Manifestation of Systemic Diseases Flashcards
What is the definition for periodontitis as a manifestation of systemic disease?
Diseases and conditions that can affect the periodontal tissues by influencing the course of periodontitis
What is the definition for systemic diseases and conditions affecting the periodontal supporting tissues?
Diseases and conditions that can affect the periodontal tissues by affecting the periodontal supporting tissues independently of dental plaque biofilm‐induced inflammation
What is a major impact?
Severe/early-onset periodontitis
What is a moderate impact?
- 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
What types of genetic disorders have major impacts on periodontal inflammation?
- Diseases associated with immunologic disorders
- Diseases affecting the oral mucosa and gingival tissue
- Diseases affecting the connective tissues
- Metabolic and endocrine disorders
What are examples of genetic diseases associated with immunologic disorders?
- down syndrome
- leukocyte adhesion deficiency
- papillon-lefevre syndrome
- chediak-higashi syndrome
- congential neutropenia
What is down syndrome?
- Trisomy 21
- Characteristic physical appearance
- Mental deficiency and growth retardation
How does down syndrome affect the periodontium?
- Moderate to severe periodontitis with rapid progression
- Local factors alone fail to explain the severity of periodontal destruction
- Intrinsic immune system defects
How does patients with down syndrome recieve periodontal treatment?
- Scaling & root planing and flap surgery have been shown to be equally effective in improving CAL and in reduction of PD’s.
- Patients have impaired PMN function, but this does not appear to affect how they respond to periodontal therapy.
- Frequent periodontal maintenance and oral hygiene are vital in maintaining periodontal health
What is leukocyte adhesion deficiency?
- 3 types of Leukocyte Adhesion Deficiency
— LAD -1: CD11/18 gene mutation
— LAD-2: FUT8 gene mutation
— LAD-3: ITGA4 gene mutation - Deficiency in integrins
— Neutrophils cannot adhere to blood vessel walls at site of infection
— Neutrophils cannot migrate into affected tissues - History of severe recurrent infection but no purulence
- Diagnosis based on clinical symptoms, family history, and laboratory tests
How does leukocyte adhesion deficiency affect the periodontium?
- Severe gingival inflammation, acute gingival lesions
- Early-onset and rapidly progressive alveolar bone loss
- Early loss of the primary and permanent teeth
- Systemic treatment by physician with bone marrow or stem cell transplant helps in periodontal treatment outcome
What is papillon-lefevre syndrome?
- Mutations in the cathepsin C gene (CTSC)
— Chromosome 11q14 - Compromised neutrophil function
— Decreased phagocytosis (Rathi, 2002) - Hyperkeratotic lesions
— Palms, soles of the feet, elbows, knees
How does papillon-lefevre syndrome affect the periodontium?
- Severe gingival inflammation
- Early-onset and rapidly progressive alveolar bone loss
- Early loss of the primary and permanent teeth
What is chediak-higashi syndrome?
- Mutations in CHS1, LYST gene
— Lysosomal trafficking defect - Partial oculocutaneous albinism
— Defects in melanin granules - Recurrent pyogenic infections
— Circulating leukocytes exhibit defective lysosomes
—Decrease in phagocytosis - Varying neurologic problems
— Intellectual deficit and dementia
How does chediak-higashi syndrome affect the periodontium?
- Severe gingival inflammation
- Early-onset and rapidly progressive alveolar bone loss
- Early loss of the primary and permanent teeth
- Poor response to dental treatment
- Patients who undergo bone marrow transplant early in life are less susceptible to aggressive periodontitis
What is congential neutropenia (kostmann disease)?
- Mutations in the ELANE (50%), HAX1(10%) gene
— 1/3 causes remain unknown - Decrease in number of neutrophils
— ANC < 500 cell/uLand static - Deficiency in the immune response
— Severe and recurrent infections - Treated with Granulocyte Colony-Stimulating Factor (G-CSF)
— Patients still can have gingivitis and periodontal disease when taking this, but they do not get severe systemic infections
How does congenital neutropenia affect the periodontium?
- Severe periodontitis is common
- Higher risk for tooth loss
- Oral ulcers
What is the example of genetic disease affecting the oral mucosa and gingival tissue?
- epidermolysis bullosa
What is epidermolysis bullosa (kindler syndrome)?
- Mutation in the fermitin family homologue 1 gene
— (kindlin-1; FERMT1) - Lack of integrin activation
— Affect keratinocyte cell adhesion
— Lead to molecular defects in basement membrane zone - Photosensitivity
- Recurrent blister formation
How does the epidermolysis bullosa affect the periodontium?
- Oral blisters formation
- Severe periodontitis
— Alveolar bone loss that progresses rapidly - Need immunofluorescence to confirm diagnosis
— Skin biopsy of an included blister
What is the example of genetic disease affecting the connective tissues?
ehlers-danlos syndrome
What is ehlers-danlos syndrome?
- Mutations in genes encoding fibrillar collagens or enzymes involvedin the biosynthesis of these proteins
- Affecting collagen production and composition
— Mainly involves joint, skin, and walls of the blood vessels - Joint hypermobility, skin extensibility, and tissue fragility
How does the ehlers-danlos syndrome affect the periodontium?
- Generalized, early-onset severe periodontitis and gingival recession
- Early loss of the primary and permanent teeth