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key points AI diseases Flashcards

1
Q

SS primary

A

no CT disease

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2
Q

SS triad

A

exocrinopathy - dryness mouth and eyes
fatigue
joint pain

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3
Q

SS epidemiology

A

usually 40-60yrs

F

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4
Q

SS aetiology

A

infections
biological factors
chemical
genetic - HLA association

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5
Q

SS proposed immunopathogenic mechanisms

A 
microbial trigger
destroys SG epithelium
autoantigens
differentiation of B cells into plasma cells
auto-ABs form complex with auto Ags
captured by DCs - T1 interferon
activation of cytotoxic CD8

innate and adaptive

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6
Q

oral S+S SS

A 
salivary hypofct
caries
fungal infections
oral trauma
lip dryness
orofacial pain
dysphagia
dysgeusia
swollen SGs
GORD
oral lesions of AI aetiology
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7
Q

SS systemic manifestations

A 
30-40%
constitutional
LNs
renal - interstitial nephritis
articular - arthralgias
cutaneous
peripheral neuropathy
muscular - myositis
pulmonary
glandular
CNS
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8
Q

SS ESSDAI

A

clinical index to measure disease activity in pts w primary SS
each has a weighting - give score depending on severity

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9
Q

SS salivary hypofct/dysfct

A

diminished secretions on palpation
thicker/opaque/viscous secretions
recurrent SG infection
enlarged SGs in 30% (parotid)

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10
Q

SS caries

A

hyposalivation - reduced IgA secretion
- AB responsible for oral mucosal immunity that prevents caries
saliva in pSS not effective buffer
cervical and other atypical sites e.g. lingual surface, incisal edge and cusps

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11
Q

what fungal infection is prevalent in SS?

A

candidiasis

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12
Q

SS AI/immunomediated lesions

A

oral LP/lichenoid lesions
RAS
MMP
PV

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13
Q

secondary SS

A 
when pt already has another AI disease then presents with extreme dryness of the eyes and mouth
CT disease (SLE, RA, systemic sclerosis)
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14
Q

SS eye

A 
dry eye
extraglandular ocular complications
 - corneal inflammation
 - conjunctival inflammation
 - uveitis
 - scleritis/episcleritis
 - optic neuritis
 - retinal vasculitis
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15
Q

SS autoABs

A

anti-SSA ABs - antiRo
anti-SSB ABs - antiLa
present in 2/3 pts

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16
Q

SWSF >0.7ml/min

A

normal/mild dysfct
non-pharmacological stimulation
(saliva subs/pharmacological stimulation)

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17
Q

SWSF 0.1-0.7ml/min

A

mod dysfct
non-pharmacological/pharmacological stimulation
(saliva substitutes)

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18
Q

SWSF <0.1ml/min

A

severe dysfct

saliva substitutes

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19
Q

SS classification criteria - American College of Rheumatology/European League against Rheumatism
inclusion criteria

A

≥1
daily, persistent, troublesome dry eyes >3m?
recurrent sensation sand/gravel in eyes?
use tear substitutes ≥3 times daily?
daily feeling of dry mouth for >3m?
freq drink liquids to aid swallowing dry food?

or when suspicion of SS from EULAR SS DAI (≥1 positive)

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20
Q

SS classification criteria - American College of Rheumatology/European League against Rheumatism exclusion criteria

A 
history of H+N radio
active Hep C
AIDS
sarcoidosis
amyloidosis
GvHD
IgG4-related disease
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21
Q

SS classification criteria - American College of Rheumatology/European League against Rheumatism - what do you need to score?

A

meet inclusion criteria
no exclusion criteria
score ≥4

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22
Q

SS classification criteria - American College of Rheumatology/European League against Rheumatism - scoring

A 
biopsy - 3
antiSSA/Ro + - 3
ocular staining score ≥5 - 1
schirmer's test ≤5mm/min - 1
UWSF ≤0.1ml/min - 1
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23
Q

biopsy requirements SS

A

labial SG with focal lymphatic sialadenitis and focus score of ≥1 foci/4mm2
- ≥1 dense aggregates of ≥50 lymphocytes usually located in perivascular or periductal locations

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24
Q

biopsy technique SS

A

≥5 minor glands, L lip in paramedian region, incision parallel to labial frenum, identify minor gland, lift, remove, suture

