Autoimmune & Inflammatory Disorders Flashcards

1
Q

presence of antibodies (auto-antibodies or auto-reactive T-
cells) directed against normal host antigens (auto- or self-
antigens)

A

Autoimmunity

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2
Q

What are the autoantibodies seen in mucous membrane pemphigoid?

A

BP 180

BP 230

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3
Q

What are the autoantibodies seen in pemphigus vulgaris?

A

Demsoglein 1 and 3

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4
Q
Pathogenesis •Unknown etiology
•Environmental triggers in a 
genetically predisposed individual
•Chronic, inflammatory autoimmune 
disorder
•Autoantibodies and immune 
complexes activate complement 
system
•Vasculitis, fibrosis, tissue necrosis
Complications Multi-organ
Epidemiology •90% are young-middle aged women
•2.5 times increased risk in AA
A

Systemic lupus erythematousus

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5
Q

What autoantibodies are present in SLE?

A

anti DS DNA and anti-SM

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6
Q

What other condition looks identical to SLE histologically and clinically intraorally?

A

Oral Lichen Planus

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7
Q

Which disease presents with a butterfly rash?

A

SLE

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8
Q
Management Rheumatologists: organ-specific approach
•Long-term Prednisone
•Immunomodulating agents (eg. 
mycophenolate mofetil, azathioprine, 
methotrexate)
Oral Manifestations 
-lichenoid lesions
•Ulcerations/erosions
•Hard palatal mucosa ulcer
•White radiating striae from a central 
ulcer
A

SLE

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9
Q
The following are dental considerations needing to be taken if pt has \_\_\_\_\_\_:
Determine the status of the stability of
their disease
Leukopenia 
•Immunosuppressive rx
Thrombocytopenia (25% of pts)
•extreme thrombocytopenia 
(<20,000 platelets)
Nonbacterial verrucous valvular Libman–
Sacks endocarditis
•most common cardiac lesion
Renal disease
•localization of immune complexes 
in the kidney is the precipitating 
factor in the development of 
lupus nephritis
•rapidly progressing 
glomerulonephritis or a less 
aggressive form
Neuropsychiatric disease
•psychosis, seizures, 
cerebrovascular accidents
A

SLE

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10
Q
Pathogenesis •Unknown etiology
•Environmental, hormonal, 
infectious factors, in a genetically 
predisposed individual
•external trigger (infection, 
trauma) elicits an 
autoimmune reaction
•hypertrophy of the synovial 
lining of the joint and 
endothelial cell activation
•uncontrolled inflammation 
and destruction of cartilage 
and bone
A

Rheumatoid arthritis

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11
Q
•1.3 million US adults
•Women 3 times more affected than 
men
•sex difference diminish in older 
age groups, suggesting a 
hormonal component
Diagnosis 6/10 score based on a 4-prong algorithm:
1. joint involvement
2. serology test results (Rheumatoid 
factor, ACPA anticitrullinated protein 
antibody)
3. acute-phase reactant test results (CRP, 
ESR)
4. pt self-reporting of signs/symptom 
duration
A

Rheumatoid arthritis

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12
Q

What lab value will be higher in Rheumatoid arthritis?

A

Rheumatoid factor

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13
Q
Management Nonpharmacological
•Physical, occupational therapies, 
orthotic devices, surgery
Pharmacological
•NSAIDs, disease-modifying 
antirheumatic drugs, 
immunosuppressants, biological 
response modifiers, corticosteroids
Oral Manifestations •TMJ dysfunction
•Medication induced ulcerations
•Salivary hypofunction
Dental Considerations See ADA guideline on prophylactic 
antibiotics for pts with prosthetic joints
undergoing dental procedures
A

Rheumatiod arthritis

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14
Q
Pathogenesis •Unknown etiology
•Endothelial cell injury
•Fibroblast activation
•Vascular dysfunction
Complications •skin thickening/induration 
•tissue fibrosis and chronic 
inflammation
•infiltration of heart, lungs and 
kidneys
•prominent fibroproliferative vascular 
disease
Epidemiology •Peak onset (30–50 years)
•4-9 times higher in women than in 
men
A

Progressive Systemic Sclerosis

Scleroderma

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15
Q
Diagnosis Localized scleroderma: 
•skin on the hands and face
•slow disease course
•indolent and rarely spreads more 
widely or results in serious 
complications
Systemic scleroderma:
•affects large areas of skin and heart, 
lungs, or kidneys
2 main types of systemic scleroderma:   
(1) limited disease or CREST syndrome, 
calcinosis, Raynaud’s syndrome, 
esophageal dysmotility, sclerodactyly, 
telangiectasia                                                
(2) diffuse disease
A

