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Immunology/Rheumatology Flashcards

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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
CGD genetics
X-linked and AR Defect in P-450 or NADPH oxidase system Inability to kill cells that produce catalase (ie staph)
26
CGD treatment
interferon gamma; Bactrim prophylaxis
27
Nitroblue tetrazolium dye reduction test (NBT)
Measures oxidative burst (frequently used to diagnosis CGD)
28
Chediak-Higashi syndrome findings
Recurrent staph infections, partila oculocutaneous albinism
29
Most common complement defect
C2
30
Complement deficiency disorder findings
``` Classical pathway (C1,C2,C4) - autoimmune, sinupulmonary infections Alternative pathway (C3) - recurrent pyogenic infections Membrane attack complex (C5-C9) - Neisseria ```
31
Diagnosis of defects in cell-mediated immunity
skin testing (pt > 24 months), T-cell subsets
32
CH50 test
Detects disorders of proteins in the cascade from C1 to C9
33
Diagnosis of JIA
onset < 16 yo, presence of arthritis, duration > 6 weeks
34
Chronic arthritis more common in boys
enthesitis-related
35
Risk for uveitis
pauciarticular, polyarticular RF-, enthesitis-related
36
Highest risk for uveitis
girls, 1-3 yo, pauciarticular, ANA+
37
Felty syndrome
splenomegaly and leukopenia
38
Chronic arthritis with risk of atlantoaxial subluxation
polyarticular
39
Rash in systemic JIA
salmon-pink macular, Koebner phenomena
40
Findings in psoriatic arthritis
nail pitting, onycholysis, dactylitis (sausage digit)
41
Biologic agents for systemic JIA
anti-IL1, anti-IL6
42
Triggers for reactive arthritis
GI (shigella, salmonella, campylobacter, yersinia), GU (chlamydia)
43
Drug-induced lupus
anti-convulsants, hydralazine, isoniazid, procainamide, minocycline
44
Highest prevalence of SLE in US
African-American females
45
Hemolytic manifestations of SLE
hemolytic anemia, thrombocytopenia, lymphopenia, anti-phosopholipid antibodies
46
SLE renal disease class I
normal/minimal change
47
SLE renal disease class II
mesangial nephritis, no progression
48
SLE renal disease class III
focal segmental proliferative GN, more serious
49
SLE renal disease class IV
diffuse proliferative GN, most severe, low C3, elevated anti-dsDNA
50
SLE renal disease class V
membranous GN
51
SLE renal disease class VI
glomerular sclerosis, end-stage
52
anti-dsDNA
present in active renal dz, pathognomonic for SLE
53
anti-SM
SLE, CNS dz
54
anti-RNP
mixed CT dz
55
SLE treatment
NSAIDS (no ibuprofen - aseptic meningitis), steroids, hydroxychloroquine, azathioprine, cyclophosphamide, MMF
56
Leading cause of death in SLE
sepsis
57
HSP peak incidence
4-7 years
58
Most specific finding of dermatomyositis
Gottron papules
59
Diagnosis of dermatomyositis
CK, LDH, LFT's, MRI, muscle biopsy (gold standard)
60
Most common finding in infants of mothers with SLE
No findings!
61
Most common finding in neonatal lupus
transient photosensitivity rash
62
Treatment of heart block in neonatal lupus
pacemaker
63
PFAPA syndrome
periodic fever, aphthous stomatitis, pharyngitis, adenopathy (elevated ESR/CRP)
64
Management of PFAPA
T&A
65
Treatment of juvenile fibromyalgia
PT, CBT, improved sleep, amitripytline
66
Takayasu's arteritis findings
stenosis of aorta and branch arteries
67
Rate of extra-cutaneous manifestations in linear scleroderma
20%
68
Wegener's granulomatosis findings
sinusitis, hemopytisis, GN w/ hematuria
69
Wegener's diagnosis
c-ANCA, anti-proteinase-3 IgG
70
Findings that indicate underlying rheumatologic condition in Raynaud's
+ANA, abnormal nail fold capillaries
71
Rash associated with sarcoid
erythema nodosum
72
Diagnosis of sarcoid
elevated ACE (60%) - less common in african americans, biopsy w/ noncaseating granuloma
73
Familial Mediterranean fever presentation
attacks of fever, abdominal pain and arthritis
74
FMF natural history
amyloidosis leading to renal failure (reduced by use of colchicine)

Pediatrics (14 decks)

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