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25
SS non-pharmacological stimulation
gustatory stimulants: SF acidic candies, lozenges | mechanical stimulants: SF chewing gum
26
SS caries prevention
``` freq recall 4-6m OH F non-F remineralising agents salivary stimulation AM agents e.g. CHX ```
27
why is pilocarpine not always possible?
salivary residual secretory capacity needed - still need fct parenchyma
28
pilocarpine dose
5mg lozenges | 3-6 daily
29
pilocarpine contraindications
``` uncontrolled asthma/COPD uncontrolled cardiorenal disease narrow-angle closure glaucoma gall bladder stones acute iritis ```
30
main primary sites of lymphoma in primary SS
``` SALIVARY GLANDS (parotid) lungs, gastric, ocular, oral/ENT, spleen ```
31
lymphoma risk in SS
``` risk of haematological malignancy most B-cell origin 3 subtypes - MALT lymphoma - DLBCL - MZL small number get non-B-cell haematological cancers - myeloid leukaemia, Hodgkin disease or T/NK cell lymphoma ``` test regularly (1-2yrs, high risk 6m)
32
SS risk factors for lymphoma
``` recurrent swelling of parotid glands splenomegaly/lymphadenopathy purpura > on ESSDAI RF cryoglobulinaemia low C4 level CD4 T-cell lymphocytopenia presence of ectopic germinal centres focus score of >3 germinal mutations in TNF AIP3 ```
33
SS tx
inhibit proinflammatory cytokines - infliximab interfere w T1 interferon production - hydroxychloroquine immunobiologics - rituximab, infliximab
34
sicca syndrome
partial SS findings
35
American European Consensus Group Revised International Criteria SS
``` 1 - dry eyes subjective 2 - dry eyes objective 3 - dry mouth subjective 4 - dry mouth objective 5 - autoAB findings 6 - histopathology ``` ≥4 positive criteria (must inc 5 and/or 6)
36
T1 hypersensitivity
IgE - anaphylaxis
37
T2 hypersensitivity
IgG/M - cytotoxic - AgAB
38
T3 hypersensitivity
IgG/M - immune complex deposition (Ag-Ab)
39
T4 hypersensitivity
T cell mediated (delayed type)
40
what type of blister is pemphigus?
intraepithelial
41
what hypersensitivity reaction is pemphigus?
T2 - cytotoxic Ag-Ab reaction - destruction of desmoglein damage to LP IgG Abs form
42
pemphigus HP
take perilesional biopsy of area not ulcerated/blistered - as won't have epithelium in ulcer acantholysis suprabasal split tzank cells basal cells still fully attached by hemidesmosomes
43
acantholysis
spaces between spinous cells
44
tzank cells
in pemphigus | epithelial cells fall off into blister/bullae
45
DIF
tissue biopsy usually for diagnosis AB and fluorophore
46
IIF
serum usually to measure ABs but can be used for diagnosis can monitor progress and response to therapies unlabelled Ab (primary) and supporting Ab with fluorophore (secondary)
47
positive DIF pemphigus
basket weave pattern reflect where you get Ag-Ab reaction grey areas - intraepithelial split/cleft desmosomes
48
positive DIF pemphigoid
linear pattern | in hemidesmosomes - basal cell layer
49
what type of blister is pemphigoid?
subepithelial
50
what type of hypersensitivity reaction is pemphigoid?
T2
51
pemphigoid HP
sub basal split no acantholysis or tzank cells LP shows inflammation - inflammatory cells eosinophils characteristic of bullous pemphigoid not so obvious in MMP
52
why do many diseases affect both skin and oral/genital mucosa?
they share many common antigens and epitopes antigens - provoke immune response but epitope binds epitopes - part of Ag that binds to Ab
53
general pathogenesis of AMBDs
``` auto-AB attack on skin components causing loss of cell-cell adhesion - desmosomes - hemidesmosomes (basal cell layer) split/cleft forms in skin - fills with inflammatory exudate - vesicle (<5mm)/bulla (>5mm) ```
54
SS histology minor gland
``` focal lymphocytic sialadenitis acinar loss fibrosis focal collections of lymphocytes (50+) - ≥1 collection per 4mm2 ```
55
SS histology major gland
lymphocytic infiltrate, extends to whole lobules epithelial hyperplasia of duct (myoepithelial islands) eventually occludes atrophy of acini
56
pemphigus pathogenesis
``` autoABs against desmoglein intraepithelial blister acantholysis autoABs deposited in epithelium anti-Dsg3 IgF complement activated and plasmin ```
57
pemphigus classification
