CH 10--IMMUNOPATHOLOGY--3 Q'S Flashcards by ROGER ALONSO

Immunity

________(non-specific) immunity:
Inherited & do not depend on previous exposure
(a)First line of defense: mechanical (skin), cilia in
trachea and bronchi, nasal and gastric secretions
(b) Second line of defense: lysozymes, complement and phagocytic cells.
________(specific) immunity: Specific response following exposure to a particular antigen.

Natural

Acquired

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Essential features of immune system X4
1 -___________: for one particular antigen
2 -__________: can recognize different antigens
3 -__________: to previous antigens, responsiblefor rapid, higher and persistent secondary immune response
4 -__________: by releasing cytokines that recruit & activate other defense mechanisms

Specificity

Diversity

Memory

Recruitment

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Hypersensitivity VIA Allergy
-Abnormal __________immune reactions resulting in tissue injury

Four types on the basis of mechanism of injury:
Type I:immediate (anaphylactic)
Type II:cytotoxic
Type III:immune complex
Type IV:cell--mediated hypersensitivity

exaggerated

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Hypersensitivity–1

Type I: Immediate (Anaphylactic) Reaction
1st exposure to allergen stimulates IgE = binds to mast cell and blood basophils. Activation of mast cell & the potent inflammatory mediators
released.

2 Clinical Types:
(1) _________
a -Local form (affecting one organ) e.g. urticaria (hives), allergic rhinitis, bronchial asthma
b -Response to ingested or inhaled environmental allergens
c - Affects 10% of population
d -Strong familial predisposition

(2) __________
- Systemic from release of vasoactive amines into circulation e.g. peripheral circulatory failure, shock, hypotension, even death
- Follows injection of allergens e.g. serum, drugs (penicillin)

Atopy

Anaphylaxis

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Hypersensitivity–2

Type II: ________ Reaction & Antibody
-mediated Reaction Mechanism:
Specific antibody (IgG or IgM) reacts with cell membrane antigen, activates the complement resulting= CELL LYSIS
Clinical types
1. Transfusion reaction due to blood incompatibility
2. Rh incompatibility
3. Autoimmune hemolytic anemia
4. Myasthenia gravis=Auto antibodies against ________ receptors at neuromuscular junction
= profound muscle weakness
5. Graves’ disease: The binding of the antibody to the TSH receptor in Graves disease results in _________, whereas the inhibition of synaptic transmission in myasthenia gravis leads to profound muscle weakness.
6. Goodpasture’s syndrome-____:hemorrhage,necrosis, fibrosis & _______: glomerulonephritis, fibrosis, failure

Cytotoxic

acetylcholine

hyperthyroidism

Lung…..Kidney

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Hypersensitivity–3

Type III: immune Complex Reaction “Arthus phenomenon”
A. Activation of complement = acute inflammation and tissue damage
(1) Systemic form:-serum sickness-rheumatoid arthritis
(2)Local form:-pneumonitis that develops 6-8 hours after
inhalation of moldy hay (_____ lung) or moldy cheese (____ ________ lung)

Farmer’s

Cheese maker’s

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Hypersensitivity-4

Type IV: __ ________ Hypersensitivity
***Type IV is mediated by T CELLS rather than antibodies
1 -DTH: Delayed-Type Hypersensitivity:-Reaction is mediated
by ___ cells.
2.–2-T cell-mediated cytotoxicity:-Release of IL-2 for activation of ___
3. _________=which are typical of type IV hypersensitivity,
consist of epithelioid cells, giantcells,and lymphocytes.
Clinical types:
(1) contact dermatitis
(2) Killing of tumors and virus infected cells
(3) Transplant rejection
.

