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CHHD > Rheumatology II > Flashcards

Rheumatology II Flashcards

1
Q

Name some inherited non-autoimmune bone disorders

A
  • osteogenesis imperfecta
  • Cleidocranial Dysplasia
  • Osteopetrosis
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2
Q

Name some acquired non-autoimmune bone disorders

A
  • Osteomalacia and rickets
  • Osteoporosis
  • Pagets disease
  • Osteomyelitis
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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

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4
Q

Describe the action of osteoblasts in osteogenesis imperfecta

A

Osteoblasts active but small, mostly woven bone formation

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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
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6
Q

What can be used to confirm diagnosis of osteogenesis imperfecta

A
  • Positive family history
  • Clinical presentation
  • Radiographs
  • Skin biopsy
  • DNA-based sequencing
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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
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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
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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

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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
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11
Q

What are some things that can confirm the diagnosis of cleidocranial dysplasia

A
  • Clinical findings - classical dysostotic features

- Radiographic imaging

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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
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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
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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
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15
Q

What investigations indicate osteopetrosis

A
  • Radiographs - dense (marble like) bone appearance

- Ca2+ and PO4^3- usually normal

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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
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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)
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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
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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’
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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
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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
Q

What are some non-modifiable risk factors of osteoporosis

A
  • Old age
  • Sex female (post menopause)
  • Race - caucasians and asians
  • Family history
26
Q

What are the clinical features of osteoporosis

A
  • Fractures typically at the thoracic and lumbar vertebrae, head of femur and distal radius (collet fracture)
  • Kyphosis and height shrinkage can occur
27
Q

What investigations can be done to indicate osteoporosis

A
  • 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
Q

What management options are there for osteoporosis

A
  • Modifiable risks avoidance
  • Dietary advice: calcium and vitamin D intake
  • Bisphosphonates
29
Q

What are the dental relevances of osteoporosis

A
  • GA may be contraindicated in deformed chest and vertebral collapse
  • Jaw osteoporosis can cause excessive alveolar loss, especially in women
  • Bisphosphonates and MRONJ
30
Q

What is osteomyelitis

A

Infection of the bone most commonly by staphylococcus, others like haemophilia influenza and salmonella.
Source can be haematogenous or direct/contiguous inoculation

31
Q

Describe how haematogenous osteomyelitis occurs

A
  • 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
Q

Describe how direct/contiguous osteomyelitis occurs

A
  • Usually due to local infection caused by trauma or surgery

- Tends to involve multiple bacteria

33
Q

What are the clinical features of osteomyelitis

A
  • 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
Q

What can be used to confirm the diagnosis of osteomyelitis

A
  • 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
Q

What is the treatment for osteomyelitis

A

Antibiotics: flucloxacillin and fusidic acid for 4-6 weeks starting with IV route

36
Q

What are the dental relevances of osteomyelitis

A
  • 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
Q

What is paget’s disease of bones

A
  • 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
Q

What are the commonly affected sites of pagets disease of bones

A 
Pelvis
Lumbar Spine
Femur
Thoracic spine
Skull
Tibia
39
Q

What is pagets disease of bones also known as

A

Osteitis Deformans

40
Q

What are the clinical features of pagets disease of bones

A
  • 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
Q

What can be used to confirm the diagnosis of paget’s disease of bones

A
  • Radiographs
  • Radionuclide bone scans
  • Raised alkaline phosphatase
  • Normal calcium and phosphate
42
Q

What treatment is there for pagets disease of bones

A

Bisphosphonates

43
Q

What are the dental relevances of pagets disease of bones

A
  • 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
Q

What is osteoarthritis characterised by

A
  • Degeneration of the articular cartilage
  • Thickening of the exposed underlying bone
  • Development of peri-articular cysts
  • Joint deformation
45
Q

What are the risk factors of osteoarthritis

A
  • Age
  • Gender
  • Genetic
  • Obesity
  • Occupation
  • Fracture through a joint
  • Congenital joint dysplasi
  • Paget’s disease and gout
46
Q

What are the clinical features of osteoarthritis

A
  • Joint pain, worse on movement, relieved by rest
  • Stiff joint
  • Deformity
  • Loss of function
  • Herberden’s nodes
47
Q

What are the management options of osteoarthritis

A
  • Regular exercise
  • Weight control
  • Good footwear
  • Use of a walking stick
  • Heat/cold
  • Medications include: NSAIDs, antidepressants, intra-articular injections
  • Surgery
48
Q

What are the dental relevances of osteoarthritis

A
  • Dental care access complicated by age and immobility
  • Bleeding tendency due to aspirin
  • Reduced manual dexterity
  • TMJ involvement
49
Q

What is Fibrous dysplasia characterised by

A
  • Replacement of an area of bone by fibrous tissue

- Localised swelling affecting single bone - mono static or less commonly several bones - polyostotic

50
Q

What can be done to confirm diagnosis of fibrous dysplasia

A
  • Ground glass appearance on radiograph

- Serum calcium and phosphate normal, alkaline phosphatase raised

51
Q

What are the management options of fibrous dysplasia

A
  • 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
Q

What are the dental relevances of Fibrous dysplasia

A
  • Facial bones frequently involved in monostotic type and in 25% of polyostotic
  • Hyperthyroidism and diabetes may be associated with the polyostotic type

CHHD (27 decks)

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