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Pathology > Immune diseases > Flashcards

Immune diseases Flashcards

1
Q

SLE

A

Type III hypersensitivity (immune complex)

  • antigen: DNA, nucleoproteins
  • clinical manifestation: nephritis, arthritis, vasculitis
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2
Q

Post-streptococcal glomerulonephritis

A

Type III hypersensitivity (immune complex)

  • antigen: strep cell wall antigen released into circulation after strep infection, bound by antibody to form immune complex that is deposited into small blood vessels of kidney
  • clinical manifestation: nephritis
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3
Q

Systemic immune complex disease

A

Type III hypersensitivity (immune complex)
aka acute serum sickness
seen in the past following vaccination with horse serum, systemic immune complex disease caused by antigen excess
clinical manifestation: arthritis, rash, fevers

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4
Q

Arthus reaction

A

Type III hypersensitivity (immune complex)
local severe acute immune complex mediated vasculitis associated with necrosis
circulating antibodies present in a patient react with injected antigen (immunization)
edema, hemorrhage, frank ulceration of skin at injection site

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5
Q

Polyarteritis nodosa

A

Type III hypersensitivity (immune complex)
antigen: hepatitis surface antigen
clinical manifestation: vasculitis

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6
Q

Reactive arthritis

A

Type III hypersensitivity (immune complex)
antigen: yersinia bacterial antigen
clinical manifestation: acute arthritis

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7
Q

TB skin reaction

A 
Delayed type (type IV) hypersensitivity
- if prior sensitization to TB, red induration at injection site with hyperemia from local vascular dilation
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8
Q

Contact dermatitis

A

Delayed type (type IV) hypersensitivity

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9
Q

Type I diabetes mellitus

A

Delayed type (type IV) hypersensitivity against pancreatic B cells

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10
Q

Multiple sclerosis

A

Delayed type (type IV) hypersensitivity against myelin basic protein

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11
Q

IPEX

A

immune dysregulation, polyendocrinopathy, enteropathy, X linked
caused by mutation in Foxp3, the transcription factor needed for production of regulatory T cells

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12
Q

Autoimmune Lymphoproliferative Syndrome

A

Lupus-like disorder caused by Fas mutation; failure of clonal deletion by activation induced cell death via death receptor pathway so autoreactive cells escape deleation

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13
Q

Autoimmune polyendocrinopathy

A

AIRE mutation

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14
Q

Rheumatic heart disease

A

Autoimmune disease brought on by molecular mimicry following Group A Streptococcal infection: Streptococcal antigens mimick self antigens in the heart, ntibodies “cross-react” and damage heart

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15
Q

Systemic Lupus Erythematosis

A
  • multi-system autoimmune disease
  • antibodies are anti-nuclear: anti-DNA, anti-histone, anti-non-histone protein bound to RNA, anti-nucleolar
  • affects joints, kidneys, serosa
  • must have 4 of 11 major criteria present to dx (malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder, neurologic disorder, hematologic disorder, immunologic disorder, antinuclear antibodies)
  • clinical manifestations: common= hematologic disorder, arthritis, malar rash, fever, fatigue, weight loss, renal disease, CNS issues, pleuritis, pericarditis, myalgia, Raynaud
  • pathophysiology: deposition of immune complexes in kidneys, joints, blood vessels- Type III hypersensitivity and hematologic manifestations due to opsonization of blood elements- type II hypersensitivity
  • granular pattern of antibodies in glomeruli
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16
Q

Antiphospholipid antibody syndrome

A

diagnosis in individuals with significant antiphospholipid antibodies, thrombotic complications, but without SLE

17
Q

Goodpasture Syndrome

A
  • The anti-glomerular basement membrane (GBM) antibodies attack the kidneys and lungs, although, generalized symptoms like malaise, weight loss, fatigue, fever and chills are also common, as are joint aches and pains
  • Linear pattern of immunoflourescence in glomeruli- “wire loop lesions”
  • Type II (antibody mediated) hypersensitivity
18
Q

Drug induced lupus erythematosus

A

Lupus like syndrome induced by drugs (hydralazine, procainamide)
Anti-histone antibodies (no dsDNA Ab)
May have no symptoms, just positive Ab titer
Arthralgia, fever, serositis (no renal or CNS involvement)

19
Q

Sjogren Syndrome

A
  • autoimmune destruction of lacrimal and salivary glands leading to keratoconjunctivitis sicca and xerostomia
  • histology: T cell infiltrate, fibrosis and destruction of glandular parenchyma, loss of secretions; biopsy of lip is required to confirm diagnosis
  • pathology: antibodies to RNP antigens SS-A Ro and SS-B La
  • more common in older women, increased risk of lymphoma
20
Q

Mukulicz syndrome

A

Refers to lacrimal and salivary gland enlargement due to any cause (with or wihtout Sjogren syndrome)

21
Q

Systemic slcoerosis

A
  • can be diffuse with widespread skin involvement and early visceral involvement, or limited with limited skin involvement/ late visceral involvement/ possible CREST
  • associated with antibody anti Scl-70 against DNA topoisomerase I
  • fibrosis due to increased secretion of TGF-B with chronic inflammation
    GI- dysphagia, GERD, MAS
    Kidney- HTN, possibly malignant
    Lungs- pulmonary HTN, interstitial fibrosis
22
Q

CREST

A
  • form of limited scleroderma
  • calcinosis, Raynaud, esophageal dysmotility, sclerodactyly, telangectasias
  • associated with anticentromere antibodies
23
Q

Churg- Strauss syndrome

A

Autoimmune condition that causes inflammation of small and medium-sized blood vessels (vasculitis) in persons with a history of airway allergic hypersensitivity (atopy)

Associated with pANCA

24
Q

Microscopic polyangiitis

A

Autoimmune disease characterized by a systemic, pauci-immune, necrotizing, small-vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation

Associated with pANCA

25
Q

Wegener’s granulomatosis

A

Systemic disorder that involves both granulomatosis and polyangiitis. It is a form of vasculitis (inflammation of blood vessels) that affects small- and medium-size vessels in many organs. Damage to the lungs and kidneys can be fatal.

Associated with cANCA

26
Q

Kawasaki disease

A

Mucocutaneous Lymph Node Syndrome

Arteritis often involving coronary arteries
Commonly in children (80% under 4 years old)
Cardiovascular sequelae in 20% (1% acute fatality)
Transmural vascular inflammation and necrosis
Vasculitis→Vessel Ectasia→Aneurysm formation
Vessel rupture, thrombosis, MI, sudden death
Immune reaction→anti-endothelial autoantibodies
Viral infection trigger in genetically susceptible

Fever, conjunctival and oral erythema, edema of hands and feet, erythema of palms and soles, skin rash with desquamation, cervical lymphadenopathy
Treat with high dose aspirin and IVIG

Pathology (7 decks)

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