Clinical Micro - Autoimmunity

Front 1

Anklosing spondylitis, acute anterior uveitis are all associated with what MHC?

Back 1

HLA type 1, B27

Front 2

Good pastures, MS are all associated with what MHC?

Back 2

HLA-DR2

Front 3

Graves and Myasthenia Gravis are all associated with what MHC?

Back 3

HLA-DR3

also DM and SLE

Front 4

RA, Pemphigus Vulgaris are all associated with what MHC?

Back 4

HLA - DR4

(So is DM)

Front 5

Hashimoto’s thyroiditis IS associated with what MHC?

Back 5

HLA DR5

Front 6

HLA - DR4 is associated with what autoimmune disorders?

Back 6

DM, RA, Pemphigus Vulgaris

Front 7

HLA DR5 is associated with what autoimmune disorders?

Back 7

Hashimotos thyroiditis

Front 8

HLA DR3 is associated with what autoimmune disorders?

Back 8

Graves, SLE, DM, myasthenia gravis

Front 9

HLA DR2 is associated with what autoimmune disorders?

Back 9

Good pastures, MS

Front 10

HLA B27 is associated with what autoimmune disorders?

Back 10

Ankylosing spondylitis

Front 11

Coxsackie virus is associated with what autoimmune disorders?

Back 11

Diabetes Mellitus

Front 12

Group A streptococcus is associated with what autoimmune disorders?

Back 12

Rheumatic fever

Front 13

If someone has a defective AIRE, they will develop an autoimmune disease called _

What is the general population that this is seen in, and what are some of the primary clinical features?

Back 13

Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy (APECED).

Finnish, Sardinians, Iranian Jews. They will have mutliple endocrine disorders and Candida Infections. Possibly dental enamel hypoplasia or dystriohic fingernails

Front 14

In absence of _, a peripheral T cell will go into anergy

Back 14

CD28/B7 interaction (secondary signal after T cell recognized an antigen in context of MHC)

Front 15

CD4+/CD25+ cells are aka _

Back 15

T regulator cells

they are upregulated in normal conditions when the body wants to stop an immune response. It suppresses the adjacent T cells via IL10 or TGFbeta

Front 16

The gene that is required to make regulatory T cells (aka CD25/CD4)?

What happens when there is a mutation in this gene?

Back 16

Foxp3

IPEX Immune Dysregulation Polyendocrinopathy Enteropathy, (x linked)

Front 17

Patients who have a mutation in the Fas gene develop a condition called _

What is the primary clinical presentation?

Back 17

Autoimmune lymphoproliferative syndrome (ALPS)

Enlarged cervical and supraclavicular lymp nodes in absence of an infection. (Can't kill off the lymphocytes, so population increases)

Front 18

Sympathetic Opthalmia is a classic example of _

Back 18

antigen sequestration


this is where trauma to one eye leaks "immune protected proteins" leak into lymph and then the body develops an immune rxn to both eyes

Front 19

_ in B cells is comparable to negative selection in T cells

Back 19

Clonal deletion

Front 20

An 18 yr boy presents with cough and hemoptysis. Vitals are taken and show a BP of 160/100. A UA shows red blood cells. If you were to take a biopsy of his kidney, what would it show on immunofluroescence?

Back 20

linear (smooth) deposition of IgG antibodies on the glomerular basement membrane.

(this is talking about goodpasture syndrome - where ab are directed toward noncollagenous domain of collagen IV)

Front 21

An 18 yr boy presents with cough and hemoptysis. Vitals are taken and show a BP of 160/100. A UA shows red blood cells.What diagnostic lab test would be + in this patient?

What is the antigen in this case?

Back 21

antibasement antibodies


the noncollagenous domain of collagen IV (type II hs)

Front 22

Pemphigus Vulgaris is an autoimmune blistering disease resulting from autoantibodies directed at _

Back 22

the epidermal desmosomes (specifically desmoglein 3)

these start in the oral mucosa and then appear on the face, scalp, axilla, trunk and groin.

Front 23

50 yo woman presents with blisters in her armpit, trunk and groin. Hx reveals difficulty eating for the previous week before these blisters showed due to sores in her mouth. What is your suspected diagnosis?