Cell Mediated

CD4

CD8

Granulomas

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AUTOIMMUNE DISORDERS

(1) _________ factors:- familial incidence
(2) Immunological factors
(3) Drugs-induced
(4) Viral infection
(5) Sex hormones

Genetic

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Pathological change
(1) _____ _________: (acute necrotizing vasculitis)
-acute vasculitis of small arteries and arterioles due to deposition of immune complexes
- necrosis & fibrinoid deposits within vessel wall
-later, fibrous thickening with luminal narrowing
-perivascular lymphocytic infilterate
(2)_________
-pericarditis
-myocarditis
-endocarditis (LibmanSacks): multiple, small, sterile, flat vegetations, detachable = embolization.
(3) Kidneys: Normal (in few cases):Diffuse proliferative glomerulonephritis (45-50%): Most serious, ALL glomeruli involved. Hematuria,proteinuria, hypertension, & renal insufficiency
EX lupus nephritis.—The mesangial component of the
glomerulus is more prominent because of an increase in both cells and matrix. A a glomerulus with thickened pink capillary
loops, the so-called “wire-loops”, in a patient with lupus nephritis.

BLOOD VESSELS

Heart:

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Pathological changes
(4) Skin: The young woman has a MALAR rash (the so-called
“butterfly”rash because of the shape across the cheeks).
Such a rash suggests______. Sunlight exposure accentuates
this erythematous rash.

(5) Joints:-________(most common presenting symptom)
resembles rheumatoid arthritis but:
-involves large and small joints
-mild (rarely causes destruction of cartilage)

lupus

arthritis

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Pathological changes
(6) C.N.S:-______ = hemorrhages, ischemia, infarcts
(7) Lung:
- pleurisy with pleural effusion
-alveolitis and ________
(8) Serosal cavities:-__________ (pleurisy,pericarditis, peritonitis) with serous effusion or fibrinous exudation
in acute cases to fibrous opacification in chronic cases
(9) Spleen:
-enlarged slightly
-capsule is thickened
-follicular hyperplasia

vasculitis

fibrosis

Inflammation

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Complications of systemic lupus erythematosus

Clinical features:

females
arthritis (larger joints)
fever
skin eruption (butterfly rash over face)
hypertension
hematuria, albuminuria, nephrotic syndrome

KNOW

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Complications of systemic lupus erythematosus

Diagnosis :- antinuclear antibodies (ANAs) in the serum
-presence of LE cells (nuclear debris ingested by blood neutrophils

Prognosis:- chronic course with repeated exacerbations
and remissions
-survival rate is 90% at 10 years (death mostly due to renal failure

KNOW

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Scleroderma/Progressive Systemic Sclerosis

Definition: Autoimmune disease characterized by ______
and excessive deposition of collagen throughout the body,
mainly in the skin ending in fibrosis & atrophy

Incidence:
Sex: ________ (3:1)
Age: 30-50 years at onset

vasculitis

females

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Possible mechanisms leading to systemic sclerosis.
PDGF = platelet-derived growth factor
FGF = fibroblast growth factor

Pathological change

(1) Vasculitis
- perivascular lymphocytic infiltration
- thickened wall
- partial occlusion
(2) Marked fibrosis and atrophy
(3) Restricted to the _____ first, later on viscera

skin

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Clinical features of Scleroderma—1

(1) skin-starts by fingers, then extends proximally to arms, shoulders, neck and face
-initially, skin is edematous with petechial hemorrhages (vasculitis), followed by progressive fibrosis:
fingers:
-tapered
- _________ range of motion
-DECREASE blood supply ulceration and gangrene
Face: - restricted movements

Scleroderma:Typical “__ ______” necroses and ulcerations of
fingertips.

DECREASED

rat bite

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Scleroderma Masklike face (stone facies)

—with stretched, shiny skin and loss of normal facial lines giving a younger appearance than actual age; the hair and
eyebrows are dyed black. Thinning of the lips and perioral
sclerosis result in small mouth, which is _________

asymmetric

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Clinical features of Scleroderma—2

(2) Gastrointestinal tract
- muscle layer: _________, replaced by collagen, deficient peristalsis
- –esophagus: in 50%, dysphagia due to narrowing of lower portion (rubber hose appearance)
- intestines : malabsorbtion

atrophies

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Clinical features of Scleroderma—3

