Periodontitis as a Manifestation of Systemic Disease Flashcards
Periodontitis can affect the ______
Likewise systemic conditions & disease can affect the _____
rest of body
peridontium
Periodontal diseases & systemic disorders 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 systemic disease is altering the course of periodontitis
How do systemic disorders result in a loss of periodontal tissues?
- influence periodontal inflammation
- influence the pathogenesis of periodontal diseases
Major impact of systemic diseases on periodontal health:
severe/early-onset periodontitis
Moderate impact of systemic diseases on periodontal health:
- increase prevalence
- no otherwise different clinical presentations of chronic periodontitis
Systemic disorders that have a major impact on the loss of periodontal tissue by influencing periodontal inflammation:
- 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 & gingival tissue
- diseases affecting the connective tissues
- metabolic & 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 or 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 (pinky)
- short, stocky build
- almond-shaped eyes with skin fold covering inner corner
- white spots on colored part of eyes
- flat, round face
What chromosome is affected in Down syndrome?
21st
Describe periodontitis in an individual with Down syndrome:
moderate to severe periodontitis with rapid progression
Describe the progression of periodontist in an individual with down syndrome:
rapid progression
T/F: in an individual with down syndrome, local factors alone explain the severity of periodontal destruction
False- local factors alone FAIL TO EXPLAIN the severity of periodontal destruction
Describe the affects of down syndrome on the immune system:
List some examples: (3)
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 leukocyte adhesion deficiency?
Beta-2 integrin (ITGB2)
In LAD, there is a mutation in Beta-2 integrin (ITFB2), describe what this mutation causes: (2)
- lack of beta-2 integrin mRNA in leukocytes
- low integrin (CD18 or CD15) expression in neutrophils
What happens to the neutrophils in LAD? What does this result in?
Neutrophils are confined to blood vessels resulting in disruption of neutrophil-associated homeostasis
In individuals affected by this disease we often see a history of severe recurrent infection with no pus; leukocytosis is common:
LAD
In individuals with LAD, we often see a history of _____. ______ is common.
Severe recurrent infection but no pus; leukocytosis
Describe the oral manifestations of LAD:
- Severe gingival inflammation
- aute gingival lesions
- early on-set & rapidly progressive alveolar bone loss
- early loss of the primary & permanent teeth
This image shows oral findings in a 6-year old bow with _____. Note 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 expérience compromised _____ function, resulting in ____.
neutrophil; decreased phagocytosis
What characteristic lesion is seen in Papillon-Lefévre Syndrome? What are some common locations for this lesion?
Hyperkeratotic lesions
-palms
-soles of the feet
-elbows
-knees
The following images are 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 the primary & permanent teeth
Case Study:
A 10yo male child presented with what appeared to be severe generalized aggressive periodontitis. His parents presented no contributory medical history except for skin lesions that had been managed by his medical doctor since birth,
CC: Parents complaining of child’s bad breath & loose teeth
History of main complaint: 1 year. Previously attended a private dentist who had done cleanings & prescribed antibiotics
Medical history: NKDA, med history unknown
-eczema on hands and feet from an early age, been treated by general practitioner with aqueous cream since early childhood
Family history: NC
Dental history & habits: Patient brushes 1x per day, no other dental aids used
(Initial presentation seen in image)
Diagnosis: Papillon-Lefévre Syndrome
Disease caused by mutations in CHS1 LYST gene:
Chediak-Highashi Syndrome
Chediak-Higashi Syndrome is caused by a mutations in:
CHS1 LYST gene
The mutation in Chediak-Higashi Syndrome (CHS1 LYST gene) results in what lysosomal defect?
Lysosomal trafficking defect
In Chediak-Higashi Syndrome, defects in melanin granules results in:
partial oculocutaneous albinims
What results in the partial oculocutaneous albinisim 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 is this caused by?
Circulating leukocytes exhibit defective lysosomes resulting in a decrease in phagocytosis
Chediak-Higashi Syndrome is associated with varying neurologic problems including:
- intellectual deficit
- dementia
Describe the oral manifestations of Chediak-Higashi Syndrome:
- severe gingival inflammation
- early-onset & rapidly progressive alveolar bone loss
- early loss of the primary & permanent teeth
- poor response to dental treatment
Caused by mutations in the ELANE (50%) & HAX1 (10%) gene:
Congenital neutropenia
Congenital neutropenia is caused by a mutation in the _____ gene 50% of the time and the _____ gene 10% of the time
ELANE; HAX1
In Congenital neutropenia _____ of the causes remain unknown
1/3
Congenital neutropenia results in a ____ number of ____
decrease; neutrophils
What neutrophil value 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 a disease affecting the _____ & ____
oral mucosa & gingival tissue
Epidermolysis bullosa (Kindler syndrome) is caused by a mutation in the:
Fermitin family homologue 1 gene
Disease caused by a mutation in the Fermitin family homologue 1 gene:
Epidermolysis bullosa (Kindler syndrome)
Epidermolysis bullosa (Kindler syndrome) is caused by a mutation in the ferritin family homologue 1 gene that causes:
- A lack of ______
- Affects _______
- Can lead to molecular defects in the ______
- integrin activation
- keratinocyte adhesion
- basement membrane zone
Epidermolysis bullosa (Kindler syndrome) is caused by a mutation in the ferritin family homologue 1 gene that causes:
- A lack of integrin activation
- Affects keratinocyte adhesion
- Can lead to molecular defects in the basement membrane zone
All of these 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
The mutations in genes coding fibrillar collagens or enzymes involved in the biosynthesis of these proteins in Ehlers-Danlos syndrome affects:
collagen production & compositions
In Ehlers-Danlos syndrome the collagen production and compositions are affected mainly in:
- joints
- skin
- walls of blood vessels
What is clinically seen in Ehlers-Danlos syndrome? (3)
- joint hypermobility
- skin extensibility
- tissue fragility
The following images are characteristic of:
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
Hypophosphatasia is categorized as a:
metabolic and endocrine disorder
Caused by a mutation in the gene alkaline phosphatase (ALPL):
Hypophosphatasia
Hypophosphatasia is caused by a mutation in:
Alkaline phosphatase (ALPL) gene
There are both _____ & ____ forms of hypophosphatasia
mild & severe
Describe the oral manifestations of hypophosphatasia:
(3)
- defective cementum
- alveolar bone loss
- premature loss of teeth
- impaired bone/tooth mineralization
- compromised periodontal attachment
In hypophosphatasia, patients have a compromised _____, resulting in reduction of the alveolar bone height
compromised periodontal attachment
In hypophosphatasia, 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 the 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 glycylation end products
-where there is excess amount of glucose in the circulation, everything gets glycated (once glycated its no longer a simple molecule)