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Block 6; Week 2-Michelle > Hunter-Autoimmune Diseases > Flashcards

Hunter-Autoimmune Diseases Flashcards

1
Q

Autoimmune diseases can be related to multiple types of hypersensitivities, but not all. Which ones does it include?

A

Types 2, 3, 4

NOT Type 1.

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2
Q

In a basic sense, what is an autoimmune disease?

A

a disease in which your body has a difficult time differentiating self from non-self

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3
Q

Which do we worry more about in terms of developing tolerance to self–B cells or T cells? Why?

A

We worry MUCH more about T cells than B cells. This is b/c a B cell can almost never mount an attack without T cell help!

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4
Q

Describe what happens to T cells when they are released from the bone marrow?

A

they go to the thymus
this is where they learn self tolerance
their recognition for things is developed by random combinations–some of these recognize pathogens & some recognize self. They kill the ones that recognize self. Only 3% make it out alive–such carnage!!

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5
Q

What are 2 questions that are addressed when a T cell is maturing in the thymus?

A

Q1: Do you have an MHCII receptor-like are you basically functional?
Q2: Do you recognize self antigens? If so, you must die.

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6
Q

How do you test these young T cells while they are in the thymus?

A

you have them come into contact with many MHCIIs that are presenting self antigen. If they react-they die.
The crazy thing is that the self antigens aren’t just thymic–they represent a ton of different things in the body like insulin! The things that aren’t represented aren’t a big deal–they are small in number or secluded in the body away from the immune system.

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7
Q

What is central tolerance in the thymus? What % does this affect?

A

self-reactive clones undergo apoptosis. 97% of the self-reactive clones die this way.

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8
Q

What happens to the 3% of self reactive clones that aren’t killed via central tolerance?

A

Peripheral Tolerance!

There are 3 signals required to activate a T cell. The bad guys rarely get that far. They usu die along the way.

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9
Q

What are the signals that are required in the periphery to turn on a T cell?

A

Have to interact with an APC w/ an MHCII that it recognizes. The APC has to be activated & have B7. CD28 on the T cell must react with that so you get costimulatory signals. Costimulatory also known as CD80/CD86.

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10
Q

If a self-reactive clone escapes central & peripheral tolerance…what is the hope?

A

regulatory T cells

These will inhibit the T cell/APC interaction & hopefully cause apoptosis.

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11
Q

There is another naturally protective mechanism that the body has to stop the bad T cells. It is the reason that a B cell attack of keratinocytes wouldn’t work.

A

Keratinocytes do have an MHCII that could react to a bad guy. But they don’t have B7 b/c they aren’t APCs.

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12
Q

A lot of us have T cells that recognize myelin basic protein. Why don’t a lot of us have MS?

A

b/c it also requires the microglia in our CNS to express B7 from a viral infection or something & to come in contact with the bad T cell. Perfect storm required.

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13
Q

What is a microglia?

A

It is like the macrophage of the CNS.

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14
Q

What is the perfect storm for autoimmunity?

A

The ingredients for autoimmunity:
T cells with receptors that see self peptides (not all self reactive T cells are removed by negative selection in the thymus)
Susceptibility genes like MHC that allow certain self peptides to be presented by antigen presenting cells (APC)
A “trigger” that upregulates co-stimulatory molecules on the APC (Infection or tissue damage)
Summary: A self reactive T cell, APC with bad MHC…activate APC to present costimulatory factors.

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15
Q

One of the requirements for a perfect storm is an APC with a bad MHCII for a self antigen. But there is another way for the perfect storm to happen…describe it.

A

Molecular mimicry.
A self reactive T cell that also recognizes microbial peptides.
APC that presents a microbial peptide that looks like self.
Then the T cell goes on with an attack of self.

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16
Q

What are 2 autoimmune diseases that include genetic susceptibility b/c of MHCI issues–as in a certain HLA?

A

ankylosing spondilitis

acute anterior uveitis

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17
Q

What are some autoimmune diseases that include genetic susceptibility b/c of MHCII issues–as in a certain HLA?

