msk mid tier Flashcards
what do all autoimmune connective tissue disorders share
- All linked by ANA antibody +(antinuclear antibody)
- All relate to immunosuppression
- Can affect any organ
- All relate to inflammation
- Scarring as a result
- Damage is irreversible
sjorgens pathophysiology and symptoms
Lymphocytic infiltration → Exocrine glands (salivary and lacrimal) destroyed → fewer secretions
Symptoms
- Dry eyes
- Dry mouth
- Arthritis
- Vasculitis
- Rash
- Neurological
marfan’s symptoms
Tall Arm span greater than height Long fingers Lens dislocation High arched palate (mouth) Aneurysm -- Collagen in vessels
ehler danos symptoms
Genetic collagen defect
vascular/skeletal types
Joint hypermobility
Skin elasticity
which connective tissue disorders are NOT autoimmune
ehler danos
marfans
sjorgen’s
- investigations
Shermer’s test = paper dipped in eye
Raised ESR and immunoglobulins
Antibodies
- antiRO
- antiLA
- ANA
- RF
sjorgen’s treatment
Replace saliva and tears
Immunosuppression for systemic complications
scleroderma pathophysiology
Increased fibroblast activity → abnormal growth of connective tissue
systemic sclerosis
collagen depsotion, inflammation, autpimmune
vascular damage
scleroderma symptoms
CREST (limited disease)
- Calcinosis = calcium deposition sub-cut (white lumps)
- Raynaud’s (vasculopathy)
- Esophageal dysmotility and strictures and dilated (reflux)
- Sclerodactly = thickened skin
- Telangiectasia = widened venules - spider naevi
\+ tight skin (restriction) ulcers (esp digits) watermelon belly short history of raynauds pulmonary hypertension
more diffuse disease
- Rapid and widespread onset
- Heartburn
- Reflux oesophagitis
- Delayed gastric emptying
- Pulmonary fibrosis and hypertension
- Skin involvement, also proximal - trunk
- Raynaud’s history shorter
- Increased risk of CV
scleroderma investigations
- ACA anticentromere antibodies
- ANA +
- Spirometry and echo to detect pulmonary and arterial hypertension
scleroderma treatment
- PPIs (GORD)
- ACEi - prevent renal crisis
- Vasodilators (raynauds) — CCB
- Monitor for pulmonary hypertension
- Treat skin oedema
- UV protection
dermatomyotis
polymyotis
what is the symptoms and treatment for these both
- skin rash - eyelids, knuckles(Derm)
- muscle weakness (polymyo)
- breathlessness (both)
steroids
immunosuppression
antiphospholipid syndrome =
- pathophys
- who
- presentation
- management
- compli
autoimmune
arterial and venous thrombosis (antiphospholipid antibodies are hypercoaguable)
fertile-age women + if black
presentation = clot-y
management = heparin
compl = clot-y (MI,stroke, miscarriage, DVT etc)
primary bone tumours
- commonness
- age group
rare (secondary more com)
young patients
most common primary bone tumour in children / adults
children - osteosarcoma
adults - chrondrosarcoma
primary bone cancer types
multiple myeloma lymphoma osteosarcoma fibrosarcomas chondrosarcome ewings tumour
osteosarcoma
- age
- where
- disease progression
- 15-19
- knees mainly (humerus, femur)
- destroys bone and spreads to surrounding tissues + metastasises to lung rapidly
- but relatively painless
what bloods might be interesting for bone tumours
- Serum alkaline phosphatase (from bone) raised
- Hypercalcemia occurs in 10-20% of those with metastatic malignancies
- FBC (low something - bone makes stuff?)
- u/e
- PSA = prostate specific antigen. May be raised with prostatic metastases
mirel’s score=
likelihood of fracture
- based on site
- pain
- lesion nature (blastic, lytic, mixed)
- lesion size
enneking system for grading
for primary bone tumours
1A : low grade- intracompartmental 1B: low grade- extracompartmental 2A: high grade -intracompartmental 2B : high grade - extracompartmental 3: metastases
how much bone density must be lost before lytic areas to be seen
60%
onion skinning
layers on edge - periosteum lifted off then bone calcifies sequentially
bone tumours
sunburst spicules
calcification of capillaries supplying tumour
bone tumour
codman’s triange
periosteum lifted off bone
bone tumour
what does the periosteum reveal on a bone tumour scan
- periosteum covers bone.
