Rheumatology II Flashcards
1
Q
Name some inherited non-autoimmune bone disorders
A
- osteogenesis imperfecta
- Cleidocranial Dysplasia
- Osteopetrosis
2
Q
Name some acquired non-autoimmune bone disorders
A
- Osteomalacia and rickets
- Osteoporosis
- Pagets disease
- Osteomyelitis
3
Q
What is osteogenesis imperfecta and what kind of inheritance does it show
A
- Rare, autosomal dominant inherited condition
- Brittle bones susceptible to fracture due to defective type 1 collagen formation
4
Q
Describe the action of osteoblasts in osteogenesis imperfecta
A
Osteoblasts active but small, mostly woven bone formation
5
Q
Describe the clinical features of osteogenesis imperfecta
A
- Multiple fractures on minimal impact, less after puberty
- Gross deformity and dwarfism due to bone distortion on healing
- Parietal bossing causing eversion of upper part of ear
- May sometimes present with cardiac complications
- Others include deafness, blue sclera, easy bruising, loose/weak tendons and ligaments
6
Q
What can be used to confirm diagnosis of osteogenesis imperfecta
A
- Positive family history
- Clinical presentation
- Radiographs
- Skin biopsy
- DNA-based sequencing
7
Q
What are the management options for osteogenesis imperfecta
A
- No cure
- Supportive therapy to decrease fractures, cope with disability and maintain overall health
- Bisphosphonates to prevent bone loss
8
Q
What are the dental relevances of osteogenesis imperfecta
A
- Handle patients carefully due to bone fragility
- Not to be confused with physical abuse
- Minimal force, support jaws, ensure haemostasis
- Chest deformity can contraindicate surgery
- Dentinogenesis imperfecta
- Bisphosphonates
9
Q
What is cleidocranial dysplasia and what kind of inheritance does it show
A
Rare autosomal dominant trait on chromosome 6
Defect of membrane bone formation e.g. skull and clavicle
10
Q
What are the clinical features of cleidocranial dysplasia
A
- Absent or defective clavicle
- Prognathic mandible due to maxillary hypoplasia
- Depressed nasal bridge
- Frontal, parietal and occipital bossing
- Kyphoscoliosis and pelvic abnormalities may be associated
11
Q
What are some things that can confirm the diagnosis of cleidocranial dysplasia
A
- Clinical findings - classical dysostotic features
- Radiographic imaging
12
Q
What are the dental relevances of cleidocranial dysplasia
A
- Facial anomalies as mentioned in the previous slide
- Deciduous teeth retention causing delayed/failure eruption of permanent teeth
- Hyperdontia, supernumerary teeth
- Twisted roots, malformed crowns
- Dentigerous cyst
13
Q
What is osteopetrosis and what kind of inheritance does it show
A
- Rare genetic disorder characterised by excessive bone density
- Defective osteoclastic activity and remodelling
- Dense but weak and fragile bone, heal normally
14
Q
What are the clinical features of osteopetrosis
A
- May be none in mild cases
- Bone pain, fractures and even osteomyelitis in severe cases
- Infection and anaemia also common
- Cranial neuropathies
- Epilepsy and learning disability possible but rare
15
Q
What investigations indicate osteopetrosis
A
- Radiographs - dense (marble like) bone appearance
- Ca2+ and PO4^3- usually normal
16
Q
What are the dental relevances of osteopetrosis
A
- Frontal bossing and hypertelorism
- Trigeminal or facial neuropathies can be a complication
- Jaw fracture
- Anaemia
- Delayed tooth eruption
- Osteomyelitis can be a complication
17
Q
What is osteomalacia and rickets and what is the difference
A
- Inadequate mineralisation of bone framework (children-rickets)
- Failure of mineralisation of replacement bone in normal bone turnover (adults-osteomalacia)
18
Q
What are some risk factors of osteomalacia and rickets
A
- Vitamin D deficiency
- Pigmented skin, sunscreen or concealing clothing, elderly, institutionalised
- Calcium malabsorption
- Renal diseases
- Drugs
- Pregnancy and lactation
19
Q
What are the clinical features of osteomalacia and rickets
A
- Weak, hypotonic muscles
- Bone pain
- Fractures
- Bone deformity and impaired growth in children
- Hypocalcaemia, tetany and seizures
- Swellings at costochondral junctions ‘Rachitic rosary’
20
Q
What can be used for the diagnosis of osteomalacia and rickets
A
- Radiology - pseudo fracture (looser’s zones)
- PO4, Ca2+ levels low or normal
- ALP usually raised
- PTH can be raised
- Low vitamin D
21
Q
What management options for osteomalacia and rickets are there
A
- Treatment of underlying causes
- Vit D and calcium supplements
22
Q
What are the dental relevances of osteomalacia and rickets
A
- Delayed eruption only in severe cases
- If associated with malabsorption syndrome, may cause vitamin K deficiency and secondary hyperparathyroidism
23
Q
What is osteoporosis
A
- Common in elderly
- Diminished bone mass leads to fragility and increased risk of fracture
- Usually affects the spine, forearm and hips
24
Q
What are some modifiable risk factors of osteoporosis
A
- Smoking
- Alcohol abuse
- Low dietary calcium intake
- Vitamin D insufficiency
- Lack of physical activity
- Drugs - steroids, anticoagulants and anticonvulsants
25
What are some non-modifiable risk factors of osteoporosis
- Old age
- Sex female (post menopause)
- Race - caucasians and asians
- Family history
26
What are the clinical features of osteoporosis
- Fractures typically at the thoracic and lumbar vertebrae, head of femur and distal radius (collet fracture)
- Kyphosis and height shrinkage can occur
27
What investigations can be done to indicate osteoporosis
- Bone density measure by DEXA = gold standard. BMD score < -2.5 is diagnostic
- Conventional radiographs useful for detecting complications from osteopenia but not diagnose osteopenia
- Calcium, phosphate and alkaline phosphatase are normal
28
What management options are there for osteoporosis
- Modifiable risks avoidance
- Dietary advice: calcium and vitamin D intake
- Bisphosphonates
29
What are the dental relevances of osteoporosis
- GA may be contraindicated in deformed chest and vertebral collapse
- Jaw osteoporosis can cause excessive alveolar loss, especially in women
- Bisphosphonates and MRONJ
30
What is osteomyelitis
Infection of the bone most commonly by staphylococcus, others like haemophilia influenza and salmonella.