A 
Goodpasture's
MS
Grave's
Myasthenia Gravis
SLE
Type I Diabetes
Rheumatoid Arthritis
Pemphigus Vulgaris
Hashimotos
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18
Q

What are some Type II Hypersensitivity Autoimmune diseases that exhibit autoantibodies against surface antigens on blood cells?

A

autoimmune hemolytic anemia

autoimmune thrombocytopenic purpura

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19
Q

What is the target antigen in autoimmune hemolytic anemia?

A

erythrocyte membrane antigens–Rh antigens

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20
Q

What is the mechanism of disease in autoimmune hemolytic anemia?

A

opsonization & phagocytosis of erythrocytes

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21
Q

What are the clinicopathologic manifestations of autoimmune hemolytic anemia?

A

hemolysis–anemia

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22
Q

What is the target antigen in autoimmune thrombocytopenic purpura?

A

platelet membrane proteins–integrin

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23
Q

What is the mechanism of disease in autoimmune thrombocytopenic purpura

A

opsonization & phagocytosis of platelets

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24
Q

What are the clinicopathologic manifestations of autoimmune thrombocytopenic purpura?

A

bleeding

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25
Q

What are some Type II Hypersensitivity Autoimmune diseases that exhibit autoantibodies against cell surface antigens or fixed tissues?

A

pemphigus vulgaris
goodpasture’s
acute rheumatic fever

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26
Q

What is the target antigen with pemphigus vulgaris?

A

proteins in intercellular junctions of epidermal cells (epidermal cadherin)

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27
Q

What is the mechanism of disease in pemphigus vulgaris?

A

antibody-mediated activation of proteases

disruption of intercellular adhesions

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28
Q

What are the clinicopathologic manifestations of pemphigus vulgaris?

A

skin vesicles (bullae)–it is a skin disease

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29
Q

What is the target antigen of good pasture’s?

A

noncollagenous proteins of the basement membrane of kidney glomeruli & lung alveoli

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30
Q

What is the mechanism of disease in good pasture’s?

A

complement & Fc-receptor mediated inflammation

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31
Q

What are the clinicopathologic manifestations of autoimmune good pasture’s?

A

nephritis, lung hemorrhages

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32
Q

What is the target antigen of acute rheumatic fever?

A

streptococcal cell wall antigen-antibody cross reacts with myocardial antigen

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33
Q

What is the mechanism of disease in acute rheumatic fever?

A

inflammation

macrophage activation

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34
Q

What are the clinicopathologic manifestations of acute rheumatic fever?

A

myocarditis & arthritis

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35
Q

What are some Type II hypersensitivity autoimmune diseases that exhibit autoantibodies against cellular receptors?

A

Grave’s Disease

Myasthenia Gravis

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36
Q

What is the target antigen for Grave’s Disease?

A

TSH receptor

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37
Q

What is the mechanism of disease in Grave’s Disease?

A

antibody-mediated stimulation of TSH receptors

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38
Q

What are the clinicopathologic manifestations of Grave’s Disease?

A

hyperthyroidism

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39
Q

What is the target antigen for Myasthenia Gravis?

A

Ach receptor

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40
Q

What is the mechanism of disease for myasthenia gravis?

A

antibody inhibits Ach binding, down regulates receptors

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41
Q

What are the clinicopathologic manifestations of myasthenia gravis?

A

muscle weakness, paralysis

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42
Q

The mechanism of autoimmune hemolytic anemia is similar to what other disease? What are the basics of it?

A

similar to extrinsic hemolytic anemia (including drug-induced)
**T cells & B cells recognize antigens on the surface of the RBC. Causes lysis or phagocytosis

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43
Q

A 29-year-old man presented with spontaneous bruising of his arms and chest. He had three recent epistaxes but no other bleeding. He was not taking any drugs and had no risk factors for HIV. The spleen was not palpable. On investigation, he had a normal hemoglobin (138 g/l) and white cell count, but a low platelet count of 10 × 106/ml (normal >150 × 106/ml). His serum CRP and immunoglobulin levels were normal; direct Coombs’ test was negative; antinuclear and DNA-binding antibodies and rheumatoid factor were absent. His bone marrow contained an increased number of normal megakaryocytes but was otherwise normal.
What does this patient have? What is the proper treatment for him?

