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Pediatrics > Immunology/Rheumatology > Flashcards

Immunology/Rheumatology Flashcards

1
Q

Types of infections with Ig deficiencies

A

Sinupulmonary infections with encapsulated bacteria (usually extracellular pathogens)

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2
Q

Most common type of immunodeficiency

A

B-cell of Ig

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3
Q

Examples of B-cell/Ig immunodeficiency

A 
X-linked agammaglobulinemia (Bruton's)
Common variable (CVID)
Selective IgA 
IgG subclass
X-linked hyper-IgM
Transient hypogammaglobulinemia of infancy
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4
Q

Bruton’s

A

X-linked agammaglobulinemia

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5
Q

Bruton’s findings

A 
Recurrent severe pyogenic infections
Persistent meningoencephalitis (enteroviruses)
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6
Q

Bruton’s diagnosis

A

Low levels of all Ig types, frew B cells

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7
Q

Bruton’s genetics

A

X-linked

Bruton tyrosine kinase

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8
Q

CVID presentation

A

Later in life, similar infections to Bruton’s but less severe

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9
Q

CVID associations

A

Autoimmune disease - hemolytic anemia, alopecia, arthritis…

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10
Q

Most common inherited immunodeficiency

A

Selective IgA

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11
Q

Selective IgA findings

A

Chronic sinupulmonary infections caused by pyogenic bacteria but less severe than Bruton’s or CVID

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12
Q

IgG subclass diagnosis

A

Antibody response may be impaired despite normal Ig level and IgG subclass

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13
Q

X-linked hyper-IgM cause

A

Defect in CD40 ligand (2/2 congenital rubella?)

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14
Q

Examples of T-cell immunodeficiency

A 
DiGeorge syndrome
SCID
Wiskott-Aldrich
Ataxia-telangiectasia
Hyper-IgE syndrome
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15
Q

Types of infections with T-cell deficiency

A

Intracellular pathogens - viruses, fungi, protozoa. Diarrhea and growth retardation common due to frequent GI infections.

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16
Q

Severe combined immunodeficiency (SCID) features

A

Lack of cell-mediated and Ab immunity
Susceptible to all infectious agents - diarrhea, candida, CMV, PCP…
Most patients die w/in 2 years

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17
Q

SCID defect

A

Adenosine deaminase deficiency

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18
Q

Wiskott-Aldrich genetics

A

X-linked

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19
Q

Wiskott-Aldrich findings

A

Eczema, thrombocytopenic purport, recurrent infections of all types

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20
Q

PI associated with lymphoreticular malignancy

A

Wiskott-Aldrich

Ataxia-telangiectasia

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21
Q

Ataxia-telangiectasia genetics

A

AR d/o of chromosome repair

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22
Q

Hyper-IgE syndrome findings

A

Recurrent skin abscesses, dental abnormalities, sinupulmonary infections, chronic dermatitis, coarse facies

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23
Q

Phagocyte disorders

A

Chronic granulomatous disease (GCD)
Chediak-Higashi syndrome
Leukocyte adhesion deficiency

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24
Q

Phagocyte disorder findings

A

Recurrent infections of skin, respiratory tract and deep tissues; abscesses; staphylococcci infection; oral ulcerations and perirectal abscesses

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25
Q

CGD genetics

A

X-linked and AR
Defect in P-450 or NADPH oxidase system
Inability to kill cells that produce catalase (ie staph)

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26
Q

CGD treatment

A

interferon gamma; Bactrim prophylaxis

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27
Q

Nitroblue tetrazolium dye reduction test (NBT)

A

Measures oxidative burst (frequently used to diagnosis CGD)

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28
Q

Chediak-Higashi syndrome findings

A

Recurrent staph infections, partila oculocutaneous albinism

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29
Q

Most common complement defect

A

C2

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30
Q

Complement deficiency disorder findings

A 
Classical pathway (C1,C2,C4) - autoimmune, sinupulmonary infections
Alternative pathway (C3) - recurrent pyogenic infections
Membrane attack complex (C5-C9) - Neisseria
31
Q

