Rhematology Flashcards
What is the MOA of sulphasalazine?
Sulfasalazine is a prodrug for 5-ASA which works through decreasing neutrophil chemotaxis alongside suppressing proliferation of lymphocytes and pro-inflammatory cytokines.
Name some cautions for sulphasalazine
G6PD deficiency
allergy to aspirin or sulphonamides (cross-sensitivity)
Name some SEs of sulphasalazine
oligospermia
Stevens-Johnson syndrome
pneumonitis / lung fibrosis
myelosuppression, Heinz body anaemia, megaloblastic anaemia
may colour tears → stained contact lenses
What is familial mediterranean fever?
Familial Mediterranean Fever (FMF, also known as recurrent polyserositis) is an autosomal recessive disorder which typically presents by the second decade. It is more common in people of Turkish, Armenian and Arabic descent.
Name some features of FMF
pyrexia
abdominal pain (due to peritonitis)
pleurisy
pericarditis
arthritis
erysipeloid rash on lower limbs
What is Marfan’s syndrome?
Marfan’s syndrome is an autosomal dominant connective tissue disorder. It is caused by a defect in the FBN1 gene on chromosome 15 that codes for the protein fibrillin-1. It affects around 1 in 3,000 people.
Name some features of Marfan’s syndrome
tall stature with arm span to height ratio > 1.05
high-arched palate
arachnodactyly
pectus excavatum
pes planus
scoliosis of > 20 degrees
heart:
dilation of the aortic sinuses (seen in 90%) which may lead to aortic aneurysm, aortic dissection, aortic regurgitation
mitral valve prolapse (75%),
lungs: repeated pneumothoraces
eyes:
upwards lens dislocation (superotemporal ectopia lentis)
blue sclera
myopia
dural ectasia (ballooning of the dural sac at the lumbosacral level)
How do you interpret T and Z scores?
Basics
T score: based on bone mass of young reference population
T score of -1.0 means bone mass of one standard deviation below that of young reference population
Z score is adjusted for age, gender and ethnic factors
T score
> -1.0 = normal
-1.0 to -2.5 = osteopaenia
< -2.5 = osteoporosis