A

A diagnosis of immune thrombocytopenia was made and he was started on a high dose of prednisolone. His platelet count rose rapidly over the next few days and the steroids were tailed off over 4 weeks. He relapsed 10 months later with further bruising, but again responded to a short course of oral steroids.

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44
Q

What happens in autoimmune thrombocytopenia?

A

autoantibodies formed against goiib: iii a–a platelet plasma membrane integrin
**these antibody coated platelets are destroyed by phagocytes in the liver & spleen

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45
Q

What is usu the proper treatment for autoimmune thrombocytopenia?

A

corticosteroids–to inhibit phagocytosis

splenectomy

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46
Q

What is the bruising called in autoimmune thrombocytopenia? What causes it?

A

purpura

here it is caused by a low platelet count–platelet involved in coagulation

47
Q

What is the significance of this patient with autoimmune thrombocytopenia have a high level of megakaryocytes?

A

these are the progenitor cells for platelets

48
Q

A 65-year-old woman of Ashkenazi Jewish descent presents to the dermatology clinic with a weeping skin lesion on the right cheek and periorbital area. She also has numerous erosions and ulcers in the mucosa of her mouth and gums. These lesions had developed over the previous month. A biopsy of the skin lesion revealed disruption of the epidermal layer with blistering, and immunofluorescence studies showed intercellular deposits of IgG. ELISA analysis reveals a 1:1250 titer of antibodies to the intercellular adhesion molecule desmoglein-3 on keratinocytes.
What does this patient have?

A

Pemphigus Vulgaris

**a disease of antibodies against a cadherin-like molecule that holds skin together

49
Q

What does a biopsy of an early skin lesion show in pemphigus vulgaris?

A

epidermal disruption
blister formation
hypercellular & deposition of lots of IgG antibodies

50
Q

What does a direct immunofluorescence study of a biopsy of tissue of a pemphigus vulgaris patient show?

A

IgG4 antibodies in intercellular spaces

51
Q

T/F Initial antibodies to desmoglein-3 (an adhesion molecule) on keratinocytes cause disease.

A

False.

52
Q

What directly causes the pemphigus vulgaris disease?

A

IgG4 antibodies to new desmoglein-3 epitopes appear by molecular epitope spreading, and these antibodies cause disease

53
Q

An ELISA analysis of serum of a pemphigus vulgaris patient would reveal what?

A

anti-desmoglein-3-antibodies. this is a pemphigus antibody.

54
Q

What is the treatment for pemphigus vulgaris?

A

prednisone

if extreme: cyclophosphamide

55
Q

A 19-year-old African American man presented to the emergency department with shortness of breath of two weeks duration associated with fever, chills a non-productive cough, and hemoptysis. He was in moderate respiratory distress with respiratory rate of 40, temperature of 101.8 F (38.8 C), blood pressure of 100/60, heart rate of 100. Laboratory work up was remarkable for leukocytosis and proteinuria. A chest x-ray revealed a diffuse bilateral alveolar infiltrate (below left). His serum had high titers of anti-glomerular basement membrane (GBM) antibodies.
What does this patient have?

A

Goodpasture’s Syndrome
**confirmed by an immunofluorescence stain of kidney that showed anti-glomerular basement membrane antibodies. These types of antibodies against the alpha 3 chain of Type IV collagen are also found in alveolar capillaries

56
Q

What is hemoptysis?

A

spitting up blood

57
Q

What is usu the end result of good pasture’s syndrome?

A

glomerulonephritis

interstitial lung disease (necrotizing hemorrhagic interstitial pneumonitis)

58
Q

What is the treatment for good pasture’s syndrome? Is it curative?

A

Not curative. Once you have it-it will be a chronic condition.
plasmaphoresis to remove circulating antibodies. Also important–therapy to suppress the underlying immune system.

59
Q

What is the end result of the antagonist antibody seen in myasthenia gravis?

A

flaccid paralysis

60
Q

A 62-year-old woman was referred to a neurology clinic with a 1-month history of double vision, difficulty swallowing and weakness in her upper arms. These symptoms were mild or absent in the morning and tended to worsen through the day. When she was seen towards the end of an afternoon neurology clinic, she was found to have a bilateral ptosis and disconjugate eye movements. Her upper limb power was initially normal but deteriorated with repeated testing. An intravenous injection of edrophonium, a short-acting cholinesterase inhibitor, completely abolished the neurological signs but her eye movements deteriorated again 30 min after the injection.
What does this woman have?
What would blood testing show?