Systemic sclerosis

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16
Q

Management •Long-term Prednisone
•Immunomodulating agents (eg.
mycophenolate mofetil, azathioprine,
methotrexate)
Oral Manifestations Severe microstomia, trismus, submucosal
fibrosis
Dental Considerations Elongation exercises to improve trismus

A

Systemic sclerosis

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17
Q
Pathogenesis •Chronic, autoimmune, inflammatory 
disorder
•Primary vs Secondary SS (RA, SLE)
•Unknown etiology
•expression of MHC-II molecules in 
activated salivary gland cells
•inherited susceptibility markers 
trigger a chronic inflammatory 
response in genetically 
susceptible individuals
•ongoing activation of the innate 
immune system as pro-
inflammatory cytokines (eg. IFN-g) 
are elevated
Complications •Lymphocytic infiltration 
•lacrimal glands causing dry eyes 
(xerophthalmia)
•salivary glands causing drymouth
(xerostomia)
A

Sjogren’s syndrome

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18
Q
Epidemiology •Elderly women 
•Female-to-male ratio is 9 : 1
Diagnosis SS ACR Classification Crietria (2/3)
1. Positive anti-Ro and/or anti-La or (+RF 
and ANA titer >1:320)
2. Minor labial salivary gland biopsy 
exhibiting focal lymphocytic
sialadenitis focus score >1 
focus/4mm2
3. Ocular staining score >3
Management •Rituximab (anti-CD20 monoclonal 
antibodies)
•Long-term Prednisone
•Immunomodulating agents (eg. 
mycophenolate mofetil, azathioprine, 
methotrexate)
Dental Considerations Salivary hypofunction
•stimulated and unstimulated
salivary flow measurements
•44 times increased risk of MALToma
A

Sjogren’s Syndrome

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19
Q
Deficiency of C1 esterase 
inhibitor so can’t inhibit 
bradykinin which results in 
increase vasodilation and 
vascular permeability
A

hereditary angioedema

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20
Q
\_\_\_\_\_ is idiopathic or immune mediated
- angular chelitis
-Lip swelling
-Tongue fissuring
-Cobblestoning
-Uclers
Ranges from segmental or diffuse erythema to edematous 
hyperplastic gingiva +/- pain
Stag horn appearance
-Median age 24
-Food sensitivity can trigger this
Tx: 
-Diet modification 
•Topical/intralesional/systemic corticosteroids
•delayed release triamcinolone
•Various response
•Adjuvant therapies
•clofazimine, sulfasalazine, hydroxychloroquine, methotrexate, 
danazol, dapsone, TNF-alpha ant., metronidazole
A

Orofacial granulomatosis

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21
Q

The following 3 signs signal what syndrome?
•Lip swelling
•Fissured tongue
•Facial paralysis

A

Melkerson-Rosenthal Syndrome

22
Q

What other systemic disease is more likely to be present with orofacial granulomatosis

A

Chron’s disease

23
Q

What variable is seen most frequently in a OFG pt that has Chron’s?

A

Ulcers

24
Q

What other bacterial species can cause angular chelitis besides candida?

A

S. Aureus

25
Q
•Multisystem 
diseases involves 
the lungs, eyes, 
and skin
•Chest radiographic 
changes during a 
routine screening 
examination
•Systemic 
symptoms: fatigue, 
night sweats, and 
weight loss
Oral Presentation
•Plaque or nodular lesion 
•Tongue and lip
Treatment
•Oral prednisone (20 to 40 mg/day); international expert panel
•Evaluate the response after 1 to 3 months
•If there has been a response, the prednisone dose should be 
tapered to 5 to 15 mg per day, with treatment planned for an 
additional 9 to 12 months
•Lack of a response after 3 months suggests the presence of 
irreversible fibrotic disease, or an inadequate dose of 
prednisone
A

Sarcoidosis

26
Q

Pathogenesis of _____
•Development and accumulation of granulomas (Compact,
centrally organized collections of macrophages and
epithelioid cells encircled by lymphocytes)
•Granulomas generally form to confine pathogens, restrict
inflammation, and protect surrounding tissue
•Macrophages, with chronic cytokine stimulation,
differentiate into epithelioid cells, gain secretory and
bactericidal capability, lose some phagocytic capacity, and
fuse to form multinucleated giant cells

