Unit 4 Flashcards
What does immunity refer to?
refers to protection against infections
What is innate immunity?
Immunity mediated by mediated by cells and proteins that are always present and poised to fight against microbes, being called into action immediately in response to infection
What is adaptive immunity?
Adaptive immunity is normally silent and responds (or “adapts”) to the presence of infections and microbes by trying to eliminate them
What is hypersensitivity,?
Injurious immune reactions
immune responses may be inad- equately _______ or inappropriately _____ to host tissues, and in such situations, the normally beneficial response is the cause of disease (Immune mediated injury)
Controlled, targeted
What is type 1 Hyper sensitivity?
Immediate allergic reaction to allergens which are usually harmless to others
Type I hypersensitivity is activated via _____ cells and produce _____ antibodies. It also causes mast cells to release ______ in order to aid with the attack
T helper cells TH2, IgE, histamine
During Type 1 hypersensitivity there is an increase in mucus secretion, vasodilation, and ____
bronchoconstriction
This type of inflammation causes itching, pruritis, diarrhea, laryngeal edema, increased mucus production, and vasodilation which lead to edema (hypotension if severe)
Systemic
In type 1 hyper sensitivity the inmediate response usually takes about ____ and subsides within_____ . It deals with vascular and sm. muscle reactions
30 minutes, 60 minutes
Late phase reaction in type 1 sensitivity is usually due to ________ _______ and the onset is typically between ___ And ____
epithelial damage, 2,8
What are type 2 hypersensitivity disorders caused by?
antibodies that bind to fixed tissue or cell surface antigens, promoting phagocytosis and destruc- tion of the coated cells or triggering pathologic inflam- mation in tissues.
What are examples of type 2 disorders?
ABO incompatibility, acute rheumatic fever,
Goodpasture syndrome, Graves’ disease
What are type 3 disorders caused by?
antibodies binding to antigens to form complexes that circulate and deposit in vascular beds and stimulate inflammation, typically as a consequence of complement activation. Tissue injury in these diseases is the result of the inflammation.
What are the two main types of Type 3 related inflammations?
• Systemic: fever, arthritis, vasculitis,
lymphadenopathy, nephritis (proteinuria)
• Local: vessel walls, kidneys, joints
What are examples of Type 3 disorders?
Lupus (SLE), polyarteritis nodosa (PAN), reactive arthritis (Reiter syndrome), serum sickness
Type 4 hypersensitivity disorders are caused by
by immune responses in which T lym- phocytes produce cytokines that induce inflammation and activate neutrophils and macrophages, which are responsible for tissue injury.
In type 4 hypersensitivity, cytokine mediated CD4 + T cells usually progress into ______ and can be caused by ______
chronicity, poison ivy
In type 4, direct cytoxicity can cause the ______ to _______
CD*8+ Cells to attack antigens (viruses, neoplastic cells, and transplants)
Examples of Type 4 disorders are
TB, leprosy
What are autoimmune disorders?
Immune reactions to self antigens which affect 2% to 5% of the population in developed countries, and appear to be increasing in incidence.
What is the mnemonic for hypersensitivities?
ACID
A Allergies Type 1
C Cytotoxic Type 2
I immune complex Type 3
D Delayed Type 4 (T cell mediated)
What is immunologic tolerance?
unresponsiveness to an antigen that is induced by exposure of specific lymphocytes to that antigen
what is Self tolerance ?
refers to a lack of immune responsiveness to ones own tissue antigens
what is Failed self tolerance?
Immune response to ones own tissue antigens AKA self reactive T and B cells
What are the two mechanisms that work in concert to select agains self reactivity and prevent immune reactions against the bodys own antigens?
Central and peripheral tolerance
What is central tolerance?
Mechanism that induces apoptosis in the thymus for T cells and B cells in the bone marrow
What is peripheral tolerance?
Mechanisms which causes functional deactivation, suppression, or apoptosis of T cells which escape the thymus
What tissues are primarily affected by Autoimmune disorders?
Connective tissues and vessels
what is the most common cause for AI disorders?
Idiopathic but can also go with Genetic mutations and environmental factors
Autoimmune diseases have a tendency to run in fami- lies, and there is a greater incidence of the same disease in monozygotic than in dizygotic twins. (True or False)
True
What are the Alleles that are linked with Autoimmune disorders?
HLA
When dealing with genetic susceptibility one would expect ____ _____ to be most commonly affected
Childbearing women
What are the mechanisms through which viruses and bacteria can cause AI reactions?
Cross reactivity and infections with necrosis and inflammation
What is SLE ?
a multisystem auto- immune disease of protean manifestations and variable clinical behavior which has Massive antibody formation due to failed self tolerance
It is extremely variable and may involve any organ but is particular to the skin, kidneys, serous membranes, joints, heart, and CNS
It has an insidious onset
Unpredictable and can relapse
Where is SLE most common?