A

myasthenia gravis

blood testing would show–a high level of autoantibodies against the ACh receptor

61
Q

What is ptosis?

A

droopy eyes

62
Q

What is a good treatment for myasthenia gravis? Is it curative?

A

Not curative-this is a chronic condition.
Anti-cholinesterase inhibitors are good.
plasma phoresis helps.
immunosuppressant-corticosteroids

63
Q

A 41-year-old woman presents to her primary care physician with weight and hair loss, diarrhea, nervousness, and eye pressure. She has no medical problems, but some of her family members have similar symptoms and require medication. On examination, she is noted to have exophthalmos, an enlarged nontender goiter, hyperreflexia, and a tremor when her arms are outstretched. The patient has a decreased thyroid-stimulating hormone (TSH) level and an increased free thyroxine (T4) level.
What does this patient have? What is going on physiologically?

A

Grave’s Disease
breakdown in self-tolerance to thyroid autoantigens. antibodies that are agonists to the TSH receptor. IgG antibody does the stimulating.

64
Q

What are the pathology findings & lab findings in patients with Grave’s Disease?

A

Pathology includes diffuse hyperplasia of the thyroid, ophthalmopathy (exophthalmos), and dermopathy.
Laboratory findings in Graves disease include elevated free T4 and T3 levels and depressed TSH levels.

65
Q

Describe how the autoantibodies of a mom can affect a baby?

A

the autoantibodies can cross the placenta & cause the symptoms of the serious autoimmune disease of the parent. This only last as long as the antibodies last, though. Either 3-6 weeks to process them out naturally or plasmaphoresis–cured instantly!

66
Q

Neonatal lupus rash and/or congenital heart block involves which autoantibodies? What are the symptoms?

A

Autoantibodies: Anti-Rho, Anti-La antibodies
Symptoms: photosensitive rash or bradycardia

67
Q

Pemphigus vulgaris involves which autoantibodies in a neonate? What are the symptoms?

A

Autoantibodies: anti-desmoglein-3
Symptoms: blistering rash

68
Q

What is the best example of a Type III hypersensitivity autoimmune disease?

A

SLE–you make antibodies against your own DNA!!

69
Q

What is the genetic factor for SLE? How common is it in females? What age does it usu hit?

A

Genetic Factor: HLA-DR3

Common: 1/200 females of child-bearing age

70
Q

What is the usual manifestation of SLE? What is the usual cause of morbidity with SLE?

A

Manifestation: Photosensitive rash, polyarthritis, serositis, and fatigue are common manifestations of disease flares
Morbidity: Lupus nephritis and central nervous system involvement are important causes of morbidity

71
Q

What are some common markers for SLE?

A

Presence of antinuclear antibodies (ANA)
Certain autoantibodies, anti-double-stranded DNA (anti-dsDNA) and anti-Smith ribonucleoprotein (anti-Sm) have great specificity for the diagnosis of SLE, but lack sensitivity. Many false negatives. Some people think they are in the clear when they’re not.

72
Q

What is the difference b/w specificity & sensitivity?

A

Sensitivity: include some healthy people saying they have the disease. If you get a negative–you are def in the clear.
Specificity: exclude some sick people saying they don’t have the disease. If you get a positive–you are def sick.

73
Q

What is the relationship b/w a high or low level of complement & SLE?

A

Complement deficiency is an important risk factor for SLE

Hypocomplementemia may occur during flares

74
Q

Describe the pathogenesis of SLE.

A

You have susceptibility genes that gives you T cells & B cells that are reactive to self nuclear antigens.
Via normal apoptosis of dying cells or an increased load of nuclear antigens from UV radiation or something—you get…
**Antigen; Antinuclear-Antibody Complexes Formed
These bind to Fc receptors on B cells & Dendritic cells-Endocytosis!
TLR receptors inside the endosome prompt the B cells to make autoantibodies & the DC to make interferons.
More inflammation=More Apoptosis. Cyclical.
Persistent High Level of Antinuclear IgG antibody production.

