A
  • Drug induced LE
  • Lupus-like syndrome with multiple organ involvment, +ANAs, rash, fever, arthralgias, serositis; no renal or CNS pathology
  • Incriminated drugs: D-penicillamine, Procainamide, Hydralazine, Isoniazid
  • Drug cessation –> remission
21
Q

Define Sjogren’s syndrome

A
  • autoimmune destruction of exocrine glands, primarily lacrimal and salivary glands
  • female predominance (90% of cases, typically during middle age, 50s)
  • DRY EYES, DRY MOUTH
  • 40% isolated, primary form called sicca syndrome
22
Q

describe Chronic discoid LE

A
  • marked by variety of skin lesions (plaques, erythema, scaling, atrophy) often involving the face and scalp with SCARRING but without systemic features
  • ANAs are detected but anti-dsDNA is rare
  • systemic disease develops in 5-10% of cases
23
Q

Clinical course of Sjogrens

A
  • Drying of mucous membranes –> xerostomia, keratoconjunctivitis sicca, nasal septal erosion and perforation, dysphagia, dypareunia
  • lymph nodes are often massively hyperplastic
  • 40% increased risk of B cell lymphoma due to emergence of single dominant clone from background of polyclonal B cell proliferation
24
Q

define systemic sclerosis

A
  • autoimmune disorder characterized by chronic inflammation, destruction of small vessels and progressive tissue FIBROSIS
  • 3F to 1 M (50-60 years of age)
25
Q

describe the pathogenesis of Systemic sclerosis

A
  • unknown trigger activates CD4+ T cells to secrete cytokines that promote fibrogenesis (TGF-BETA**), trigger also causes microvascular damage
  • B cells are also activated to produce ANAs: anti-DNA topoisomerase I Ab, anti-centromere Ab
26
Q

describe subacute cutaneous LE

A
  • shows diffuse, superficial, NON-SCARRING, photosensitive lesions with mild systemic disease
  • may be an intermediate phase between purely cutaneous and systemic lupus
27
Q

describe the pathogenesis of Sjogren’s syndrome

A
  • CD4+ T cells against glandular epithelial self-Ag initiate the disorder, possibly induced by viral infections (EBC, Hep C)
  • Systemic B cell hyperactivity –> ANAs (75% of cases also positive for rheumatoid factor)
  • Specific ANAs to ribonucleoproteins SS-A (Ro) and SS-B (La) common in 90%
28
Q

describe the morphology of Sjogren’s

A

T (activated CD4+), B, plasma cells infiltrate ducts and vessels in target glands

  • follicle formation with germinal centers
  • ductal epithelial hyperplasia –> obstruction
  • acinar atropy, fibrosis, fat replacement of parenchyma
29
Q

Clinical course of Sjogrens

A
  • Drying of mucous membranes –> xerostomia, keratoconjunctivitis sicca, nasal septal erosion and perforation, dysphagia, dypareunia
  • lymph nodes are often massively hyperplastic
  • 40% increased risk of B cell lymphoma due to emergence of single dominant clone from background of polyclonal B cell proliferation
30
Q

describe the cardiac changes in systemic sclerosis

A
  • Pericarditis with effusion occurs in 33% of cases
  • perivascular lymphoid infiltrates
  • –> arteriolar thickening
  • –> interstitial fibrosis –> restrictive cardiomyopathy which causes centricular filling to be limited thereby reducing cardiac output
31
Q

describe the pathogenesis of Systemic sclerosis

A
  • unknown trigger activates CD4+ T cells to secrete cytokines that promote fibrogenesis (TGF-BETA**), trigger also causes microvascular damage
  • B cells are also acivated to produce ANAs: anti-DNA topoisomerase I Ab, anti-centromere Ab
32
Q

describe limited variant of systemic sclerosis

A
  • limited to skin involvement of face, forearms, fingers
  • late visceral involvement, relatively benign course
  • anti-centromere Ab in up to 90% of cases
  • Called CREST symptoms
33
Q

describe the symptoms of CREST

A
  • Calcinosis = calcium deposits in the skin
  • Raynaud’s phenomenon = spasm of blood vessels in response to cold or stress
  • Esophageal dysmotility = acid reflux and decrease in motility of esophagus
  • Sclerodactyly = thickening and tightening of the skin on the fingers and hands
  • Telangiectasia = dilation of capillaries causing red marks on surface of skin
34
Q

Describe diffuse variant of systemic sclerosis

A
  • widespread skin involvement at onset
  • early visceral involvement
  • rapid progression
  • Ab to DNA topoisomerase I (anti-Scl-70) occurs in 70% of cases
35
Q

describe the skin morphology of systemic sclerosis

A
  • early findings = edema with lymphocyte (CD4+) infiltrates
  • later findings = epidermal thinning, dermal/appendage fibrosis, subcutaneous calcifications
  • vascular endothelial damage, thickening of the basal lamina
  • fibrosis and ischemia lead to contractures with claw fingers, digital amputation, mask facies
36
Q

describe the gastrointestinal changes in systemic sclerosis

A
  • esophagus = collagenization and fibrosis of muscularis causing esophageal dysmotility, LES dysfunction and ultimately resulting in REFLUX
  • Small bowel = mucosal thining, loss of villi/microvilli, submucosal fibrosis resulting in MALABSORPTION
37
Q

descrie the musculoskeletal changes in systemic sclerosis

A
  • early stage = nondestructive hyperplasia and inflammation of synovium
  • Late stage = fibrosis of synovial and periarticular connective tissue
  • 10% of cases myositis with lympcytic infiltrates and fiber atrophy
38
Q

describe the renal changes in systemic sclerosis

A
  • occurs in 2/3 of cases
  • thickening of interlobular arteries by concentric proliferation of intimal cells, deposition of collagenous or mucinous material and hyaline change
  • interlobular artery exhibits marked luminal narrowing due to pronounced intimal thickening
  • May result in hypertension in 30% of cases
39
Q

describe pulmonary changes in systemic sclerosis

A
  • Occur in 50% of cases
  • mild interstitial pneumonitis
  • alveolar fibrosis –> respiratory insufficiency
  • pulmonary hypertension from endothelial dysfunction –> cor pulmonale
40
Q

describe the cardiac changes in systemic sclerosis

A
  • Pericarditis with effusion occurs in 33% of cases
  • perivascular lymphoid infiltrates
  • arteriolar thickening
  • interstitial fibrosis –> restrictive cardiomyopathy which causes centricular filling to be limited thereby reducing cardiac output
41
Q

describe the clinical course of systemic sclerosis

A
  • Usually progressive
  • 10 year survival rate varies from 35-70% depending on organs involved
  • COD = renal, cardiac, pulmonary, GI dysfunction or failure
42
Q

Anti-double stranded DNA

A

SLE

- Peripheral rim stain

43
Q

Anti-histone

A

drug induced LE

- Diffuse homogeneous stain

44
Q

Scl-70

A

Systemic sclerosis - Diffuse

45
Q

Scl-70 and Anti-centromere

A
  • Scleroderma (CREST)

- Centromere pattern

46
Q

Sjogren syndrome

A

Ro, La

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