Autoimmunity Flashcards

1
Q

What is the lack of response to the body’s own cells known as?

A

tolerance

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is autoimmunity?

A

when a body develops autoantibodies as a reaction to its own tissues or self-antigens

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What do almost all patients with autoimmunity have present in their serum?

A

autoantibodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What does lupus affect?

A

skin, heart, brain, blood, kidney, lungs and joints

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Lupus stats (americans w/ it, worldwide pop, priamry gender, diagnosis age)

A

1.5 Americans
5 million worldwide
90% r women
diagnosed between 15 to 44

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the 4 types of lupus

A

systemic, cutaneous, neonatal, and drug induced

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is the primary form of lupus + what percent of poel have it?

A

systemic lupus, 70%

aka systemic lupus erythematosus (SLE)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

About ____ of SLE patients develop severe disease of the heart, lungs, kidneys, or brain.

A

half

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are the common signs and symptoms of SLE

A

fatigue
arthritis
butterfly rash on cheeks and nose
fever
photosensitivity
secondary Raynaud’s pehnomenon
mouth or nose ulcers
inflammation of the skin, joints, nervous system, kidneys, lungs, and other organs
malaise
wt. loss
discoids skin lesion
polyarthritis without deformity
seizures or psychosis
chronic pleuritis or pericarditis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is cutaneous (aka discoid) lupus + what % of cases

A

affects only the skin

accounts for 10% of all cases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

most common rash caused by cutaneous lupus?

A

red, scaly, raised but NOT itchy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Why is cutaneous lupus known as a discoid rash?

A

bc the rashes r usualy circular

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Where else does discoid rashes appear?

A

face, neck, scalp, nose, mouth and vagina

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Approximately __% of patients with cutaneous lupus will
develop SLE.

A

10

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

How many drugs can lead to Drug-induced lupus erythematosus (DILE) + what %

A

70+ drugs and 10% of all lupus cases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Difference between SLE and DILE?

A

DILE rarely affects organs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What are some of the medications known to cause DILE?

A

hydralazine (used to treat high blood pressure)
procainamide
quinidine (used to treat irregular
heart rhythm)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is the risk of developing DILE on most drugs?

A

less than 1%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is neonatal lupus?

A

the autoantibodies of the mother affects the fetus/ newborn’s skin, heart and blood

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is the ethiology of lupus?

A

it’s unknown but genetic + envrionmental factors have an impact

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Environmental factors of lupus?

A

infections
sulfa antibiotics and penicillin
ultraviolet light
stress
certain drugs
hormones

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

__% of lupus patients have a parent or a sibling with lupus

A

20

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

__% of children develop
lupus if their mother has lupus.

A

5

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

For women when do the signs and symptoms of lupus worsen?

A

before menstrual cycle and during pregnancy so estrogen may have an impact in women