75
Q

Why is it that the TLR receptors inside the endosomes can see the antigen?

A

b/c it is designed to see double-stranded DNA in viruses or bacteria. Here: it sees its own DNA!! Sad.

76
Q

Describe in more detail how these antibodies against our own DNA are first formed via linked recognition.

A
  • *T cells & B cells that recognize histone proteins escape central tolerance.
  • *B cell that recognizes self histone phagocytizes a nucleosome complex & presents histone peptide to the T cell. Inflammation.
  • *B cell that recognizes self DNA phagocytizes a nucleosome complex & presents histone peptide to the T cell. Linked Recognition. Inflammation.
77
Q

T/F Systemic lupus erythematosus is a heterogeneous disease that can affect virtually any organ system in variable ways.

A

True.

78
Q

T/F Renal Failure is a real possibility for pts with SLE.

A

True.

79
Q

Your patient is a 16-year-old girl who had enjoyed good health all her life. After spending several days in the sun at the beach, she developed a red rash on her cheeks. Her parents took her to the family doctor who immediately recognized the butterfly pattern of the rash as typical of systemic lupus erythematosus (SLE). She was referred to Children’s Hospital where a detailed history revealed that she had stiff finger and hip joints in the morning when she awakened.

What does this patient likely have?

A

SLE

80
Q

What are some tests that you might want to try?

A

A blood sample was taken from Nicole to ascertain if she had anti-nuclear antibodies (ANA). This was positive at a titer of 1:1280. Further tests revealed antibodies to double-stranded DNA, and her serum C3 level was 73 mg/dL (normal 100-200 mg/dL). Her Coombs tests were negative, and no protein was found in her urine.

81
Q

What is the proper treatment?

A

Nicole was sent home and advised to take the antimalarial agent Plaquenil (hydroxychloroquine sulfate) which sometimes has a beneficial effect on SLE. She was told to avoid direct sunlight. She did alright for a while, but in a month her symptoms were worse. She developed a fever of 39 ºC every morning and had shaking chills. Enlarged lymph nodes were felt behind her ears and in the back of her neck.
When she returned to Children’s Hospital a month later, her butterfly rash had disappeared, but she had diffuse swelling of the proximal joints in her fingers and toes. She had increased levels of anti-DNA antibodies and her serum C3 was 46 mg/dL.
Nicole was given 10 mg of the steroid prednisone twice a day, as well as the non-steroidal anti-inflammatory drug Naprosyn, 250 mg twice a day. This quickly controlled her symptoms and she remained well. At her next visit to the Children’s Hospital her serum C3 level was 120 mg/dL.

82
Q

What did the negative direct & indirect Coombs test tell you?

A

This pt doesn’t have some sort of blood disorder.

83
Q

T/F Antinuclear Antibody Patterns Are Used to Diagnose Various Autoimmune Diseases

A

True.

84
Q

SLE exhibits which antinuclear antibody patterns most commonly?

A

SLE: ANA, Anti-double stranded DNA, maybe Anti-histone, Anti-Smith. Nuclear RNP.

85
Q

Describe the process of cytokine-mediated inflammation in Type IV hypersensitivity.

A

APC presents self antigen. CD4 T cell connects & releases cytokines that produce inflammation & tissue injury. Otherwise healthy self tissue is damaged by inflammation.

86
Q

Describe the process of T cell-mediated cytolysis in type IV hypersensitivity.

A

APC presents tissue antigen. CD8 cell connects & has its inspiration. It then connects to this self antigen on other self tissues & kills them all.

87
Q

AUTOIMMUNE DISEASES MEDIATED BY T CELLS
Organ-specific autoimmune diseases
Give some examples.

A 
Type 1 diabetes mellitus
Multiple sclerosis 
Hashimoto thyroiditis 
Crohn disease 
Celiac disease
88
Q

AUTOIMMUNE DISEASES MEDIATED BY T CELLS
Systemic autoimmune diseases
Give some examples.

A

Rheumatoid arthritis
Systemic sclerosis
Sjőgren syndrome

89
Q

Describe in detail the pathogenesis of Multiple Sclerosis.

