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Immunologic Disease Flashcards

1
Q

What mediates anaphylaxis?

A

IgE

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2
Q

How do you dx anaphylaxis?

A

Hx of exposure
Rapid onset
Serum tryptase @ 1, 4, 8 hours

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3
Q

How do you treat anaphylaxis?

A 
ABC
100% O2
Epi (1:1000 - 0.01 ml/kg) 0.1 mL - 0.3 mL
Diphenhydramine
Cimetidine
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4
Q

What are risk factors for severe anaphylactic reaction

A

Asthma
Beta-blockade
Adrenal Insufficiency

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5
Q

What is the most common cause of urticarial in children?

A

Viral infections

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6
Q

What causes hives? (6)

A 
Viruses & Bacteria
Medications
Foods
Insect stings
Autoimmune diseases
Malignancies
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7
Q

What type of hypersensitivity reaction is hives?

A

Type 1

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8
Q

What type of hypersensitivity reaction is serum sickness?

A

Type 3

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9
Q

What can cause serum sickness

A 
Anti-gout: allopurinol
Antimicrobials: PCN, ceph
Antiarrhythmic: quinidine, procainamide
Antihypertensive: captopril, hydralazine
Thyroid Meds
Serum blood products/vaccines
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10
Q

What are s/sx of serum sickness

A

6-21 days post exposure
Fever, joint pain, LAD, edema
Red, purpuric rash at junction of palmar or plantar skin
+/- renal/CV/pulm/neuro compx

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11
Q

How do you treat serum sickness

A

Symptoms usually spontaneously resolve

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12
Q

What type hypersensitivity is a drug reaction

A

All Types!

`

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13
Q

Type 1 Hypersensitivity is mediated by what?

A

IgE!!!

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14
Q

What is type 3 hypersensitivity mediated by?

A

Immune complex = Serum Sickness

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15
Q

What types of hypersensitivity reactions can penicillin cause?

A

Types 1-3

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16
Q

Ampicillin rash

A

Not urticarial!

Seen with Mono and Hyperuricemia

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17
Q

True or False: Most adverse food reactions have an immunologic basis?

A

False - most are intolerances i.e. N/V/D

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18
Q

The following is a classic triad of what disease:

  • Conjunctivitis
  • Oral ulceration
  • Urethritis
A

Stevens-Johnson Syndrome

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19
Q

What causes SJS?

A

Drugs: Sulfonamides and anticonvulsants

Mycoplasma pneumoniae

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20
Q

What is the prodromal phase of SJS?

A

1-14 days: Fever, headache, and malaise

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21
Q

What does the rash of SJS look like?

A

Red blistering target lesions

Inflamed bullous lesions

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22
Q

What is the clinical criteria for SJS

A

Cutaneous lesion + 2 mucosal surfaces

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23
Q

How do you diagnose SJS? What do you expect to find?

A

Skin biopsy

Perivascular mononuclear cell infiltrate

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24
Q

How do you treat SJS?

