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