A

T cells with recognition of neuroantigens escape central tolerance.
There is a trigger for inflammation in the CNS that disrupts the BBB enough for these bad guy T cells to get in.
Microglial cells that happen to have bad MHCII molecules that recognize myelin basic protein are involved in this trigger inflammation & start bearing co-stimulatory molecules (B7). They happen to connect with the bad guy T cells in a perfect storm & you get T cells releasing mediators & demyelination of axons.

90
Q

A 29-year-old woman as in good health until one morning when she noticed a loss of vision in her left eye. Her physician referred her to a neurologist who diagnosed optic neuritis. She was given a 5-day course of IV corticosteroids and the neuritis cleared up. She was fine for 3 years then developed weakness of the muscles on the left side of her face. An MRI scan demonstrated multiple plaques in the white matter of the brain under the cortex and around the ventricles. Her CSF revealed discreet bands of IgG on electrophoresis.
What does this patient have? What are the frequently presenting symptoms of this condition?

A

Multiple Sclerosis. Visual compromise & stiffness & weakness. This is caused by plaques in CNS.

91
Q

What do the oligoclonal bands of IgG in the CSF indicate?

A

Clonal expansion of the B cell clones.

92
Q

What is the best treatment for multiple sclerosis?

A

IV corticosteroids, immunomodulating agents—alpha & beta interferons & glatiramer acetate.

93
Q

What is the prevalence of multiple sclerosis? Which HLA is it associated with?

A

Prevalence is 1 in 700, 10X more frequent in women, and associated with HLA-DR2

94
Q

What are the 3 possible routes of the pathogenesis in Hashimoto thyroiditis?

A
  1. T cell mediated cytotoxicity: CD8 T cell binds to thyroid epithelium.
  2. Thyrocyte Injury: CD4 T cell releases IFN gamma & activates macrophages to give the thyroid epithelium a hard time.
  3. Antibody-dependent cell-mediated cytotoxicity: Plasma cells release anti-thyroid antibodies. These connect to the thyroid epithelium & to the Fc receptor of an NK cell. Trouble in paradise.
95
Q

A 45-year-old woman presents complaining of fatigue, 30 pounds of weight gain despite dieting. On physical examination, the thyroid is not palpable; the skin is cool, dry, and rough; the heart sounds are quiet; and the pulse rate is 50 beats/min. Rectal and pelvic examinations show no abnormalities, and the stool is negative for occult blood. Laboratory tests reveal antibodies to both thyroglobulin and thyroid peroxidase.
What diagnosis does this suggest? What is the prevalence of this condition?

A

Hashimoto Thyroiditis

Prevalence=1/200.

96
Q

What is the basic clinicopathology of hashimoto’s?

A

Loss of self-tolerance to thyroid auto-antigens with the presence of circulating autoantibodies against thyroglobulin and thyroid peroxidase

97
Q

What happens to your hormone levels in Hashimoto’s?

A

As hypothyroidism develops, the thyroid shrinks and T4 and T3 levels fall accompanied by a compensatory increase in TSH

98
Q

Hashimoto’s puts you at increased risk for developing other autoimmune diseases, including ______.

A

B-cell non-Hodgkin lymphomas (e.g. MALT lymphomas)

99
Q

What is systemic sclerosis?

A

Systemic sclerosis (SS) is characterized by excessive fibrosis in a variety of tissues from collagen deposition by activated fibroblasts.

100
Q

What is Crest Syndrome? What is another name for it?

A

Limited scleroderma or CREST syndrome is the benign form of SS, serologically suggested by the presence of anti-centromere antibody
C = Calcinosis in skin and elsewhere
R = Raynaud’s phenomenon, sensitivity to cold
E = Esophageal dysmotility from submucosal fibrosis
S = Sclerodactyly from dermal fibrosis
T = Telangiectasias

101
Q

Anti-DNA topoisomerase I antibody (SCL-70) shows specificity for which form of scleroderma? How severe is this form?