(3) Joints
- _________: proliferation of synovial membrane, later fibrosis & DECREASE movement
- resembles rheumatoid arthritis, but destruction of articular cartilage is rare

synovitis

Scleroderma, Gastrointestinal fibrosis, trichrome stain

This trichrome stain of the stomach demonstrates intense blue staining in the submucosa from the collagen
deposition. Such fibrosis can occur anywhere in the gastrointestinal tract, but is most common in the lower ___________, leading to the esophageal dysmotility with
systemic sclerosis

esophagus

Clinical features of Scleroderma—4

(4) Muscles- focal inflammatory infiltrates & edema, followed by fibrosis & _________
(5) Kidneys–______ glomerular filtration rate and renal failure

(6) Lungs—-diffuse interstitial pneumonitis and fibrosis
- progressive thickening of the walls of small vessels

atrophy

DECREASE

__________ DISEASE

Definition:
Autoimmune disease characterized by immune-mediated destruction of salivary glands and lacrimal glands.

Incidence:

Sex: females (9:1)
Age: 40-60 years
Either primary (40%) or secondary (60%) associated with other autoimmune disease

Pathological lesions;

involve the salivary & lacrimal glands, mucous secreting glands of respiratory tract
marked infiltration with lymphocytes & plasma cells replacing the normal acinar cells
____________ of duct epithlium = lumen obstruction

SJOGREN’S

hyperplasia

SJOGREN’S DISEASE—Clinical findings

(1) _____ _____ (keratoconjunctivitis sicca):
- DECREASE tears
- blurring of vision
- itchy eyes
- corneal ulceration

Dry eye

SJOGREN’S DISEASE—-Clinical findings

(2) ____ ________ (xerostomia):
- DECREASE salivary secretion
- atrophy and ulceration of oral mucosa
- DECREASE taste
- dysphagia

Dry mouth

SJOGREN'S DISEASE-----Clinical findings (3) __________ tract: - dryness and crusting of nasal mucosa - laryngitis, bronchitis, pneumoni 4) Lymphadenopathy (5) Arthritis (60%)

Respiratory

Polymyositis/Dermatomyositis Definition:________ disease that affects primarily the skeletal muscles and skin Incidence: Sex: females (2:1) Age: two peaks, 5-15 and 50-60 year (1) Skeletal muscle - symmetrical, proximal muscle groups (2) Skin - 50% of cases - vasculitis with edema and cellular infiltration - later on,atrophy, fibrosis and calcification

Autoimmune

Dermatomyositis Violaceous erythema of the _______of the hands and fingers, especially over the metacarpophalangeal and interphalangeal joints; the light-protected areas of the forearms are not involved.

dorsum

Clinical features of Dermatomyositis (1) Muscles - __________ weakness, pain and tenderness - stiff fingers - dysphagia due to affection of pharyngeal muscles (2) Skin - erythematous rash on: - malar area of face (butterfly rash) - V area of neck - forehead - shoulders - chest - __________ rash of upper eyelids - atrophy

symmetrical heliotrope

Clinical features of Dermatomyositis (3) _________ involvement (uncommon) constipation and abdominal pain (4) RAYNAUD'S PHENOMENON (30%) (5) INCREASE risk of malignancy (25%)--particularly in adult

Visceral

Amyloidosis Definition:- _______ accumulation of fibrillar proteins = pressure atrophy of adjacent parenchyma

Extracellular

Amyloidosis Clinical forms----I Systemic amyloidosis (i) Primary amyloidosis-----associated with multiple myeloma, the most common form in the US. - --(Bence Jones protein) ``` (ii) Secondary (reactive) amyloidosis associated with (secondary to): a-Chronic inflammatory diseases: b-Autoimmune diseases: c--Neoplasms: --renal cell carcinoma ---HODGKIN'S LYMPHOMA ------In secondary amyloidosis there is systemic distribution of _______ protein, type AA protein(amyloid associated protein) which accumulates in the tissues of *liver *kidneys *spleen *lymph nodes *thyroid ```

amyloid

Amyloidosis Clinical forms (iii) Hemodialysis-related amyloidosis = associated with renal failure affects 70% of patients maintained on hemodialysis - amyloid protein (B2-_________): accumulates mainly in joints & synovium

microglobulin

Amyloidosis Clinical forms (iv)________ amyloidos--- present in certain geographical locations (e.g. Familial Mediterranean Fever: recurrent inflammation of serosal linings and fever

