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BLOCK 2 FINAL > Handout Autoimmune > Flashcards

Handout Autoimmune Flashcards

1
Q

Immediate (Type I) Hypersensitivity

A

Rapid immunological reaction within minutes after the combination of an antigen with antibody bound to mast cell in individuals previously sensitized to the antigen

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2
Q

What is anaphylaxis

A

System form of typic I hypersensitivity characterized by shock, edema, and respiratory compromise (due to upper airway edema)

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3
Q

Type II hypersensitivity

A

antibody-mediated; immunological reaction caused by antibodies against cell surface or extracellular matrix antigens

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4
Q

Type III hypersensitivity

A

immune complex-mediated; damaging inflammatory reaction to antigen-antibody complexes, especially in blood vessels or glomeruli

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5
Q

Type IV hypersensitivity

A

cell-mediated; immunological reaction caused by sensitized CD4+ T-lymphocytes, sometimes producing aggregates of activated macrophages working together (granulomas)

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6
Q

RA antibody

A

Anti-cyclic citrullinated peptide (anti-CCP)

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7
Q

CREST syndrome (limited systemic sclerosis) antibody

A

Anti-centromere

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8
Q

Polymyositis/dermatomyositis antibody

A

Anti-Jo-1 (one type of anti-synthetase)

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9
Q

Systemic sclerosis antibody

A

Diffuse –> Anti-Scl70 (= anti-DNA topoisomerase)

Anti-RNA polymerase (anti-U3 RNP)

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10
Q

Mixed CT disease antibody

A

Anti-U1 RNP

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11
Q

Lupus antibody

A

Anti-Smith (anti-Sm)*
Anti-dsDNA
Anti-nuclear (ANA)

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12
Q

Sjogren Syndrome, neonatal lupus, subcutaneous lupus antibody

A

Anti-SSA (=anti-Ro)

Anti-SSB (=anti-La)

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13
Q

Microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg Strauss) antibody

A

Anti-myeloperoxidase (= perinuclear anti-neutrophil cytoplasmic, P-ANCA)

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14
Q

Granulomatosis with polyangiitis (Wegner’s) antibody

A

Anti-proteinase-3 (= diffuse cytoplasmic anti-neutrophill cytoplasmic, C-ANCA)

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15
Q

Does finding serum antibodies confirm diagnosis of rheumatological diseases?

A

NO! Many people with no apparent rheumatological disease have antibodies associated with rheumatological disease, and should not be falsely diagnosed on the basis of serology alone

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16
Q

SLE Definition

A

prototype multisystem autoimmune disease

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17
Q

SLE Epidemiology

A

fairly common, up to 13X more common in women, more common in blacks, affects 1 in 245 black women, most common in child-bearing age (15-45), more severe in blacks and Asians

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18
Q

SLE Pathogenesis

A

failure of self-tolerance, antinuclear antibodies, genetic factors, environmental factors (medications, UV light, sex hormones), immunologic factors (activation of helper T cells and B cells, IgG autoantibody production, immune complexes)

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19
Q

SLE Gross Pathology

A

non-erosive synovitis in 90%, pleuritis in 46%, pericarditis in 25%, peritonitis (commonly polyserositis), Libman-Sacks endocarditis (1-3mm verrucous vegetations on either side of valve), moderate splenomegaly

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20
Q

SLE Microscopic Pathology

A

acute necrotizing vasculitis of small arteries in arterioles (with fibrinoid deposits), nephritis in 50%, cerebritis in 50%

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21
Q

What are the 5 patterns of glomerulonephritis in SLE

A 
Minimal
Mesangial
Focal proliferative
Diffuse proliferative
Membranous
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22
Q

What is the characteristic histology of glomerulonephritis in SLE

A

glomerular granular deposits of IgG and complement, subendothelial dense deposits causing wire loop lesions, most common in diffuse proliferative and indicative of active disease

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23
Q

SLE Symptoms

A

Commonly relapsing and remitting
Joint pain (without deformity) in 90%
Fever (sometimes all alone “fever of unknown origin”) in 83%
Fatigue in 81%
Weight loss in 63%
Pleuritic chest pain, photosensitivity, nephrotic syndrome (edema), angina, alopecia (hair loss), myalgias