In young adult females of african american heritage
What is the mechanism of injury in SLE?
Most organ damage in SLE is caused by immune complex deposition (TYpe 3) and Autoantibodies against red cells, white cells, and platelets (type 2)
What type of damage can SLE cause to vessels?
An acute necrotizing vasculitis affect- ing small arteries and arterioles may be present in any tissue.
Fibrinoid necrosis
What is the most common cause of death in SLE and how does it happen?
renal failure is the most common cause of death via glomerulonephritis which could cause proteinuria and hematuria forming RBC casts in the urine
SLE can cause idiopathic fever?
Yes
How is the skin affected in SLE?
Exposure to sunlight (UV light) exacerbates erythema already present. (discoid rash)
50% of the time patients present a malar rash
Liquefactive degeneration or necrosis in the basal layer of epidermis
IGG deposits in uninvolved skin
With SLE this organ is tipically enlarged and has formations of capsular fibrous thickenings are common as well as follicular hyperplasia
spleen ‘(splenomegally)
Which serosal membranes show inflammatory changes in SLE?
Pericardium and pleura
How is the heart involved in SLE?
manifested primarily in the form of pericarditis
however , Libman Sacks endocarditis can also occur.
What is Libman Endocarditis?
nonbacterial verrucous endocarditis takes the form of irregular, 1- to 3-mm warty deposits, seen as distinctive lesions on either surface of the leaflets
How is the CNS involved in SLE?
CNS disease often is ascribed to vascular lesions causing ischemia or multifocal cerebral microinfarcts.
Which antibodies are found in 100% of the patients in SLE?
ANAs which are 99% sensitive and 80% specific
SLE can also cause lowering of RBCS, WBCS, and platelets which is known as ______
pancytopenia
What are the 11 diagnostic criterion for SLE?
Malar rash, discoid rash, photosensitivity, oral ulcers, arhtritis, serositis, rena disorder, neurologic disorder, hematologic disorder, immunologic disorder, and ANAcount
In twins, which type is more likely to get SLE?
Monozygotic
What is the HLA allele that is associated with SLE?
HLA-DRB1
What are the risks for getting SLE?
estrogen hormones, UV light,smoking, ADRs and infections
What is the treatment for SLE?
Corticosteroids, Immunosuppressive meds
What other pathology can be acquired via SLE?
B cell lymphoma
What is Sjorgren Syndrome?
a clinicopathologic entity character- ized by dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia), resulting from immune-mediated destruction of the lacrimal and salivary glands.
Less commonly affected areas are the vagina and nasopharynx
Could test positive for ANA
This AI disorder is secondary to other AI disorders 60% of th time (accompanied by RA, SLE or systemic Sclerosis) and is most common in middle aged females (familial). It also possesses an increased risk of developing B cell lymphoma
Sjorgren Syndrome
What is Systemic sclerosis?
is an immunologic disorder characterized by excessive fibrosis in multiple tissues, obliterative vascular disease (destroys small arteries )
Which AI disorder affects the Skin (95%), G.I. (90%), kidney (65%), lungs (50%), heart (30%), skeletal muscle and has features like Raynaud phenomenon, malabsorption, dysphagia, renovascular HTN, renal failure,
pulmonary HTN, dyspnea, myalgia, arthritis and is 3x more likely to affect 40-60 females?
Systemic sclerosis
When it is limited scleroderma is often only confined to the ____ and ______
fingers and face
What are the features of the Crest syndrome present in Limited scleroderma?
- Calcinosis
- Raynaud phenomenon
- Esophageal dysmotility (fibrosis of ↓ 2/3)
- Sclerodactyly (hard/eroded skin)
- Telangiectasia (spider veins)
What is diffuse scleroderma characterized by?
characterized by initial widespread skin involvement, with rapid progression and early vis- ceral involvement
What are Primary (Congenital) Immune Deficiencies?
immune deficiencies that are genetically determined and affect either adap- tive immunity (i.e., humoral or cellular) or innate host defense mechanisms, usually exhibit recurring infections
what isX-Linked agammaglobulinemia (Bruton disease) characterized by?
chacterized by the failure of pre-B cells to differentiate into B cells and, as the name implies, there is a resultant absence of antibodies (gamma globulin) in the blood.
Prevalent in 1/100,000 male infants which are very susceptible to • Bacterial/viral infxn: otitis media, pharyngitis, sinusitis and are usually treated with IGG infusions
What Immunodeficiency has ↓ antibody response to infxn., normal B cells,
but no plasma cells, G.I. infections has a Diagnosis of exclusion. The prevalence in male and females of ages 10-30 is about 1/50k
Common variable immunodeficiency
What type of immunodeficiency affects caucasians more typically has decreased Iga Production and is most commonly asymptomatic and or very mild yet causes respiratory and GI infections?