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
What should lupus patients do?
limit exposure to sunlight never use tobacco prevent fatigue and stress eat a healthy diet exercise take care of fevers over 100°F promptly
26
How to diagnos lupus?
no single test to confirm so blood test, biopsy, urinalysis, autoantibody testing and measurement of general level of inflammation
27
Medication to control lupus:
NSAIDs corticosteroids antimalarial medications immunosuppressive drugs monoclonal antibodies
28
between ___ and ___ % of lupus patients die prematurely as a result of complications
10 and 15
29
What scleroderma?
chronic, progressive autoimmune disease of the connective tissue in with sclerosis of skin, scarring of certain internal organs occurs - classified as either diffuse or limited, depending on extent and location of skin involvement
30
What is the word scleroderma dervied from?
Gre words sklera for hard and derma for skin
31
When was the word scleroderma first used?
In 1836 Giovambattista Fantonetti described a patient who has dark leathery skin that results in loss of joint movement range
32
Stas of scleroderma (ppl in us, worldwide, gender, dagnosis age)
300,000 in us 2.5 mill worldwide women are 4x more likely to get it compared to men diagnosis age is in 40s
33
2 types of scleroderma + what is more common
localized scleroderma (LSc) which only affects the skin Systemic scleoriss scleroderma (SSc) which affects skin + internal organs
34
How does LSc appear?
looks like waxy patches of skin (morphea) or strakes on the skin (linear sclerosis) stop progresses or regresses w/o treatment
35
What are the 2 types of SSc?
limited cutaneous SSC (lcSSc) and diffuse cutaneous SSc (dcSSc)
36
What is lcSSc?
has skin tightening on fingers, hands, arms below elbows, feet and les below knees
37
What parts of the body does dcSSC affect?
fingers, hands, and arms below the elbows, skin on the feet legs below the knees, skin on the trunk, above the elbows, and on the knees
38
Do pt with lcSSc or dcSSc have a greater risk for significant organ dysfunction?
dcSSc
39
The 10-year survival rate for lcSSc is __%
71
40
The 10-year survival rate for dcSSc is __%.
21
41
What are the 1st signs and symptoms of scleroderman?
seconadray Raynaud's phenomenon tightening of the skin of the fingers pain in 2 or more joints, heartburn diffulcuty swallowing SOB
42
Impact of immune system on scleroderma?
could stimulate fibroblasts to produce too much collagej = stiffening
43
What are some other factors to scleroderma?
exposure to viral infection and certain chemicals can = scleroderma in ppl genetically predisposed to it may be linked to estrogen
44
How to diagnose scleroderma?
no single test - autoantibodies and biopsy - complete history and physical exam - lab testing including Scl-70 and centromere antibodies can be supportive of diagnosis - skin biopsy is only rarely necessary - blood tests and urinalysis r performed to rule complications or accompanying conditions - lung function testing and echocardiography
45
What should scleroderma pt do to manage the disease?
small frequent meals, no smoking, healthy diet, exercise, avoid exposure to cold, moisturize, protect skin
46
treatments to manage scleroderma
anti-inflammatory drugs, immunosuppressive drugs, and vasodilators, corticosteroids - treatment is directed to area of the body affected - rheaumatology referral is recommended - physical therapy helps pt maintain muscle strength but doesnt chagne course of joint disease
47
What is Sjögren’s syndrome?
autoimmune disease that causes inflmmation of the exocrine (moisture producing) glands in the body resulting in dryness in affected areas - cause is unknown but genetic factors seem to play a role in predisposing a person to Sjorgren syndrome
48
stats of Sjögren’s syndrome (ppl in us = gender)
4 mill in us 90% r women
49
About ____ of the cases are called primary Sjögren’s syndrome.
half
50
What is secondary Sjögren’s syndrome?
Sjögren’s syndrome that occurs with another disease
51
symptoms of Sjögren’s syndrome
dry eyes and mouth (xerostomia) - keratoconjunctivitis sicca - difficulty talking, chewing and swallowing from dryness of nasal, oral and laryngeal pharynx - sores in or on mouth and nose - dental decay may occur - can cause complications in lungs, heart, GI tract, bv, liver, pancrease and CNS
52
When does Sjögren’s syndrome often affect women?
during childbearing years (shows possible link to estrogen)
53
how to diagnose Sjögren’s syndrome?
- lip and salivary gland biopsy, tears and saliva tests, autoantibody testings - screen for med side effects, autoimmune thyroid disease, RA, SLE, sarcidosis, scleroderma associated with Sjorgen syndrome - schirmer (tests for dry eyes) - enlarged salivary glands = lower lip bopsy, which may indicate characteristci infiltration of lyphmocytes - blood studies often demonstrate anemia, decreased WBC count and elecated ESR - test for Sjogren syndrome antibodies (SSA and SSB antibodies) and high rheumatoid factor titers
54
Drugs for Sjögren’s syndrome
treatment is directed toward releiving symptoms esp dryness in oral cavity & eyes - increasing fluid intake, chewing sugarless gum, using oral sprays to help relieve oral dryness - artifical tears + wearing sunglasses - sometimes ophthalmologists block tear ducts w/ silicone or by cautery to maintain adequate eye moisutre - occasionally prednisone and/or antimalarial med such as hydroxychloroquine r used to lessen immune inflammation eye drops, NSAID, immunosuppressive drugs and vasodilators
55
etiology of autoimmune disorders?
thought to be acquired, not genital, however certain gene markers identify pt at increased risk for many autoimmune disorders
56
What is autoimmune hemolytic anemia?
- autoimmune condition in which RBCs r destroyed by antibodies
57
s/s of autoimmune hemolytic anemia?
- pt experience fatigue, weakness, chills, fever, dyspnea, itching - skin is pale and jaundiced and bruises easily (pt may be hypotensive