Back 23

Pemphigus Vulgaris (ab directed at desmoglein 3)

Front 24

50 yo woman presents with blisters in her armpit, trunk and groin. Hx reveals difficulty eating for the previous week before these blisters showed due to sores in her mouth. If you were to bx one of the lesions, what would you see in histology? Immunflurescence?

Back 24

acantholysis (separation of keratinocytes - polyhedral cells)

Fish net-like pattern

Front 25

You are volunteering abroad in Brazil and your patient comes in with crusted lesions over her trunk and groin, a few lesions are tiny bullae. There is no oral involvement. After a bx you suspect an autoimmune disease where antibodies are directed against desmoglein 1. What other condition is associated with this?

Back 25

Myasthenia Gravis


This ? is describing pemphigus foliaceus

Front 26

22 yo female presents with heat intolerance, increased appetite, and 'heart racing'. She also claims that despite eating so much, she's actually lost a few pounds. What do you suspect?

Back 26

Hyperthyroid State

(Possibly Graves, but should run some tests)

Front 27

22 yo female presents with heat intolerance, increased appetite, and 'heart racing'. She also claims that despite eating so much, she's actually lost a few pounds. Lab results show increased T3/T4, and decreased TSH. If you were to biopsy her thyroid, what would you see?

Back 27

prominent infoldings of the hyperplastic epithelium and scalloping of the epithelium
Intact architecture

(Grave's Disease)

Front 28

35 yo female presents with heat intolerance, increased appetite, and 'heart racing'. She also claims that despite eating so much, she's actually lost a few pounds. On PE you note that she has bulging eyes. Lab results show increased T3/T4, and decreased TSH. What is the pathophys?

Back 28

antibodies bind to and activate the TSH receptors, leading to a hyperthyroid state.

(Grave's)

Front 29

22 yo female presents with heat intolerance, increased appetite, and 'heart racing'. She also claims that despite eating so much, she's actually lost a few pounds. On PE you note that she has bulging eyes. Lab results show increased T3/T4, and decreased TSH. What could you find on auscultation of her heart that fits in with case?

Back 29

functional pansystolic murmur (no defect in the valve but d/t hyperdynamic circulation)

(Grave's)

Front 30

35 yo female presents with heat intolerance, increased appetite, and 'heart racing'. She also claims that despite eating so much, she's actually lost a few pounds. what lab results would you expect to find?

Back 30

Increased T3/T4, decreased TSH

TSH-R antibody

Possible ANA, antithyroperoxidase ab, and antithyroglobulin antibody (although these are also seen in Hashimotos)

Front 31

35 yo female presents with heat intolerance, increased appetite, and 'heart racing'. She also claims that despite eating so much, she's actually lost a few pounds. How would you treat this?

Back 31

Propanolol (sx relief), thyourea drugs (not given for more than 1-2 yrs)

(hyperthyroid...graves)

Front 32

In _, the architecture of the thyroid gland is destroyed, whereas in _ the architecture is preserved

Back 32

Hashimotos thyroiditis

Graves disease

Front 33

A 62-year-old woman complains of chronic constipation and progressive weight gain over the past 6 months despite maintaining a pure vegan diet. She takes no prescription or over-the-counter medications. Other findings include a symmetrically enlarged, nontender thyroid gland, delayed deep tendon reflexes, and proximal muscle weakness in the lower extremities. The serum creatine kinase level is 350 U/L. What set of thyroid function studies is most likely present?

Back 33

Decreased T3/T4, Increased TSH


(Hypothyroidism, probably dt Hashimotos)

Front 34

A 40-year-old woman consulted her primary-care physician because of “fatigue”. She felt that she had been “losing energy” over the past several months. She had gained some weight, about 10 lb.. Her physical examination was normal, other than slight thyroid tenderness. The thyroid gland did not feel enlarged. Serum thryoxin (T4) was low, and serum thyroid-stimulating hormone (TSH) was markedly elevated. What type of rxn is this?

Back 34

Type II hypersensitivity

(Hashimotos thyroiditis)

Front 35

35 yo female presents with constipation, weight gain, hair loss, and fatigue. Lab shows normal thyroxine (T4) and thyroid-stimulating hormone (TSH) levels, and high titers of thyroid peroxidase antibodies. What is the dx?

Back 35

Hashimotos Thyroiditis

Early testing in hashimotos may return with normal T3 and TSH, but later in the course these would be decreased and increased respectively

Front 36

40 yo woman complains of progressive weakness throughout the day. She has droopy eyelids, weakness of facial muscles, and also complains of double vision. What diagnostic test would be most useful to you?