A

Worst form! Diffuse scleroderma
scleroderma presents with CREST findings and arterial intimal thickening and proliferation (hyperplastic arteriolosclerosis, “onion skin” lesion) leading to malignant hypertension with arterial fibrinoid necrosis, thrombosis, and renal infarction
Half of diffuse SS patients die from renal disease

102
Q

A 52-year-old woman presented to her primary care physician for color changes of her fingers. She said that in cold weather her digits turn white, then bluish, and then a tingling bright red. He diagnoses Raynaud’s phenomenon and she is instructed to avoid cold environments and to use heat when the symptoms are severe. She returns returns a year later with puffiness of her hands and skin tightness of her fingers, and she has had difficulty putting on and taking off her rings. She has noted difficulty with eating large meals (dysphagia), causing her bloating, abdominal pain, and increased symptoms of gastroesophageal reflux. Head and neck examination reveals slightly dry oral mucosa, and she has fissuring of the skin around her mouth. Musculoskeletal examination reveals skin sclerosis from the metacarpophalangeal joints to the tips of the fingers, with flexion deformities of all of the fingers and telangiectasias observed in the palmar aspect of both hands. She is unable to fully flex her fingers to make a fist.
What does this patient likely have? How common is this condition in women? What age group often gets it? What are its distinctive features?

A

Systemic sclerosis is a rare disorder with a female-to-male ratio of 3 :1
Peak incidence in the 50- to 60-year age group.
Its distinctive features are the striking skin fibrosis, but the gastrointestinal tract, kidneys, heart, muscles, and lungs also are frequently involved

103
Q

Raynaud’s phenomenon is seen in scleroderma. What is this?

A

Raynaud’s phenomenon involves episodic vasoconstriction of the arteries and arterioles of the extremities; it is seen in virtually all patients and precedes other symptoms in 70% of cases

104
Q

What is dysphagia attributable to in patients with scleroderma? What percentage of these patients experience dysphagia?

A

Dysphagia attributable to esophageal fibrosis and its resultant hypomotility are present in more than 50% of patients

105
Q

SLE + Rheumatoid Arthritis=?

A

Sjogren

106
Q

What is sicca syndrome?

A

A decrease in tears & saliva

107
Q

What causes sicca syndrome in Sjogren patients?

A

Lymphocytic infiltration & fibrosis of the lacrimal gland & salivary glands. Get a lot of inflammation, tissue damage, & fibrosis.

108
Q

What could be the initial trigger in Sjogren patients?

A

The initiating trigger may be a viral infection of the salivary glands, which causes local cell death and release of tissue self-antigens
Note: The person may also be genetically susceptible w/ CD4 & B cells that recognize self antigens & have escaped central tolerance.

109
Q

What antibodies are present in Sjogren patients?

A

Rheumatoid factor, ribonucleoproteins: SS-A (Ro) & SS-B (La)

110
Q

A 58-year-old woman comes to her primary care physician with a complaint of dry eyes and mouth that has been getting progressively worse over the last few months. She also notes some loss of smell and taste. Examination reveals enlargement of the parotid glands. Laboratory tests reveal some proteinuria, rheumatoid factor, and antibodies to SS-A(Ro) and SS-B (La) antigens. A lip biopsy is performed which shows lymphoid cell inflitration of the minor salivary glands. A Schirmer test is performed which shows a significant reduction in tear formation.
What does this patient have? What is the prevalence of this condition? Is it more common in men or women? Age?

A

Sjogren Syndrome
Prevalence = 1 in 300
Female:male ratio of 9:1, with greatest incidence in 40-60 year-old females

111
Q

What is the usual clinical presentation of Sjogren?

A

Clinical presentation dominated by dryness of eyes (xerophthalmia) and mouth (xerostomia) caused by T cell-mediated destruction of lacrimal and salivary glands

112
Q

T/F Sjogren always occurs on its own. It is never accompanied by other autoimmune diseases.

A

False. Can occur in isolation (primary Sjőgren syndrome) or in association with other autoimmune disorders like rheumatoid arthritis

113
Q

What does the Schirmer test measure?

A

Quantity of tears secreted

114
Q

What is the treatment?

A

Drugs to ameliorate dryness (pilocarpine) and symptomatic treatment (artificial tears)
& artifical saliva!!

Block 6; Week 2-Michelle (8 decks)

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