58
etiology of autoimmune hemolytic anemia?
- b cell-produced antibodies don't identify RBCs as self, resulting in agglutination of RBCs and an attack on and destruction of the red corpuscles - 2 types r warm antibody (agglutinin) anemia and cold antibody anemia (agglutinin)
59
What is warm antibody anemia
- excess of IgG antibodies that react with protein antigens on the RBC surface at body temp (most cases r idiopathic, some may be activated by certain drugs (eg penicillins), autoimmune diseases (eg SLE) or malignancies (esp chronic lymphocytic leukemia)
60
What is cold antibody anemia
- results from fixation of complement proteins on IgM that occurs at colder temp (best around 30F) - type is regularly seen in conjunction with infectious mononucleosis or Mycopasma pneumonia
61
How to diagnose autoimmune hemolytic anemia?
- direct Coombs test indicates antibody-coated RBCs that agglutinat when atinglobulin is added to medium - some RBCs appear spherical (spherocytes) as visualized under microscope - serum bilirubin levels r elevated - RBC count, platelet count, hemoglobin concentration, hematocrit usually are all decreased - reticulocytet (immature RBC) count is usually elevated - examination of peripheral blood smear for agglutination of RBCs and flow cytomentry may be performed
62
How to diagnose autoimmune hemolytic anemia?
- direct Coombs test indicates antibody-coated RBCs that agglutinat when atinglobulin is added to medium - some RBCs appear spherical (spherocytes) as visualized under microscope - serum bilirubin levels r elevated - RBC count, platelet count, hemoglobin concentration, hematocrit usually are all decreased - reticulocytet (immature RBC) count is usually elevated - examination of peripheral blood smear for agglutination of RBCs and flow cytomentry may be performed
63
how to treat autoimmune hemolytic anemia?
- 1st treat underlying disease or drug use - for warm antibody anemia, corticosteroids and cytotoxic drugs r administered to reduce antibody production, splenectomy, IVIG - avoidance of cold by dressing warmly, plasmapheresis
64
What is SLE?
- chronic, inflammaotry autoimmune disease characterized by unusual autoantibodes in the blood that targets tissues of the body
65
lab tests for SLE?
- false-positive serologic test result for syphilis - DNA or Sm (Smith) antibodies in blood or cardiolipin antibody - greater than 0.5 protein in urine in 24 hr periods, cellular casts in urine hemolytic anemia, thombocytopenia, persence of abn antibodies in bloodstream, LE cells (WBCs) that r created in lab as part of ttesting, CBC w/ differential leukocyte count, platelet count, erythrocyte sedimentation rate (ER), antinuclear antibody determination, anti-DNA antibody, anti-Sm antibody and anti-chromatin antibody
66
how to treat SLE?
- antiinflammatory drugs: aspririn, NSAIDs, prednisone - antimaralarial agents to control skin involvement and arthralgia - corticosteroids - immunesuppressive meds
67
s/s of scleroderma?
- sclerosis and shrinking of skin and certain internal organs such as GI tract, heart, lungs and kidneys - onvolved skin becomes taut, firm, edematous and is firmly attached to subcutaneous tissue - skin feels touch and leather, mau itch and pigmented patches - raynaud phenomenon is often first s/s, follsoed by swelling, stiffness and pain in joint s
68
What is vasculitis?
- inflammation in walls of blood vessels - affected vessel become necrotic when obstructed b y thrombus and an infarct of adjacent tissue results - bopsy of involved vessel can demonstrate presence of inflammation in blood vessel - classified into 2 main types: small vessel vasculitis and systemic necrotizing vasculitis
69
What is small vessel vasculitis?
- primarily affects capillaries, arterioles and venules
70
s/s of small vessel vasculitis
- petechiae (nonblanching) - purpura - erythema - ulcerations and edema (most often on skin of lower extremities) - pain and burning sensation can accompany these lesions - ocular lesions - genital or oral ulcerations - abd pain - arthralgia - weakness
71
diagnosis and treatment of small vessel vasculitis
diagnose - histology and often immunoflouroscence studies of biopsied tissue, presence of Ig and complement in blood vessels, pattern of vascular involvement and the cell types presents can help define subtype of small vessel treatment - corticosteroid therapy, analgesics and rest
72
What is systemic necrotizing vasculitis + etiology?
- tends to primarily affect medium and large arteries - etiology is unknown but autoimmune respones play a role and r mediated by cellular and humoral immunity (illicit drug use, includign cocain and amphetamines, hepatitis B and C r associated w/ development of some forms of systemic vasculitis)
73
s/s of systemic necrotizing vasculitis
- impaired tissue perfusion causes ischemic pain - paralysis of an affected nerve can lead to weakness or numbness - ulceration of skin - headaches - fever - weakness - fatigue - malaise - anorexia - wt loss - muscle and joint pain - angina - dyspnea - hypertension - visual disturbances
74
diagnose of systemic necrotizing vasculitis
- comprehensive blood studies including CBC, ESR, RA factor determinatioon and serum tests for Ig - CBC indicates anemia and usually elevated WBC and platelet counts - ESR is elevated durign acute stage - CRP test shows an earlier and more intese increasecompareed with ESR - chest radiographs show pulmonary infiltrates - hematuria and proteinuria - aneurrysm and myocardial ischemia - biopsy confirms diagnosis by demonstrating invasion of leukocytes in blood bessels - angriography
75
treatment of systemic necrotizing vasculitis
- decreasing inflammation of arteries and improving the function of affected organs - addresses underlying causative factors and systemic involvement - corticosteroids and analgesics - immune-suppressing meds such as azathioprine and cyclophsphamide - plasmapheresis - antihypertensive drugs such as angiotensin-converting enzyme (ACE) inhibitor