``` vulgaris foliaceous drug-induced paraneoplastic IgA ```
58
age pemphigus
40-60s
59
pemphigus foliaceous
superficial form - skin lacks mucosal involvement anti-Dsg1 oral lesions rare
60
pemphigus prognosis
untxed often fatal - metabolic consequences, dehydration/infection mucosal lesions may persist after skin controlled - topical CS/tacrolimus now often complications of tx major cause of death - CS, MAbs
61
pemphigus mucosal features
progressive - typically begins oral cavity, can spread to skin oral cavity - anywhere - non-healing erosions/ulcers, rarely see intact blisters - desquamative gingivitis - yellow fibrinous slough - painful, plaque irritates lesions - easily peel off - haemorrhagic erosion - spongiosis - no scarring
62
pemphigus diagnosis
DIF - basket weave/fishnet like pattern in epithelium - IgG, C3, IgM, IgA (variant) IIF - high % positive - IgG, C3, IgM, IgA histopathology - intraepithelial blisters
63
pemphigus tx
CS adjuvant CS sparing agents CHX 0.12% alcohol free rinse anti-mycotic agents triamcinolone injections advanced therapeutic options - IVIgG - rituximab (anti-CD20) - anti-TNFa - plasmapheresis - immunoadsorption
64
pemphigoid epidemiology
>75s | rare
65
pemphigoid pathogenesis
autoABs against hemidesmosomes complement, leukocytes, BM damage subepithelial blister
66
pemphigoid clinical
``` bullae/vesicles - often blood filled erosions/ulcers - yellow fibrinous slough desquamative gingivitis ```
67
MM pemphigoid
mostly mucosae 20% skin HP/SP, uvula, gingivae
68
bullous pemphigoid
skin (flexor) | oral 20%
69
cicatritial pemphigoid
scarring
70
pemphigoid diagnosis
``` DIF - linear pattern - u or n serrated - deposition along BMZ - IgG, C3, IgA IIF salt split (NaCl scissors at lamina lucida) - IgG HP - subepithelial blister ```
71
pemphigoid tx
low risk - oral +/- limited skin - topical CS high risk - diffuse and progressive oral mucosa, ocular, genital, oesophageal, laryngeal CS +/- azathioprine etc IVIgG/rituximab (partial response/progressive)
72
diagnosis of AMBDs
``` Nikolsky sign biopsy need immunologic signal to confirm DIF IIF ELISA ```
73
Nikolsky sign
marginal - next to blister direct - healthy skin induce erosion by rubbing on skin
74
DIF
oral mucosa, detect tissue bound immunodeposits
75
IIF
pts sera | detect circulating IgG autoABs
76
ELISA
detect anti-Dsg ABs
77
why is early diagnosis of AMBDs essential?
``` may not be AMBDs prevent mucosal spread prevent MC spread mucosal scarring phenotype of MMP - oesophageal stricture - eye scarring - blindness ```
78
PAMS
``` MC syndrome heterogeneous S+S - desquamative stomatitis - polymorphous cutaneous eruption - progressive resp failure underlying malignancy ```
79
pathogenesis of PAMS
cancer cells activate wide number of immune cells - activated autoreactive T cells induce both humoral and cell mediated immunity
80
PAMS spectrum
humoral immunity ----- cell mediated immunity | acantholysis, bronchiolitis obliterans, lichenoid dermatitis
81
PAMS spectrum similar to oral diseases
humoral immunity ---- cell mediated immunity | pemphigus, bullous pemphigoid, EM like, GvHD like, lichenoid/LP like
82
what is the major antigenic target in PAMS?
plakins | - desmoplakin 1 and 2, envoplakin, periplakin, plectin, BP230
83
lesions in PAMS?
ocular lip oral
84
what is the key thing to do in PAMS?
detect a neoplasm | tx of malignancy essential but not always sufficient to tx PAMS
85
AMBDs
Ig-mediated diseases with autoantibodies against desmosomal or BM zone molecules
86
PAMS suggested criteria
clinical signs - severe stomatitis, blisters, LP like plaques, bronchiolitis obliterans HP DIF IIF detection of circulating auto-ABs - envoplakin/periplakin detection of a neoplasm
87
what BPs are the target in pemphigoid?
BP180 | BP230
88
Behcet disease triad
oral ulcers genital ulcers uveitis
89
Behcet disease - what is now recognised along with the triad?
involvement of most organ systems recognised systemic vasculitis often delayed diagnosis
90
what is Behcet disease known as and why?
silk road disease | more common in people of this bloodline
91
Behcet disease pathogenesis