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24
Q

SLE Signs

A 
Erythematous skin rash over bridge of nose and cheeks (malar distribution, "butterfly rash") present in 50% and in other site sin 35% more
Edema (first in feet)
Hematuria (usually only microscopic)
Neuropsychiatric (psychosis, seizures)
Oral ulcers 
Interarticular skin rash on fingers
Peri-ungual erythema around fingernails
Alopecia
25
Q

SLE Diagnosis

A

ANA (present in 100%, but also up to 15% of normals)
Anti-dsDNA or anti-Sm antibodies (much more specific)
Hematologic abnormalities (anemia, thrombocytopenia, etc., present in 100%), proteinuria, urinary red cell casts, kidney biopsy

26
Q

SLE Treatment

A

corticosteroids and immunosuppressive medications

27
Q

SLE Prognosis

A

90% of 5-year survival, 80% 10-year survival

28
Q

Sjogren Syndrome Definition

A

autoimmune chronic inflammatory disease of lacrimal glands and salivary glands, causing dry eyes, and dry mouth

Eye involvement (kerato-conjunctivitis sicca)
Oral involvement (xerostomia)
29
Q

What is the primary form of Sjogren Syndrome

A

Sicca Syndrome

Limited to eyes and mouth

30
Q

What is the secondary form of Sjogren Syndrome

A

associated with other autoimmune diseases: rheumatoid arthritis (most common), lupus, polymyositis, systemic sclerosis, vasculitis or thyroditis

31
Q

Can you have extraglandular involvement with Sjogren Syndrome

A

YES! Extraglandular involvement of kidneys, joints, skin, muscle, peripheral nerves and even brain

32
Q

Sjogren Syndrome Epidemiology

A

Uncommon
90% in 35-45 year old women
Primary form a little less common (40%) than secondary (60%)

33
Q

Sjogren Syndrome Pathogenesis

A

Thought to be T lymphocyte-mediated immunological attack of some self antigen in ductal epithelial cells of these glands

34
Q

Sjogren Syndrome Gross Pathology

A

dry ocular and oral mucosa, enlarged salivary and lacrimal glands

35
Q

Sjogren Syndrome Microscopic Pathology

A

1) intense infiltration of CD4 T lymphocytes
2) destruction of gland architecture, +/- plasma cells, +/- germinal centers.
Renal involvement: interstitial nephritis rather than glomerulonephritis

36
Q

Sjogren Syndrome Symptoms

A

dry eyes, dry mouth

37
Q

Sjogren Syndrome Signs

A

dry mucus membranes of eyes and mouth, conjuctival ulcers, oral ulcers, enlarged salivary and lacrimal glands

38
Q

Sjogren Syndrome Diagnosis

A

Anti-SSA (=anti-Ro) and/or Anti-SSB (=anti-La) antibodies (not entirely specific).

39
Q

Sjogren Syndrome Treatment

A

Topical therapy of dry eyes, dry mouth and other dry mucosal surfaces, systemic cholinergic agents to stimulate secretions.
Hydroxychloroquine, sometimes rituximab for extraglandular disease

40
Q

Sjogren Syndrome Prognosis

A

usually not bad, but ESPECIALLY NOTORIOUS FOR DEVELOPING LYMPHOMA

41
Q

Systemic Sclerosis Definition

A

Chronic disease characterized by abnormal accumulation of fibrous tissue in skin and other organs.
Diffuse = widespread skin and early visceral involvement
Limited = skin of fingers/forearms/face and late visceral invovlement (CREST Syndrome)

42
Q

Systemic Sclerosis Epidemiology

A

Female predominance (F:M = 3:1)
Highest incidence in 50-60 year olds
Most severe in AA, especially women

43
Q

Systemic Sclerosis Pathogenesis

A

abnormal immune response + vascular damage = increased growth factors leading to fibrosis
CD4 T-cells responding to unidentified antigen release cytokines stimulate fibroblasts to produce collagen
Microvascular disease: intimal proliferation, capillary dilation, endothelial injury (increased vWF) + platelet activation leads to fibrosis. Genetics poorly understood, but HLA-II and fibrillin-I genes implicated