- comes off with tumour, infection, trauma
- indicates tumour speed of growth
- slow – callus
- fast – interputs process causing layers. may burst through periosteum. (onion skinning, sunburst spicules, codman’s triangle)
secondary bone tumours
- metastasise from where to bone
- nature of these tumours
Lungs, bronchus Breast Prostate - often osteosclerotic too Rarer - Thyroid - kidney
osteolytic (bone resoprption via osteoclast activation by tumour cells (RANK-L binds to RANK…))
chondrosarcoma
- what
- symptoms
- where
cancer of the cartilage
dulll, deep pain
swollen
tender
pelvis
also - femur, humerus, scapula, ribs
red flags for bone tumours
- Suspected infection
- Systemically unwell inc weight loss
- Night pain / rest pain
- – Non-mechanical
- – Constant
- Rapid deterioration
- Structural deformity
- Loss of movement/function
- Lump present
- – Tender, enlarging, deep to fascia, large
- Neurological symptoms
bone tumour presentation
- tender at site
- systemic - weight loss, malaise, fever,
- aches, pains
- ewing’s sarcoma can be associated with paralysis, numbness, incontinence
primary/ secondary tumours sclerotic/lytic
primary = lytic secondary = i think depends on from where
is a well/ill defined zone of transition more optimistic for bone tumours
well defined = more benign
ill defined = malignant, or could be infection
fibromyalgia
- age
- gender
- other risk factors
- assocaited with what
- triggers
- 30-60
- female
- low household income, divorced, low education status
- chronic fatigue, IBS, chronic headaches, depression
- physical trauma, PTSD, infection, distress, hormone alteration
fibromyalgia presentation
- Chronic pain - Responds poorly to analgesia
- Soft tissue tenderness - Usually at multiple sites
- Depression , anxiety
- Fatigue
- Poor concentration
- Sleep disturbance
- Altered bowel habit
- Headache
- parasthesia
fibromyalgia management
- Address social stressors and depression
- Education
— Not “in their head”
— Not destructive arthritis → exercise will not harm joint. Not degenerative like OA - Exercise/ strength programmes- may reduce pain and fatigue
- CBT
- physiotherapy
- accupuncture
- Tricyclic antidepressants eg amitriptyline
may help pain and improve sleep - Anticonvulsants eg pregabalin, gabapenlin (If amitriptyline ineffective)
- Dual reuptake inhibitors eg duloxetine– Good for anxiety/depression
- Antiinflammatory ones have no use
- analgesia but poor response
fibromyalgia investigations
Examination of tender trigger points (need teens of them)
Exclude other diagnoses - all other investigations are normal
mechanical lower back pain
- age
- common
- where
- 20-55
- common
- back :lumbrosacral. buttock/thigh
mechanical back pain
back pain - lumbrosacral buttock/ thigh varies with physica; activity and time normally resolves in 6w may persist/ relapse
mechanical back pain management
Conservative management - Education - Exercise Analgesia NSAIDs Muscle relaxants
osteomalacia treatment
Vitamin D replacement= calciferol
osteomalacia symptoms
- Rickets - bowed legs, knock knees
- Proximal Muscle weakness + pain
- Widespread bone pain (diffuse but esp hips) + tenderness
- Bone weakness–Fractures, esp femoral neck
- Muscle spasm and numbness – due to hypocalcaemia
osteomalacia pathophysiology
- Normal amount of bone but its mineral content is low
- Defective mineralisation
- Excess uncalcified osteoid and cartilage
- Bone mineralisation needs adequate calcium and phosphate
- so bones are soft and weak
how does vit d play a part in osteomalacia
Vitamin d promotes calcium absorption in the intestines and promotes bone resorption (by increasing osteoclast number) - so more for bones
so vit d deficiency is common cause
osteomalacia causes (inc epidemiology!)
Vitamin D def
- Lack of sunlight
- – Dark skin *
- – Elderly *
- – institutionalised
- Poor diet (not enough oily fish /egg yolk)
- Renal disease
- Liver disease
- Some drugs eg anticonvulsants breakdown vit D precursor
Calcium def
Phosphate def
- GI malabsorption
osteomalacia investigations
Blood
- Low calcium and phosphate
- Raised serum alkaline phosphatase
- Elevated parathyroid hormone
- Low 25-hydroxy vitamin D
Biopsy = gold standard
- Incomplete mineralisation in bone
X ray
- Psuedo fractures, low BMD, osteopenia
