Periodontitis as A Manifestation of Systemic Disease Flashcards
Periodontitis can affect the ____
Likewise, systemic conditions & diseases can affect the ____.
rest of the body; periodontium
Periodontitis is a ____ mediated disease
host-mediated
Periodontitis is a host-mediated disease but can have ____ that alter its course
environmental factors
Periodontal disease and systemic condition have three common contributing factors including:
- environmental factors
- host factors
- microbial factors
What do we mean by :
Periodontitis as a manifestation of a systemic disease
meaning system disease is altering the course of peridontitis
How do systemic disorders result in loss of periodontal tissues?
- influence periodontal inflammation
- influence the pathogenesis of periodontal disease
Major impact of systemic diseases on periodontal health:
severe/early-onset periodontitis
Moderate impact of systemic disease on periodontal health:
- increase prevalence
- No otherwise different clinical presentations of chronic periodontitis
Systemic disorders that have a major impact on loss of periodontal tissue by influencing periodontal inflammation include: (3)
- genetic disorders
- acquired immunodeficiency diseases
- inflammatory diseases
List the categories of genetic disorders: (that affect the periodontium)
- diseases associated with immunologic disorders
- diseases affecting the oral mucosa and gingival tissue
- Diseases affecting the CT
- metabolic and endocrine disorders
Diseases associated with immunologic disorders: (that affect the periodontium)
- down syndrome
- leukocyte adhesion deficiency
- papillon lefevre syndrome
- chediak higashi syndrome
- congenital neutropenia
Down syndrome may also be called:
- Trisomy 21
- Mongolism
Down syndrome is characterized by:
- characteristic physical appearance
- mental deficiency
- growth retardation
List some physical characteristics associated with Down syndrome:
- small nose, mouth & ears
- slightly bent 5th finger
- almond-shape eyes with skin fold covering inner corner
- white spots on colored part of eyes
- flat, round face
What chromosome is affected with Down syndrome?
21st
Describe periodontitis in an individual with Down syndrome:
moderate to severe periodontitis with rapid progression
Describe the periodontitis progression in an individual with Down syndrome:
rapid progression
T/F: In an individual with Down syndrome, local factors alone explained the severity of the periodontal destruction
False- local factors alone FAILED to explain the severity of periodontal destruction
Describe the effects of Down syndrome on the immune system:
List some examples:
Intrinsic immune system defects
- poor PMN chemotaxis
- poor phagocytosis
- poor intracellular killing
Caused by a mutation in beta-2 integrin (ITGB2) gene:
Leukocyte adhesion deficiency
What gene is affected in LAD?
Beta-2 integrin (ITGB2)
In LAD, the mutation in beta-2 integrin (ITGB2) results in: (2)
- lack of beta-2 integrin mRNA in leukocytes
- low integrin (CD18 or CD15) expression on neutrophils
What happens to the neutrophils in leukocyte adhesion deficiency?
neutrophils are confined to the blood vessels (resulting in disruption of neutrophil- associated homeostasis)
In individuals affected by this disease we often see a history of severe recurrent infection but no pus; leukocytosis is common:
LAD
In individuals with LAD, we often see a history of ______. ____ is common
severe infection but no pus; leukocytosis
Describe the oral manifestations of LAD: (4)
- severe gingival inflammation
- acute gingival lesions
- early-onset and rapidly progressive alveolar bone loss
- early loss of primary AND permanent teeth
This image shows oral findings in a 6 year old boy with ___. Note the diastema, gingival retraction, and partial root exposure with cervical erosions affecting primary dentition. Permanent lower incisors are erupting.
LAD1
Caused by a mutation in the cathepsin C gene (CTSC)
papillon-lefévre syndrome
What gene is mutated in papillon-lefévre syndrome? What chromosome is this located on?
Cathepsin C gene (CTSC); chromosome 11q14
In papillon-lefévre syndrome, these patients experience compromised ______ function resulting in _____.
neutrophil function; decreased phagocytosis
What characteristic lesion is seen in papillon-lefévre syndrome? What are some common locations for this lesion?
Hyperkeratotic lesions
- palms
- soles of feet
- elbows
- knees
The following images of characteristic of:
papillon-lefévre syndrome
Describe the oral manifestations of papillon-lefévre syndrome: (3)
- severe gingival inflammation
- early-onset and rapidly progressive alveolar bone loss
- early loss of primary & permanent teeth
Case Study:
- A 10 year old male child presented with what appeared to be severe generalized aggressive periodontitis. His parents presented no contributory medical history excepted for skin lesions that had been managed by his medical doctor since birth.
CC: Parents complaining about child’s bad breath and loose teeth
History of main complaint: 1 year. Previously attended da private dentist who had done cleanings and prescribed antibiotics
Medical history: NKDA, med history unknown
Eczema: Hands and feet from early age, been treated by GP with aqueous real since early childhood
Fam medical history: none known
Dental history & habits: Patient brushes once er day, no other dental aids used
Initial presentation seen in image:
Diagnosis: papillon-lefévre syndrome
Disease caused by mutations in CHS1 and LYST gene:
Chediak-Higashi Syndrome
Chediak-Higashi Syndrome is caused by mutations in:
CHS1 and LYST genes
The mutations in Chediak-Higashi Syndrome (CHS1 and LYST genes) result in what lysosomal defect?