44
Q

Systemic Sclerosis Gross Pathology

A

Skin involved in 100% - affected areas initially edematous, ultimately fibrotic. Begins in face and fingers, progresses proximally. Decreased mobility leads to masklike face and clawlike hands. GI tract (involved in 90%): fibrous replacement of muscular wall, most commonly in esophagus. Lower esophageal sphincter dysfunction and decreased peristalsis causes GERD and Barrett metaplasia

45
Q

Systemic Sclerosis Microscopic Pathology

A

Skin: dense collagen deposition in dermis with decreased appendages
Thinning of epidermis, loss of rete pegs
Perivascular infiltrates of CD4 T-cells
Thickening of capillary/arterial basal lamina.
Kidneys (involved in about 67%): vascular changes promote HTN and renal failure
Intimal thickening of interlobular arteries leading to ischemia and infarction.
Lungs (involved in over 50%): vascular changes promote pulmonary HTN and interstitial fibrosis

46
Q

Systemic Sclerosis Symptoms

A

Raynaud’s phenomenon
Numbness/tingling/cyanosis of peripheral skin
Joint pain and/or stiffness
Digestive problems secondary to decreased gut motility

47
Q

Systemic Sclerosis Signs

A

Early: edema of the hands and feet, more prominent in morning
Later: thickened, hard and/or shiny skin, especially prominent over long bones of arms and lower legs
Mask-like immobile face and claw-like immobile hands

48
Q

Systemic Sclerosis Diagnosis

A

SUGGESTED by generalized cutaneous sclerosis, HTN, renal failure, pulmonary HTN and fibrosis
DRG: Anti-DNA topoisomerase I (anti-Scl70) in 28-70% of diffuse systemic sclerosis patients. Anticentromere Ab in 22-36% of limited systemic sclerosis patients

49
Q

Systemic Sclerosis Prognosis

A

death most commonly d/t extracutaneous organ involvement: pulmonary HTN, renal crisis or aspiration PN

50
Q

What is the earliest manifestation of systemic sclerosis?

A

puffy fingers

51
Q

What is C4d

A

degredation product of activated complement C4 and is deposited in peri-tubular capillaries in some cases of kidney transplant rejection, but it is also deposited in some cases with no other evidence of rejection, so positive C4d staining has to be combined with histological evidence of rejection and the finding of donor-specific antibodies in the recipients blood before antibody-mediated rejection is diagnosed

52
Q

What must kidney transplant biopsies be evaluated for?

A

1) Evidence of BK virus infection, also called polyomavirus nephropathy. This is manifested by intranuclear inclusion bodies and epithelial cell injury and lysis
2) Post-transplant lymphoproliferative disorder (PTLD): spectrum ranging from proliferation of lymphocytes that halts with decreasing immunosuppressive therapy to a full blown malignant lymphoma - proliferating lymphocytes are infected with EBV

53
Q

What type of cell most commonly leaves a transplanted organ and resides elsewhere in the recipient?

A

LYMPHOCYTE - recipient lymphocytes commonly recognize the cells around them as foreign and attack them

54
Q

GVHD defiition

A

attack and injury of host organs (especially skin, liver, or GI tract) by lymphocytes from the donor of a transplant

55
Q

What does GVHD cause?

A

SKIN: generalized erythematous rash, which goes on to cause fibrosis similar to systemic sclerosis.
LIVER: biliary and hepatocyte injury, manifested clinically by jaundice and elevated liver transaminases.
GI TRACT: injury of the epithelium, manifested clinically by bloody diarrhea.

56
Q

In GVHD, what happens to the attacked cells in the affected organs?

A

Die by apoptosis

57
Q

What are the two holy grails of transplantation?

A

Immunologic Tolerance

Growing Replacement Organs form Stem Cells

58
Q

What is immunologic tolerance?

A

state of being incapable of mounting an immune response to a specific antigen - immunologic tolerance to the specific antigens which vary between the donor and recipient organs of the type transplanted has been the goal of transplant immunologists

BLOCK 2 FINAL (10 decks)

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