A

Sarcoidosis

27
Q
•Acute presentation 
•Arthritis, erythema nodosum, and 
bilateral hilar adenopathy (9 to 34% 
of patients)
•Erythema nodosum is observed 
predominantly in women
•Marked ankle periarticular
inflammation or arthritis without 
erythema nodosum is more 
common in men

-seen in sarcoidosis

A

Lofgren’s syndrome

28
Q

•Acute hypersensitivity reaction to various antigens
•Characterized clinically by target lesions distributed symmetrically
on extremities and trunk
•Usually self-limiting but may be recurrent
•Many different classifications exist
Epidemiology
•Prevalence < 1%
•Young adults 20-40 years old
•1:1.5 M:F ratio
•Reported recurrence rate: 37%
•Oral involvement: as high as 70%

A

Erythema multiforme

29
Q

Clinical presentation
•Vesiculobullous condition that may affect skin or mucous
membranes
•Symmetric involvement of extremities and trunk
•Characteristic lesion: target/iris lesion
•‘Multiform’ clinical features:
•Characteristic target lesions
•Macules
•Vesicles
•Papules
Management
•Treatment of relevant infections or removal of causal drugs
•Complete recovery usually occurs in 2-4 weeks
•No treatment identified that predictably alters clinical course
•Topical or systemic steroids
•0.5-1.0 mg/kg/day tapered over 7-10days
•Steroid sparing agents
•Lesions usually heal without scarring

A

Erythema multiforme

30
Q

What viruses are most commonly associated with Erythema multiforme?

A

HSV 1 and 2

31
Q

What bacteria is most common in erythema multiforme?

A

Mycoplasma pneumoniae

32
Q

____ associated EM
•No or mild prodromal symptoms
•More likely to be recurrent
•70-80%: HSV infection is precipitating event
•HSV-specific CD4+ Th1 cells and inflammatory cytokines (IFN-γ)
recruit auto-reactive CD8+ T-cells
•CLA+ (skin-homing) CD8+ T-cells triggered by viral antigen-positive
cells
•Keratinocyte growth arrest
•Keratinocyte lysis and apoptosis
•Release of cytotoxic factors
•Mechanism of auto-reactive T-cell generation unclear

A

HAEM (Herpes associated EM):

33
Q

_____ induced EM):
•Flu-like prodrome common
•Less likely to be recurrent

A

DIEM (drug induced

34
Q

•EM ____:
•Rashes/target lesions symmetrically distributed on extremities
•Mucous membrane involvement limited to one surface ->
typically oral mucosa

A

minor:

35
Q

EM _____:
•Skin lesions may also be atypical -> raised, bullous
•Involvement of multiple mucous membranes

A

major:

36
Q

•Severe cutaneous blistering hypersensitivity reaction clinically
similar to EM but with more frequent mucosal involvement
•Etiology, genetic susceptibility, and pathomechanism distinct from
EM
•Etiology, genetic susceptibility, and pathomechanism identical to
Toxic epidermal necrolysis
•Now thought to form single disease entity, distinct from EM:
SJS/TEN

A

Stevens-Johnson syndrome

37
Q

•Severe cutaneous adverse reaction that affects skin and
mucosal surfaces
•Erythema and blister formation of varying extent
•Hemorrhagic erosions of mucous membranes
•Fever and malaise:
•Often first sign of disease
•May persist after mucocutaneous lesions appear
•Mainly caused by drugs but infections and other unidentified
risk factors may play a role

A

SJS/Toxic

epidermal necrolysis

38
Q
•T-cell immune mediated chronic muco-cutaneous inflammatory 
disease
•Stratified squamous epithelium of skin, oral mucosa, genital mucosa, 
larynx
•Is it an auto-immune condition?
•No circulating auto-antibodies
•Unknown etiology
•1-2% adult population
•Most common skin condition with oral involvement
•Age: 30-60yrs
•F>M (1.4:1)
Clinical Features
•10-15% have skin LP
•6 P’s
•Polygonal
•Pruritic
•Purple
•Papular
•Planar 
•Plaques
•Koebner phenomenon
•Wickham striae on flexor surface of wrists
•LP lesions at sites of trauma
A

Oral lichen planus

39
Q
Histopath of \_\_\_\_\_\_
•Chronic dense band-like infiltrate of lymphocytes
•Saw tooth epithelial rete pegs/ridges
•Degeneration of basal 
cell layer
•Colloid/Civette bodies
•degenerating keratinocytes
A

Oral lichen planus

40
Q

•Autoimmune vesicullobullous condition
Many subtypes
•Incidence: 5/1,000,000 cases per year
•Median Age: 50 yrs
•No sex predilection
•Mediterranean, South Asian or Jewish heritage
•HLA-DR4, DRw14 & DQB1 associated with PV