Back 36

Edrophonium

(Myasthenia gravis)

Front 37

40 yo woman complains of progressive weakness throughout the day. She has droopy eyelids, weakness of facial muscles, and also complains of double vision. After a dose of Edrophonium, she improves. What is the treatment for this condition?

Back 37

Pyridostigmine (AChEI) - 1st line

(Myasthenia gravis)

Front 38

2 antibodies specific for SLE include _

Back 38

dsDNA
Smith Ab

(ANA is often positive but not specific)

Front 39

What antibodies will you see in subacute cutaneous lupus?

What about drug induced lupus?

Back 39

SSA (a/w HLA DR3)

Anti-histone antibody (HLA DR4)

Front 40

Patient presents with weight loss, fever, and malaise. She has no cough or difficulty breathing. She also complains of muscle pain, and abdominal pain.
Lab shows antineutrophil cytoplasmic antibodies, what part of the artery is most likely affected?

Back 40

Transmural

(this is describing polyarteritis nodosa - PANCA, no lung involvement)

Front 41

In a case of thyrotoxicosis which lab or study will help differentiate Graves dz from toxic adenoma OR toxic multinodular goiter?

Back 41

Thyroid Scan

Front 42

HLA DQ2 and HLA DQ8 are associated with what autoimmune disease?

What is the typical presentation of this disease?

What other conditions are these patients at risk for?

Back 42

Celiac disease (autoreactive T cell response damaging gut epithelium)

Diarrhea, flatulence, weight loss, anemia, fatigue

Non Hodgkins lymphoma, adenocarcinoma of small intesteine, and squamous cell carcinoma of the esophagus

Front 43

CREST syndrome is associated with what condition

What is the 'giveaway' lab result?

Back 43

Limited Scleroderma

Anticentromere Ab


CREST stands for Calcinosis Raynaud phenomenon Esophageal dysmotility Sclerodactylyl Telangiectasia

Front 44

Anticentromere antibody is associated with what condition?

Back 44

Limited Scleroderma


(Antitopoisomerase is associated with Diffuse)

Front 45

Reiter's Syndrome is associated with HLA _

Back 45

B27

Front 46

40 yo woman presents with ulcerations on the tips of her fingers. She also claims that she has difficulty swallowing lately and has heart burn. PE shows perioral constriction, and smooth shiny skin, and a BP of 156/90. UA is taken and shows mild proteinuria. What do you suspect?
What lab results would you expect to come back +?

What type of rxn is this?

Back 46

Limited Scleroderma

+ ANA, + Anticentromere

(ps this is a CD4 T cell mediated rxn)

Front 47

T regulator cells (CD4CD25) bind to _ on the APC as opposed to _. It is this binding that sends a negative signal telling the immune response to stop.

Back 47

CTLA-4

B7

(the binding of the CTLA-4 to the T regulatory cell triggers release of IL10 and TGFb both of which are antiinflammatory)

Front 48

Kidney biopsy reveals thickened glomerular basement membrane and glomerular hypercellularity (particularly mesangial cells).

What is this diagnostic of?

Back 48

Systemic Lupus Erythematosis

This is the classic description given when reviewing a renal bx on a lupus patient

Front 49

Patient presents to your office complaining of a severe sunburn on her face after spending the previous afternoon playing beach volleyball. Physical exam shows a malar rash on face. What lab result would be diagnostic for your suspicion?

Back 49

ds DNA (or anti smith)

This is talking about SLE

Front 50

What are the 4 drugs that can cause drug induced lupus?

What is the antibody present in these patients?

Is there a genetic component?

Back 50

Hydralazine, Procainamide, D-penicillamine, Isoniazid
(Hawaii PD)

Anti HIstone Antibody

HLA DR4

Front 51

What is the antigen to which T cells react in multiple sclerosis?

Back 51

Myelin basic protein, proteolipid protein, myelin oligodendrocyte glycoprotein

Front 52

Patients with Sjorgren's syndrome are at risk for developing what?

Back 52

lymphoid malignancies

Front 53

What is the possible antigen recognized by T cells in Sjorgrens syndrome

Back 53

alpha fodrin (a cytoskeletal protein)