A

Hospital
IVF
Hyperalimentation i.e. TPN
Skin Care

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25
Hyperalimentation
TPN
26
Left shift
Neutrophilic predominance
27
A 10 month old male infant presents with oral candidiasis. What should you consider?
Immune deficiency
28
What is the defect in SCID?
KO Humoral & Cellular immunity | Dec. adenosine deaminase --> defect in stem cell maturation
29
How do you dx SCID? What else might you see?
Lymphopenia + dec. serum IgG, IgA, IgM * Absent thymus * *Frequent and severe infections, FTT
30
What are 2 T-cell deficiencies
DiGeorge Syndrome | Chronic Mucocutaneous Candidiasis
31
What are 3 humoral deficiencies?
IgA Deficinecy Brutons agammaglobulinemia CVID
32
What are 3 diseases with deficient phagocytosis?
Chronic granulomatous disease Chediak-Higashi Syndrome Job Syndrome
33
What do you get with a 22, q 11 deletion
DiGeorge Syndrome
34
What two diseases have no thymic shadow?
SCID, DiGeorge Syndrome
35
What structures are affected in DiGeorge?
``` 3rd and 4th brachial pouches Thymus - absent T cells Parathyroid - hypoCa tetany Esophageal atresia R. sided aortic arch, ASD/VSD ```
36
How do you treat DiGeorge?
Thymic tissue transplant BMT - at risk for GVDH **Irradiated blood products only**
37
Recurrent/persistent candidal infections of skin, nails and mucous membranes
Chronic Mucocutaneous Candidiasis | -Hypo PTH, hyper thyroid
38
Selective IgA Deficiency S/Sx (4)
RTI, UTI, GI infections - giardia | AI diseases
39
What 2 things are contraindicated in someone with Selective IgA deficiency?
Blood products and IVIg
40
How do you dx IgA deficiency
IgA less than 5 mg/dL
41
What is defective in Brutons Agammaglobulinemia?
Protein tyrosine kinase on B cell (Xq22) | X-linked
42
What types of infections are those with Brutons agammaglobulinemia more likely to get?
Strep H flu Echovirus meningoencephalitis **Small tonsils, adenoids and LN**
43
CVID
Hypogammaglobulinemia, less severe than SCID thymoma alopecia areata pernicious anemia non-caseating sarcoid-like granulomas of lungs, spleen, skin and liver
44
How do you diagnose chronic granulomatous disease?
``` Leukocytosis Inc. ESR Abnormal CXR Hypergammaglobulinemia Leukocytes unable to reduce nitroblue tetrazolium ```
45
What are the s/sx of chronic granulomatous disease?
Severe recurrent skin and LN infections Pneumonitis -> pneumatocele Osteomyelitis HSmegaly
46
What are the symptoms of Chediak Higashi Syndrome (4)
Skin and respiratory infection Albinism Pancytopenia Progressive peripheral neuropathy
47
How do you diagnose Chediak Higashi?
Large inclusions in all nucleated cells
48
How do you treat Chediak Higashi? (3)
High-dose ascorbic acid BMT - doesn't treat/prevent neuropathy Abx
49
What is the defect in Job Syndrome? How do you diagnose it
Neutrophil chemotactic defect | IgE > 10,000 and eosinophilia
50
Recurrent Staph infections, resistant to therapy Pruritic eczematoid dermatitis Coarse facial features
Job Syndrome (Hyper IgE)
51
How do you treat Job Syndrome
Penicillinase-resistant abx | IVIG
52
Ataxia telangiectasia: What are the 3 main symptoms
Telangiectasia Ataxia Variable Immunodeficiency
53
When does Ataxia Telangiectasia present?
First 6 years Wheelchair by 10-12 years *Inc. risk of malignancy
54
What lab abnormalities are seen in Ataxia Telangiectasia?
Inc. serum AFP | Inc. CEA
55
How do you treat Ataxia Telangiectasia?
Supportive - pulmonary fx and immunologic status
56
What disease has the triad of (male) Thrombocytopenia Eczema Recurrent bacterial infections
Wiskott-Aldrich syndrome
57
What is wrong in Wiskott-Aldrich? What labs do you see?
Can't form antibodies to bacterial capsular polysaccharide antigens Low IgM High IgA
58
Excessive bleeding from circumcision site can be an indicator for what disease?
Wiskott-Aldrich - thrombocytopenia
59
In whom do you see hypocomplimentemia?
Lupus nephritis Post strep GN **not HSP or minimal change**
60
C1q is deficient in ____.
SLE
61
C1r, C1r/C1s, C4, C2 and C3 deficiency
Vasculitis - SLE
62
C5-C8 deficiency
Neisserial infection
63
C3 deficiency
Pneumococci
64
C1 esterase inhibitor deficiency
Hereditary angioedema
65
What bugs can pts with chronic granulomatous disease not kill?
``` Catalase positive (SPACE) Staph, Pseudomonas, Aspergilus, Candida, Enterobacteriaciae (klebsiella, seratia ) ```
66
What diseases can cause hyposplenia?
SLE, RA, IBD, GVDH, nephrotic syndrome | Sickle cell or hemoglobin-opathy
67
What are asplenic individuals more susceptible to?
``` Encapsulated organisms S neuro H flu Neisseria E coli Salmonella Klebsiella ```
68
What s/sx occur with acute GVHD? (4)
``` 5-40 days Erythroderma Cholestatic hepatitis - abnormal LFTs Diarrhea Inc. susceptibility to infections ```
69
What s/sx occur with chronic GVHD? (5)
``` Sjogrens SLE Scleroderma Lichen planus Primary biliary cirrhosis Infections ```

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