Hereditary

II _________ amyloidosis (i) Senile amyloidosis (amyloid of aging) - occurs in old age(80-90 years) - -heart (senile cardiac amyloidosis) and - -brain (senile cerebral amyloidosis), as in ALZHEIMER'S (ii) Amyloid in neoplasms (endocrine amyloid)

Localized

Morphology of affected organs--- amyloidosis (1) Liver Gross: -normal or enlarged in size -pale, waxy, gray and firm in consistency (2) Kidneys---larger in size -gray in color and firm in consistency -involvement of renal artery =_______ ,atrophy and DECREASE in size (in long-standing cases) Amyloid nephropathy---The mesangial areas are expanded and the glomerular capillaries are obstructed by amorphous acellular material. The deposits of amyloid may take on a __________ appearance.

ischemia nodular

Morphology of affected organs--amyloidosis (3) Heart---normal or enlarged Amyloid deposition, myocardium, microscopic = Amyloidosis is characterized by ____ deposition over years of increasing amounts of an amorphous proteinaceous material in one or more tissues.

slow

Clinical features General -weight loss, weakness, fatigue (non-specific) Local-related to affected_______ (e.g. hepatomegaly,cardiac abnormalities, proteinuria)

organ

IMMUNOLOGIC DEFICIENCY (1) Primary: = GENETIC, present at birth (2) Secondary: = ACQUIRED (e.g. infections, malnutrition, chemotherapy, irradiation)

know

Primary immune deficiencies--1 1. CONGENITAL AGAMMAGLOBINEMIA (BRUTON'S) - --Absence of B lymphocytes - --serum immunoglobulins (all classes) are markedly decreasedor absent

tip----BRUTON HAS NO B

Primary immune deficiencies--2 (2) Congenital thymic hypoplasia (DI'GEORGES sy n d r om e): - ---Developmental defect of the 3rd and 4th pharyngeal pouches - -----absence of the thymus & parathyroid glands - ----Lack of T lymphocytes - ---HYPOCALCEMIA & TETANY

TIP...GEORGE HAS NO T

Primary immune deficiencies--3 (3) Severe combined immunodeficiency (SCID): - ---failure of development of both T and B lymphocytes, marked lymphopenia

SCID HAS NO T OR B

Primary immune deficiencies--4 (4) Dysgammaglobulinemia: - -----Most common primary immunodeficiency - --Failure of differentiation of B lymphocytes into IgA-producing plasma cells

"DYSGAMMA IS MOST COMMON AND HAS A FAILURE TO DIFFERENTIATE"

Primary immune deficiencies--5 (5) Wiskott-Aldrich syndrome: - --Thrombocytopenia = bleeding - Eczema - Recurrent infections (T cell deficiency and low serum IgM level

"WISKOTT HAS BLEEDING ECZEMA"

Secondary (acquired) immune deficiencies 1. Acquired Immunodeficiency Syndrome (AIDS) Etiology: Human immunodeficiency virus (HIV), a small enveloped RNA retrovirus Transmission: (1) Sexual contact (homosexual, heterosexual) (2) Transfusion of blood and blood products (hemophiliacs, drug addicts sharing needles) (3) Maternofetal: through placenta, during delivary or breastfeeding Pathogenesis - Virus attacks helper T cells(CD4)-Depletion of helper T cells: - depression of CMI and humoral immunity

"AIDS HATES T"

(AIDS)--2 Clinical features (1) Early (acute) stage: 3-6 weeks----non-specific symptoms: fever, sore throat, skin rash, headache, myalgia, resolve in 2- 3 weeks (2) Middle (latent) stage:---Asymptomatic, lasts for years (4-20) ---AIDS-related complex:persistent fever, diarrhea, weight loss, lymphadenopathy (3)Late (immunodeficiency) stage; --DECLINE IN NUMBER OF HELPER T CELLS ---Malignancies: EX KARPOSI'S SARCOMA HIV Testing Primary Test: ELISA (Test for antibodies) Confirmatory Test: Western Blot (test for antibodies)

KEYS =--DECLINE IN NUMBER OF HELPER T CELLS | ---Malignancies: EX KARPOSI'S SARCOMA