A

PV

41
Q
•application of firm 
lateral pressure on 
normal-appearing 
mucosa adjacent to a 
pre-existing bulla 
induces new bulla 
formation
A

Nikolsky sign

42
Q

•application of pressure
directly to a bulla causes
lateral extension

A

Asboe-Hansen sign

43
Q
Oral Features
•“first to show, last to 
go”
•oral lesions 1st sign >50% 
of cases >1 year
•almost all have oral 
features
•most difficult to resolve 
with tx
•Untreated oral & 
cutaneous lesions
•persist progressively 
involving more surfaces
Histopath:
•Intraepithelial 
separation
•Acantholysis
•epithelial spinous layer 
cells fall apart
•rounded shape for loose 
cells (Tzanck cells)
•Lamina propria
•mild-moderate chronic 
inflammatory cell 
infiltrate
A

Pemphigus vulgaris

44
Q

What are the 3 conditions that Tzank cells can be seen in?

A

EM
PV
HSV

45
Q

What is the tx that must be used for pt with PV?

A

Long term corticosteroids

46
Q
•Most common autoimmune blistering condition
•1 in 100,000 annually
•Age: 6th-8th decade
•Gender: 2:1 male
•Oral involvement is uncommon
•8-39%
- Skin only
•Good prognosis
•spontaneous remission after 2-5 years
•up to 27% mortality rate
•Systemic immunosuppressive therapy
•Prednisone QD/QOD
•Azathioprine added if no response
•Alternative therapies
•Dapsone
•Tetracycline
•Niacinamide
•Refractory cases
•Prednisone & Cyclophosphamide
A

Bullous pemphigoid

47
Q
  • Chronic, blistering, mucocutaneous autoimmune disease
  • Autoantibodies to hemidesmosomes
  • Positive Nikolsky sign
  • Unknown incidence
  • 2x as common as PV
  • most common oral AD
  • Age: 5th-6th decade
  • Gender: 2:1 female
  • Ocular involvement only
  • Oral involvement only
  • Mucosal & cutaneous involvement
  • Multiple mucosal sites without cutaneous involvement
  • nose, esophagus, larynx, vagina
A

Mucous Membrane Pemphigoid (MMP)

48
Q
•Conjunctival mucosal 
scarring
•Entropion
•Symblepahron
•Trichiasis
•Scarring closes 
lacrimal gland 
openings
•loss of tears
•extremely dry
•cornea produces 
excess keratin
•Blindness
•if untreated
•opacification from 
excess keratinization
•Referral to an ophthalmologist
•25% of oral MMP pts develop ocular lesions
•Referral to a dermatologist
•20% of oral MMP pts develop cutaneous lesions
•Topical corticosteroids (custom trays)
•0.05% fluocinonide gel BID
•0.05% clobetasol gel BID
A

MMP

49
Q
  • Neoplasia-induced Pemphigus
  • Paraneoplastic Autoimmune Multi-organ Syndrome (PAMS)
  • Rare vesicullobullous disorder
  • 150 documented cases
  • Neoplasm hx
  • Non-Hodgkin Lymphoma (42%)
  • Chronic Lymphocytic Leukemia (29%)
  • Sarcoma (6%)
  • Thymoma (6%)
  • Castleman Disease (6%)

Pathogenesis
•Unknown
•Multifaceted immunologic attack
•Evidence suggests abnormal cytokine levels
•IL-6 produced by lymphocytes in response to patient’s tumor
•IL-6 stimulates abnormal production of antibodies
•antibodies against desmosomal complex antigens
•Mediated by cytotoxic T-lymphocytes in some cases

A

Paraneoplastic Pemphigus

PNP

50
Q

What 2 conditions are seen with PNP?

A

Non hodgkin lymphoma

51
Q
Oral Manifestations
“Erythema multiforme-
like stomatitis”
•Hemorrhagic lip 
crusting
•All sites
•Oral involvement 
only in some cases
•Multiple painful 
irregular ulcers
Management
•High morbidity & mortality (>90%)
•complications from vesiculobullous lesions
•immunosuppressive therapy
•trigger a reactivation of malignant neoplasm
•~ 50% develop bronchiolitis obliterans
•Systemic prednisone + immunosuppressant
•azathioprine
•methotrexate 
•cyclophosphamide
A

Paraneoplastic pemhigus

52
Q

What autoantibodies are seen in PNP?

A

Plakins