Isolated IgA deficiency
What deficiency is characterized by production of normal (or even supranormal) levels of IgM antibodies to antigens but no production of the IgG, IgA, and IgE isotypes/ has recurrent pyogenic bacterial infections and is commonly associated with X linked mutations?
Hyper IgM syndrome
What is SCID?
epresents a constellation of genetically distinct syndromes with the common feature of defects in both humoral and cell- mediated immune responses. Rendering the patient vulnerable if not defenseless to all sorts of microbes
What population is SCID most prevalent in?
Native Americans
What defect is characterized by Defective TH17: chronic mucocutaneous fungal infxns.?
Defects in lymphocyte activation
What defect is characterized by complement proteins, phagocytes, TLRs, and multiple infections?
innate immunity
What type of Immune deficiency disorders are more common than primary immune deficiencies and are the result of infections, malnutrition, aging,cancer, and therapy induced I-suppression (MC)
Secondary
Which retroviral disease is caused by HIV and is characterized by nfection and depletion of CD4+ T lymphocytes (via cytotoxicity and apoptosis) leading to Opportunistic infxns., tumors, CNS defects?
AIDS
In the US AIDS has more prevalent transmission via
Male-male sex (MC): 48%, U.S., declining
In the World AIds has more prevalent transmission via
Male-female sex: 35%, MC group in Africa & Asia
Drug abusers make up ____ of the population affected by aids, where as blood recipients make up ____ and children (tansplacental, childbirth and transmission via breastmilk) make up __
17%, 1-2%, 1%
This disease is 75% sexually transmitted via Semen, abrasions and has an increased risk of other STDS
AIDS
What are the two tests used to Dx aids?
Elisa and western blot
Acute AIDS causes
Acute: pyrexia, pharyngitis, myalgia, viremia • 50-70% of cases, 3-6 weeks after infxn.
• ↓ viremia after ~12 weeks
Chronic (Latent ) HIV/AIDS entails
- Minor signs/symptoms, generalized lymphadenopathy
* Steady ↓ of CD4+ T cells, gradually ↑ viremia • Infxns: candidiasis, VZV (pyrexia, fatigue)
Crisis of HIV/AIDS entails
≤200 cells/μL, ↑ viremia, cachexia, fatigue, TM
pyrexia (>1 mo.), diarrhea, wt. loss, CNS defects • Pneumocystis jirovecii, Kaposi sarcoma, Burkitt
Immune deficiency disorders
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lymphoma, 1° CNS lymphoma, cervical cancer
The main mechanism of injury of the CNS in aids is via
meningitis caused by infected monocytes
What causes 80% of AIDS death?
Opportunistic infections Fungal: pneumocystosis, candidiasis and also Neoplasia: Kaposi sarcoma, 1° CNS lymphoma
What is amyloidosis?
a condition associated with extracellular deposits of fibrillar proteins are responsible for tissue damage and functional compromise
Inherited condition that is due to misfolded proteins or unstable proteins
Failed phagocytosis of these leads to accumulation and disruption of function
What is an amyloid light chain?
protein is produced by plasma cells and is made up of complete immunoglobulin light chains, the amino-terminal fragments of light chains, or both.
ssociated with some form of monoclonal B cell proliferation.
What is an Amyloid associated protein?
A protein that comes from the liver and is dervied from a larger serum (SAA) it is found or associated with chronic inflammation TB, RA, AS, IBD, osteomyelitis
What is a Beta Amyloid?
An amyloid precursor protein that leads to cerebral plaques involved in Alzheimers
This condition involves AL proteins, is systemic, caused most commonly by malignant plasma cells, and is associated with Multiple myeloma
Primary systemic amyloidosis
This condition is reactive, found in chronic inflammation and involves AA proteins
Secondary systemic amyloidosis
Amyloidosis in the elderly
Senile systemic amyloidosis
This condition attacks a single organ or tissue, it is associated with AL proteins and usually attacks the brain heart kidney and or tongue
Localized Amyloidosis
This condition is rare and is due to geographic locations and population. It is associated with AA proteins and inflamasome mutations and example of a symptom would be mediterranean fever
Hereditary amyloidosis
- Tongue: macroglossia
- Kidney: common & severe
- Enlarged, pale, firm, ↓ function
- Brain: Alzheimer disease: Aβ proteins
- Spleen: splenomegaly, firm, ↓ function
- Liver: massive hepatomegaly, pale, ↓ function
- Heart: cardiomegaly, ↓ function • Restrictive cardiomyopathy
- Wrist ligaments: long-term dialysis,
Are all morphological factors of
Amyloidosis