Diagnosis of defects in cell-mediated immunity

A

skin testing (pt > 24 months), T-cell subsets

32
Q

CH50 test

A

Detects disorders of proteins in the cascade from C1 to C9

33
Q

Diagnosis of JIA

A

onset < 16 yo, presence of arthritis, duration > 6 weeks

34
Q

Chronic arthritis more common in boys

A

enthesitis-related

35
Q

Risk for uveitis

A

pauciarticular, polyarticular RF-, enthesitis-related

36
Q

Highest risk for uveitis

A

girls, 1-3 yo, pauciarticular, ANA+

37
Q

Felty syndrome

A

splenomegaly and leukopenia

38
Q

Chronic arthritis with risk of atlantoaxial subluxation

A

polyarticular

39
Q

Rash in systemic JIA

A

salmon-pink macular, Koebner phenomena

40
Q

Findings in psoriatic arthritis

A

nail pitting, onycholysis, dactylitis (sausage digit)

41
Q

Biologic agents for systemic JIA

A

anti-IL1, anti-IL6

42
Q

Triggers for reactive arthritis

A

GI (shigella, salmonella, campylobacter, yersinia), GU (chlamydia)

43
Q

Drug-induced lupus

A

anti-convulsants, hydralazine, isoniazid, procainamide, minocycline

44
Q

Highest prevalence of SLE in US

A

African-American females

45
Q

Hemolytic manifestations of SLE

A

hemolytic anemia, thrombocytopenia, lymphopenia, anti-phosopholipid antibodies

46
Q

SLE renal disease class I

A

normal/minimal change

47
Q

SLE renal disease class II

A

mesangial nephritis, no progression

48
Q

SLE renal disease class III

A

focal segmental proliferative GN, more serious

49
Q

SLE renal disease class IV

A

diffuse proliferative GN, most severe, low C3, elevated anti-dsDNA

50
Q

SLE renal disease class V

A

membranous GN

51
Q

SLE renal disease class VI

A

glomerular sclerosis, end-stage

52
Q

anti-dsDNA

A

present in active renal dz, pathognomonic for SLE

53
Q

anti-SM

A

SLE, CNS dz

54
Q

anti-RNP

A

mixed CT dz

55
Q

SLE treatment

A

NSAIDS (no ibuprofen - aseptic meningitis), steroids, hydroxychloroquine, azathioprine, cyclophosphamide, MMF

56
Q

Leading cause of death in SLE

A

sepsis

57
Q

HSP peak incidence

A

4-7 years

58
Q

Most specific finding of dermatomyositis

A

Gottron papules

59
Q

Diagnosis of dermatomyositis

A

CK, LDH, LFT’s, MRI, muscle biopsy (gold standard)

60
Q

Most common finding in infants of mothers with SLE

A

No findings!

61
Q

Most common finding in neonatal lupus

A

transient photosensitivity rash

62
Q

Treatment of heart block in neonatal lupus

A

pacemaker

63
Q

PFAPA syndrome

A

periodic fever, aphthous stomatitis, pharyngitis, adenopathy (elevated ESR/CRP)

64
Q

Management of PFAPA

A

T&A

65
Q

Treatment of juvenile fibromyalgia

A

PT, CBT, improved sleep, amitripytline

66
Q

Takayasu’s arteritis findings

A

stenosis of aorta and branch arteries

67
Q

Rate of extra-cutaneous manifestations in linear scleroderma

A

20%

68
Q

Wegener’s granulomatosis findings

A

sinusitis, hemopytisis, GN w/ hematuria

69
Q

Wegener’s diagnosis

A

c-ANCA, anti-proteinase-3 IgG

70
Q

Findings that indicate underlying rheumatologic condition in Raynaud’s

A

+ANA, abnormal nail fold capillaries

71
Q

Rash associated with sarcoid

A

erythema nodosum

72
Q

Diagnosis of sarcoid

A

elevated ACE (60%) - less common in african americans, biopsy w/ noncaseating granuloma

73
Q

Familial Mediterranean fever presentation

A

attacks of fever, abdominal pain and arthritis

74
Q

FMF natural history

A

amyloidosis leading to renal failure (reduced by use of colchicine)

Pediatrics (14 decks)

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