Source can be haematogenous or direct/contiguous inoculation
31
Describe how haematogenous osteomyelitis occurs
- Bacteria seeding from the blood
- Usually involves a single species of bacteria
- Occurs primarily in children
- Most common site is rapidly growing and highly vascular metaphysic of growing bones.
32
Describe how direct/contiguous osteomyelitis occurs
- Usually due to local infection caused by trauma or surgery
| - Tends to involve multiple bacteria
33
What are the clinical features of osteomyelitis
- Fever, localised bone pain and erythema
If in the jaw:
- Deep seated boring pain with swelling, trismus and regional lymphadenopathy
- Lower lip paraesthesia
- Teeth are tender and may be mobile
- Pus discharges from gingival crevices or from several sinus if cortical plate is penetrated.
34
What can be used to confirm the diagnosis of osteomyelitis
- Usually from the history and clinical findings
- Radiographs will show marked bony destruction in late stages, maybe not in early stages
- Blood test: leukocytosis with neutrophilia, raised ESR
35
What is the treatment for osteomyelitis
Antibiotics: flucloxacillin and fusidic acid for 4-6 weeks starting with IV route
36
What are the dental relevances of osteomyelitis
- Can affect the jaws
- Osteomyelitis of the jaws can be acute or chronic
- Acute: adults, mandible mostly, several causes, few radiographic changes
- Chronic: marked radiographic changes, de novo from low virulent bacteria
- Delay all elective dental treatments until resolution of infection
- Impact of underlying patient's medical condition on dental management
37
What is paget's disease of bones
- Progressive bone enlargement and deformity from osteoclastic and osteoblastic over-activity
- Increased abnormal bone resorption followed by weaker new bone formation (chaotic repair and renewal)
- Increased local bone blood flow and fibrous tissue
38
What are the commonly affected sites of pagets disease of bones
```
Pelvis
Lumbar Spine
Femur
Thoracic spine
Skull
Tibia
```
39
What is pagets disease of bones also known as
Osteitis Deformans
40
What are the clinical features of pagets disease of bones
- Asymptomatic in early stages
- Hands and feet usually spared
- Pain: around hips and knees, most common features in the elderly
- Deformity: skull enlargement, bowed tibia
- Pathological fractures
- Cranial nerves compression
- High output cardiac failure due to bone hypervascularity can occur if disease is widespread
- Osteosarcoma is a rare but possible complication
41
What can be used to confirm the diagnosis of paget's disease of bones
- Radiographs
- Radionuclide bone scans
- Raised alkaline phosphatase
- Normal calcium and phosphate
42
What treatment is there for pagets disease of bones
Bisphosphonates
43
What are the dental relevances of pagets disease of bones
- Patients can develop heart failure so take usual precaution
- Avoid GA if possible
- Hypercementosis may complicate extractions
- Gross symmetrical widening of alveolar ridges
- Poor blood supply may predispose to chronic osteomyelitis
- Sense of hearing, sight and smell may deteriorate
- Osteosarcoma in the jaw is rare but possible complication
44
What is osteoarthritis characterised by
- Degeneration of the articular cartilage
- Thickening of the exposed underlying bone
- Development of peri-articular cysts
- Joint deformation
45
What are the risk factors of osteoarthritis
- Age
- Gender
- Genetic
- Obesity
- Occupation
- Fracture through a joint
- Congenital joint dysplasi
- Paget's disease and gout
46
What are the clinical features of osteoarthritis
- Joint pain, worse on movement, relieved by rest
- Stiff joint
- Deformity
- Loss of function
- Herberden's nodes
47
What are the management options of osteoarthritis
- Regular exercise
- Weight control
- Good footwear
- Use of a walking stick
- Heat/cold
- Medications include: NSAIDs, antidepressants, intra-articular injections
- Surgery
48
What are the dental relevances of osteoarthritis
- Dental care access complicated by age and immobility
- Bleeding tendency due to aspirin
- Reduced manual dexterity
- TMJ involvement
49
What is Fibrous dysplasia characterised by
- Replacement of an area of bone by fibrous tissue
| - Localised swelling affecting single bone - mono static or less commonly several bones - polyostotic
50
What can be done to confirm diagnosis of fibrous dysplasia
- Ground glass appearance on radiograph
| - Serum calcium and phosphate normal, alkaline phosphatase raised
51
What are the management options of fibrous dysplasia
- Usually self-limiting, ceasing progression after adolescence
- Bisphosphonates treatments may have implications
- Testolactone for precocious puberty of Albright's
- Surgery to correct any residual cosmetic defect
- Other endocrine conditions may complicate treatment
52
What are the dental relevances of Fibrous dysplasia
- Facial bones frequently involved in monostotic type and in 25% of polyostotic
- Hyperthyroidism and diabetes may be associated with the polyostotic type