disruption of innate immunity disruption of adaptive immunity AB dependent cellular cytotoxicity to oral epithelium hypercoagulability autoABs deposited around blood vessel walls
92
Behcet disease aetiology
``` genetic predisposition - HLA B51 association env factors - HSV - s sanguis - pollutants ```
93
Behcet disease clinical manifestations
``` oral aphthous ulcers genital lesions ocular lesions skin lesions - palpable purpura, SC nodules vasculitis ``` can be more extensive
94
Behcet disease pathargy test
needle prick (SC puncture) 24-48hrs exaggerated inflammatory reaction - pustule/nodule forms
95
Behcet disease diagnosis
difficult no lab test mostly clinical history key
96
Behcet disease - consensus classification of paediatric Behcet disease
recurrent oral aphthosis ≥3 a genital ulceration and aphthosis skin - necrotic folliculitis, acneform lesions, erythema nodosum ocular - uveitis, retinal vasculitis neurological signs (not isolated headaches) vascular signs - venous thrombosis, arterial thrombosis/aneurysm score ≥3
97
Behect disease tx
``` CS topical colchicine azathioprine biologic therapies ```
98
what is the most common manifestation of Behcets?
oral aphthous ulcers 90-100%
99
Behcet disease differential diagnoses
``` RAS infections skin diseases Crohns SJS pemphigoid pemphigus SLE IBD sarcoidosis ```
100
SLE what is it?
AI multisystem disorder CT disease almost all tissues and organs systemic inflammation and tissue damage
101
SLE antibody
ANA+
102
SLE epidemiology
more F
103
SLE contributing factors
``` genetic predisposition env - smoking, UV, EBV, exposure to silica dust, petroleum, organic solvents and mineral oils hormonal epigenetic immunoregulatory factors ```
104
SLE manifestations
``` constitutional - fatigue and fever renal - lupus nephritis CV - pericarditis MS - arthritis/arthropathy neuropsychiatric pulmonary GI ocular cutaneous ```
105
types of cutaneous lupus
chronic/discoid cutaneous subacute cutaneous acute cutaneous
106
discoid/chronic cutaneous lupus
most common clinical manifestation of cutaneous lupus disk lesions - plaques, scar scarring alopecia dsDNA+ oral mucosa - OLLs 25% (mainly HP) oral DLE is an OPMD but malignant transformation is extremely rare
107
subacute cutaneous lupus
erythematous macules/papules, evolve into either - scaly papulosquamous psoriasiform lesions - annular patches and plaque oral lesions rare, similar to discoid healing - post-inflammatory hyper and/or hypopigmentation, greyish atrophic scarring and telangiectasias
108
acute cutaneous lupus
``` can be 1st sign of SLE - preceding onset of systemic disease by weeks/months malar/butterfly rash - erythematous oral lesions - ulcerations - mainly HP - intense erythema +/- petechiae also when your systemic lupus is active ```
109
SLE lab testing
+ANA mandatory entry criterion - 99% cases present v high sensitivity but limited specificity (occur in other CT diseases) anti-SM and anti-dsDNA highly specific, less sensitive
110
SLE oral manifestations
``` oral mucosa - ulcerations - LLs - erythematous lesions - leukoplakias - petechiae - cheilitis with erythema salivary gland - low flow rate (associated with SS) caries TMJ involvement PDDs ```
111
SLE classification criteria (not diagnostic)
entry criterion ANA titre ≥1:80 additive criteria - don't count if more likely explanation than SLE - one occasion sufficient - don't need to occur simultaneously - need ≥1 clinical criterion and ≥10 points - in each domain count only highest criterion
112
SLE pharmacological tx
``` based on pt manifestations CS - prednisolone hydroxychloroquine immunosuppressive e.g. azathioprine NSAIDs for MS pain drugs targeting B cells: rituximab ```
113
SLE and cancer
``` slight increase in cancer risk overall haematologic (lymphoma, leukaemia, MM), NHL, H+N cancer possible factors mediating risk - lupus-related meds e.g. cyclophosphamide - inherent immune system abnormalities - overlap with SS - viral infections e.g. HPV/EBV - traditional cancer RFs e.g. smoking ```
114
what oral effect can hydroxychloroquine cause?
oral pigmentation
115
SLE and lichenoid oral reactions
central atrophic/shallow erosions with white striae at margins often patchy and unilateral may be on HP (usually spared by OLP)