Lysosomal trafficking defect
In Chediak-Higashi Syndrome, defects in melanin granules results in:
partial oculocutaneous albinism
What results in the partial oculocutaenous albinism in chediak-higashi syndrome?
defects in melanin granules
In Chediak-Higashi Syndrome, the circulating leukocytes exhibit defective lysosomes causing a decrease in phagocytosis, clinically, what does this cause?
recurrent pyogenic infections
In Chediak-Higashi Syndrome, we clinically see recurrent pyogenic infections. What causes this?
circling leukocytes exhibit defective lysosomes resulting in a decrease in phagocytosis
Describe the neurologic aspects of Chediak-Higashi Syndrome:
varying neurologic problems; intellectual deficit & dementia
Describe the oral manifestations of Chediak-Higashi Syndrome:
- Severe gingival enlargement
- Early-onset and rapidly progressive alveolar bone loss
- Early loss of the primary and permanent teeth
- Poor response to dental treatment
Cause by a mutation in the ELANE (50%), or HAX1 (10%) gene:
congenital neutropenia
Congenital Neutropenia is caused by a mutation in ____ in 50% of cases and ____ in 10% of cases.
ELANE (50%); HAX1 (10%)
In Congenital Neutropenia, ____ causes remain unknown
1/3
Congenital Neutropenia results in a ____ number of ____
decreased number of neutrophils
What neutrophil level is considered “neutropenic”?
ANC < 500 cell/uL and static
The decreased number of neutrophils in Congenital Neutropenia, results in: (2)
- deficiency in the immune response
- severe & recurrent infections
Describe the oral manifestations of congenital neutropenia:
- severe periodontitis is common
- higher risk for tooth loss
- oral ulcers
Epidermolysis Bullosa (Kindler Syndrome) is categorized as a disease affecting the:
oral mucosa and gingival tissue
Mutation in the fermitin family homologue 1 gene:
Epidermolysis Bullosa (Kindler Syndrome)
Epidermolysis Bullosa (Kindler Syndrome) is a disease caused by a mutation in:
Fermitin family homologue 1 gene
In Epidermolysis Bullosa (Kindler Syndrome) , the mutation in the fermitin family homologue 1 gene causes:
- lack of ____
- affects ____
- can lead to defects in the ____
- integrin activation
- keratinocyte adhesion
- basement membrane zone
In Epidermolysis Bullosa (Kindler Syndrome) , the mutation in the fermitin family homologue 1 gene causes:
- lack of integrin activation
- affects keratinocyte adhesion
- can lead to defects in the basement membrane zone
And these all contribute clinically to:
- photosensitivity
- recurrent blister formation
The following images show symptoms characteristic of:
Epidermolysis Bullosa (Kindler Syndrome)
Ehlers-Danlos Syndrome is categorized as a disease affecting the:
connective tissue
Caused by mutations in genes coding fibrillar collagens or enzymes involved in the biosynthesis of these proteins:
Ehlers-Danlos Syndrome
In Ehlers-Danlos Syndrome, the mutations in genes coding fibrillar collagens or enzymes involved in the biosynthesis of these proteins result in alterations of:
collagen production and compositions
What is clinically seen in Ehlers-Danlos Syndrome? (3)
- joint hyper mobility
- skin extensibility
- tissue fragility
Based on these images, what disease does this individual have?
Ehlers-Danlos Syndrome
What is the MAIN oral manifestation seen in Ehlers-Danlos Syndrome. What are some others seen?
- GENERALIZED LACK OF ATTACHED GINGIVA
- generalized, early-onset severe periodontitis
- gingival recession
- early loss of primary & permanent teeth
Hypophosphotasia is categorized as a:
metabolic and endocrine disorder
Caused by a mutation in the alkaline phosphatase (ALPL) gene:
Hypophosphotasia
Hypophosphotasia is a disease caused by a mutation in:
alkaline phosphatase (ALPL) gene
There are both ___ & ___ forms of Hypophosphotasia
mild and severe
Describe the oral manifestations seen in Hypophosphotasia:
- defective cementum
- alveolar bone loss
- premature loss of teeth
- impaired bone/tooth mineralization
- compromised periodontal attachment
In Hypophosphotasia, patients have a compromised ____, resulting in reduction in alveolar bone height
compromised periodontal attachment
In Hypophosphotasia, the teeth are not adequately:
anchored to the alveolar bone via the PDL
Oral signs of HPP (common dental signs): (6)
- exfoliation of the primary dentition before the age of three
- exfoliation of primary teeth with roots intact
- alveolar bone loss
- loss of permanent teeth with no signs of periodontal inflammation
- enlarged pulp chambers and root canals
- thin dentinal walls
Disorder of glucose metabolism:
diabetes mellitus
AGEs:
Advanced Gllycation End Products
- Where there is excess amount of glucose in the circulation, everything gets glycated (once glycated no longer a simple molecule)
