Rheumatology II Flashcards
Name some inherited non-autoimmune bone disorders
- osteogenesis imperfecta
- Cleidocranial Dysplasia
- Osteopetrosis
Name some acquired non-autoimmune bone disorders
- Osteomalacia and rickets
- Osteoporosis
- Pagets disease
- Osteomyelitis
What is osteogenesis imperfecta and what kind of inheritance does it show
- Rare, autosomal dominant inherited condition
- Brittle bones susceptible to fracture due to defective type 1 collagen formation
Describe the action of osteoblasts in osteogenesis imperfecta
Osteoblasts active but small, mostly woven bone formation
Describe the clinical features of osteogenesis imperfecta
- 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
What can be used to confirm diagnosis of osteogenesis imperfecta
- Positive family history
- Clinical presentation
- Radiographs
- Skin biopsy
- DNA-based sequencing
What are the management options for osteogenesis imperfecta
- No cure
- Supportive therapy to decrease fractures, cope with disability and maintain overall health
- Bisphosphonates to prevent bone loss
What are the dental relevances of osteogenesis imperfecta
- 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
What is cleidocranial dysplasia and what kind of inheritance does it show
Rare autosomal dominant trait on chromosome 6
Defect of membrane bone formation e.g. skull and clavicle
What are the clinical features of cleidocranial dysplasia
- 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
What are some things that can confirm the diagnosis of cleidocranial dysplasia
- Clinical findings - classical dysostotic features
- Radiographic imaging
What are the dental relevances of cleidocranial dysplasia
- 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
What is osteopetrosis and what kind of inheritance does it show
- Rare genetic disorder characterised by excessive bone density
- Defective osteoclastic activity and remodelling
- Dense but weak and fragile bone, heal normally
What are the clinical features of osteopetrosis
- 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
What investigations indicate osteopetrosis
- Radiographs - dense (marble like) bone appearance
- Ca2+ and PO4^3- usually normal
What are the dental relevances of osteopetrosis
- Frontal bossing and hypertelorism
- Trigeminal or facial neuropathies can be a complication
- Jaw fracture
- Anaemia
- Delayed tooth eruption
- Osteomyelitis can be a complication
What is osteomalacia and rickets and what is the difference
- Inadequate mineralisation of bone framework (children-rickets)
- Failure of mineralisation of replacement bone in normal bone turnover (adults-osteomalacia)
What are some risk factors of osteomalacia and rickets
- Vitamin D deficiency
- Pigmented skin, sunscreen or concealing clothing, elderly, institutionalised
- Calcium malabsorption
- Renal diseases
- Drugs
- Pregnancy and lactation
What are the clinical features of osteomalacia and rickets
- Weak, hypotonic muscles
- Bone pain
- Fractures
- Bone deformity and impaired growth in children
- Hypocalcaemia, tetany and seizures
- Swellings at costochondral junctions ‘Rachitic rosary’
What can be used for the diagnosis of osteomalacia and rickets
- Radiology - pseudo fracture (looser’s zones)
- PO4, Ca2+ levels low or normal
- ALP usually raised
- PTH can be raised
- Low vitamin D
What management options for osteomalacia and rickets are there
- Treatment of underlying causes
- Vit D and calcium supplements
What are the dental relevances of osteomalacia and rickets
- Delayed eruption only in severe cases
- If associated with malabsorption syndrome, may cause vitamin K deficiency and secondary hyperparathyroidism
What is osteoporosis
- Common in elderly
- Diminished bone mass leads to fragility and increased risk of fracture
- Usually affects the spine, forearm and hips
What are some modifiable risk factors of osteoporosis
- Smoking
- Alcohol abuse
- Low dietary calcium intake
- Vitamin D insufficiency
- Lack of physical activity
- Drugs - steroids, anticoagulants and anticonvulsants
What are some non-modifiable risk factors of osteoporosis
- Old age
- Sex female (post menopause)
- Race - caucasians and asians
- Family history
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
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
What management options are there for osteoporosis
- Modifiable risks avoidance
- Dietary advice: calcium and vitamin D intake
- Bisphosphonates
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
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
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.
Describe how direct/contiguous osteomyelitis occurs
- Usually due to local infection caused by trauma or surgery
- Tends to involve multiple bacteria
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.
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
What is the treatment for osteomyelitis
Antibiotics: flucloxacillin and fusidic acid for 4-6 weeks starting with IV route
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
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
What are the commonly affected sites of pagets disease of bones
Pelvis Lumbar Spine Femur Thoracic spine Skull Tibia
What is pagets disease of bones also known as
Osteitis Deformans
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
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
What treatment is there for pagets disease of bones
Bisphosphonates
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
What is osteoarthritis characterised by
- Degeneration of the articular cartilage
- Thickening of the exposed underlying bone
- Development of peri-articular cysts
- Joint deformation
What are the risk factors of osteoarthritis
- Age
- Gender
- Genetic
- Obesity
- Occupation
- Fracture through a joint
- Congenital joint dysplasi
- Paget’s disease and gout
What are the clinical features of osteoarthritis
- Joint pain, worse on movement, relieved by rest
- Stiff joint
- Deformity
- Loss of function
- Herberden’s nodes
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
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
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
What can be done to confirm diagnosis of fibrous dysplasia
- Ground glass appearance on radiograph
- Serum calcium and phosphate normal, alkaline phosphatase raised
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
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