116
EM - what is it?
vasculitic AI disease acute immune-mediated inflammatory MC disease triggered by hypersensitivity reactions to various antigens with a tendency to recur can affect MM and skin
117
EM epidemiology
usually young adults
118
EM pathogenesis
T3 hypersensitivity reaction - form Ag-Ab complex in circulation - deposit of immune complex in bv's - appearance of inflammatory reaction with subsequent vasculitis
119
EM causes
INFECTIONS and DRUGS
120
EM - what is the most commonly identified cause?
HSV infection
121
what is the most common cause of EM in kids?
mycoplasma pneumoniae infection
122
EM - drugs that can cause it
``` ABs sulfonamides antiepileptics barbituates NSAIDs ```
123
other conditions that can cause EM
IBD malignancy menstruation
124
DIEM immunology
no IFNy but have TNFa produced by macrophages, which along with perforin and granzyme B, will induce keratinocytes apoptosis with subsequent epithelial destruction
125
EM subtypes
isolated recurrent persistent
126
HAEM
7-21 days after primary or recurrent viral infection - HSV DNA engulfed - migrate to epidermis to transfer these antigens to keratinocytes - expression leads to activation of HSV specific CD41 Th1 cells, which produce IFNy epithelial damage
127
isolated EM
occurs just once | - infections, drugs
128
recurrent EM
freq occurrence of EM over a period of years, usually >6 episodes p.a. - infections, menstruation
129
persistent EM
continuous occurrence of typical and atypical lesions without interruption - IBD, infections, malignancy
130
clinical forms of EM
minor | major
131
minor EM
skin <10% BSA mucosa uncommon, 1 site only, usually oral usually target lesions/bullae
132
major EM
skin <10% BSA mucosa ≥2 sites usually begins with starting a new drug or HSV myalgias, sore head, throat and fever spreads to mouth, upper resp tract, GIT, then kidneys and joints
133
HAEM cause
HSV 1 or 2
134
HAEM course
no/mild prodromal S+S acute self-limiting recurrent
135
HAEM site
skin | minimal mucosal
136
HAEM histology
focal keratinocyte necrosis, edema, predominant mononuclear infiltration CD4+
137
HAEM cytokine
IFNy
138
DIEM cause
drugs
139
DIEM course
flu-like prodrome acute self-limiting not recurrent
140
DIEM site
skin and mucosa
141
DIEM histology
extensive keratinocyte necrosis, less edema, prominent mononuclear infiltration CD4+
142
DIEM cytokine
TNFa
143
typical target lesion EM
central blister edematous ring erythematous border
144
atypical target lesion EM
raised, edematous lesion with two zones of colour change and a poorly defined border
145
oral lesions EM
small ulcers to widespread huge erosive lesions can lead to pts not being able to eat, speak or open mouths precede lesions in other SSE diffuse and widespread macules - blisters - ulceration lips swollen, cracked/crusted usually lips and anterior mouth
146
EM skin lesions
commonly distal extremities of extensor surfaces (arms, legs, elbows, knees, dorsum of hands and feet) lesions symmetric, round, slightly pruritic, may evolve into papules some lesions may form the large annular (ring shaped) target lesions, the dark centre of it being a necrotic ulcer
147
EM diagnosis
``` no standardised criteria clinical history clinical exam biopsy (skin) - HandE, DIF lab studies - test for ESR, WCC, LFT, electrolytes - IIF to rule out AI blistering disease ```
148
EM diagnosis - clinical history
acute, episodic, self-limiting symptoms of HSV, mycoplasma pneumoniae and other infections thorough meds history (often started 2-3 days prior)
149
EM diagnosis - clinical exam
skin lesions: typical, atypical morphology: pleomorphic appearance of oral lesions location: lips and anterior mouth - erythematous, erosions, fibrin, haemorrhagic
150
EM tx
tx underlying infection/medication symptomatic tx - clinical severity - prevent infections and local symptom control mild: topical antiseptics, antihistamines, analgesics, topical CS MM: high potency CS gel, oral anaesthetic solutions, topical ophthalmic preparations severe mucosal: 40-60mg prednisolone, taper 2-4wks recurrent: 6m trial of continuous antiviral therapy

Oral medicine (14 decks)

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