Pathology of Soft Tissues Flashcards
Duchenne inheritance
X-linked recessive
Becker inheritence
X-linked recessive
Limb girdle inheritance
Autosomal recessive
Fascioscapulohumeral inheritance
Dominant
Scapulohumeral inheritance
Autosommal recessive
Oculopharyngeal inheritance
Dominant
Myotonic dystrophy inheritance
Dominant
Mutations in dystrophin gene on long arm chromosome X
Alterations in anchorage of actin cytoskeleton to basement membrane
Fibres liable to tearing
Uncontrolled Ca2+ entry into cells
DMD
Muscle weakness
Myotonia
Non-muscle features (frontal balding, cardiomyopathy, low intelligence)
Myotonic Dystrophy
Atrophy of type 1 fibres
Central nuclei
Ring fibres
Fibre necrosis
Fibrofatty replacement
Myotonic dystrophy
Endomysial lymphocytic infiltrate, invasion of muscle by CD8 + T lymphocytes
Polymyositis
T-cells in polymyositis?
CD8+
Upper body erythema, swelling of eyelids with purple discolouration
Polymyositis
Weakness
Proptosis
Fatigue
Dysphagia
Myasthenia Gravis
Classical triad of rhabdomyolysis?
Myalgias
Generalised weakness
Darkened urine
Myoglobinuria
Hyperkalaemia
Necrosis & shock
Rhabdomyolysis
Outcome:
Acute Renal Failure (breakdown products of skeletal muscle and myoglobin are harmful to the kidneys)
Hypovolaemia & hyperkalaemia
Metabolic acidosis
Disseminated intravascular coagulation
Outcome of rhabdomyolysis?
Acute renal failure Hypovolaemia Hyperkalemia Metabolic acidosis Disseminated intravascular coagulation
Drugs which can cause SLE?
Hydralazine
Pracainamide
Visceral lesions of SLE which type of hypersensitivity?
Type 3
Haematological effects of SLE mediated by which type of sensitivity?
Type II
Clinical features of PAN
Clinical features
Non-specific +/- organ specific features such as hypertension, haematuria, abdominal pain, melaena, diarrhoea, mononeuritis multiplex, rash, cough, dyspnoea
Clinical features
Non-specific +/- organ specific features such as hypertension, haematuria, abdominal pain, melaena, diarrhoea, mononeuritis multiplex, rash, cough, dyspnoea
PAN, lol
Biopsy; fibrinoid necrosis of vessels
Serum contains pANCA (perinuclear antineutrophil cytoplasmic autoantibody)
PAN
Biopsy findings of PAN
Fibrinoid necrosis
pANCA
Causes of death because of scleroderma
Renal failure secondary to malignant hypertension
Severe respiratory compromise
Cor pulmonale
Cardiac failure or arrhythmias secondary to myocardial fibrosis
Scleroderma and fibrosis of organs
Excessive fibrosis of organs and tissues (excessive collagen production)
Skin – tight, tethered, joint movement
GI tract – fibrous replacement of muscularis
Heart – pericarditis and myocardial fibrosis
Lungs – interstitial fibrosis
Kidneys – affects arteries, leads to hypertension
Musculoskeletal system – polyarteritis and myositis
Crest syndrome
Calcinosis Raynaud’s Oesophageal dysfunction Sclerodactyly Telangectasia
