connective tissue disorders Flashcards
systemic lupus erythematous
- m:f
- onset
90% females
20-80
clinical features of LUPUS 12
- fever
- weight loss
- mild lymphadenopathy
4.arthritis-arthralgia
5.raynaud’s - skin
-photosensitive facial rash
-erythematous
-malar rash
or discoid rash
-livedo reticularis (antiphos syndrome)
6.kidney
-proliferative glomerulonephritis
7.cardio
-pericarditis
-myocarditis
-endocarditis
-arthersclerosis
8.lung
-pleuritic pain
-pneumonitis
-breathlessness
dvt risk
8. headaches
- haematological
- neutropenia
- lymphopenia
- thrombocytopenia - gi
- mouth ulcers
- peritoneal serositis
- mesenteric vasculitis
Renal proliferative glomerular nephritis
which of the above predicts severity
renal involvement
haematuria, proteinuria and cast
what is anti-phospholipid syndrome and what to give
more miscarriages more risk thrombosis and stroke heart valve disease low platelet count give warfarin Livedo reticularis also seen
11 criteria of lupus
malar rask discoid rash photosensitive skin rash oral ulcers arthritis: 2 or more joints serositis: pericarditis, pleuritis renal disorder >0.5g in 24 hrs neuro disorder: seizure psychosis haem disorder 4/11
blood investigation results for SLE 5
Anti ds DNA ENA antibodies or Ana - anti la -anti ro -anti sm low c3 or c4 raised esr
but normal crp
management of SLE
analgesic and nsaid hydroxychloroquine cyclophosphamide steroids dmards biologicals
sle with severe renal management
mycophenylate and steroids
main bio for sle
belimumab
advise for sle
stop smoking
control bp and lipids
management antiphos syndrome
give warfarin
management osteop
adcal
what is systemic sclerosis
autoimmune disorder of connective tissue causing fibrosis
symptoms of systemic sclerosis
crest calcinosis: calcium depositis in skin r: Raynaud's e: oesophageal dysmotility s: sclerodactyly: thickening of toes and fingers t: telangiectasia -oedema -capillary loss -arthralgia due to skin -gi involvement stomach and oesophagus -pulmonary: ILD -renal hypertensive renal crisis
f:m for systemic s
4:1
onset of ss
4th and 5th decade
poorer prognosis for ss
diffuse only 5 year survival older age higher esr proteinuria low gas transfer factor for Carbon monoxide pulmonary hypertension
gene assoc. to connective tissue disease
HLA locus
inx of ss
-ANA antibodies positive or anti ENA
Which is anti scl 70
- dss antibodies to topoisomerase 1
- ISS anticentromere antibodies
- cxr for ILD
- barium swallow
management of ss
no treatment
- calcium channel blockers: losartan, fluoxetine
- prostacyclin IV for severe disease
- bosentran: digital ulcers
- ppi for oesophagus
mixed connective tissue disease includes
ss
myositis
sle
symptoms of mixed connective tissue disease
puffy fingers dactylitis sclerodactyly Raynaud myalgia
antibody in mixed connective
anti RNP one of the anti ENA/ana
primary sjorgen syndrome onset
40-50
female to male ratio sjorgen
9:1
gene for sjorgen
HLA-B8/DR3
clinical symptoms of sjorgen 3 main
dry eyes -conjunctivitis -blepharitits dry mouth -dental carie arthritis fatigue
word for dry mouth
xerostomia
other clinical features of sjorgen
lymphoma fever anaemia thrombocytopenia lymphadenopathy ILD
antibodies for sjorgen
RF ANA ssa anti ro ssb anti la gastric parietal cell thyroid
investigations of sjorgen
schrimer tear test
- 5 minute test
- esr
- lymphocyte
- antibodies
management of sjorgen
- lacrimal substitutes: hypromellose
- saliva tablets
- oral hygiene
- pilocarpine for gland function
- hydroxychloroquine for skin and msk
what is sox syndrome
sialadentitis (inflamed saliva glands)
oa
xerostomia
Onset sle
15 to 40
