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Connective tissue disorders

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tomi1's version from 2017-02-09 13:25

Section 1

Question Answer
CTD definition autoimmune multi-organ inflammatory disease
6 groups of CTDvascultides, myositis, SLE, APS, Systemic sclerosis, Sjogren's
drug historyisoniazid,hydrazaline, OCC
medical historyHypothryodism (coexists with SLE)
arthalgia in CTDSLE = polyarthalgia + non-erosive arthritis (no bone or cartilage changes)
secondary vasculitiscomplication of RA,SLE,infection,malignancy
kidney functionurine dip - proteinuria/ red cell casts
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Section 2

Question Answer
SLE geneticsHLA DR3
Other AI associated with SLERA, Diabetes, Pernicious anaemia, Hashimoto's thyroiditis
SLE presentationjoint pain (90%), cutaneous changes(85%), plerisy/pericarditis (50%), GMN (40%),CNS(30%-epilepsy 12%),Raynauds (8%)
Alopecia is a feature inSLE
Epidemiology SLEblacks -Asians-females
Age of onsetbimodal- adolescence or >50yrs
Mgx SLEavoid UVB-NSAIDs-prednisolone-hydrochloroquine-MTX (depending on disease activity)
avoid in SLEUVB sunlight
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Section 3

Question Answer
Types of Sclerodermalimited or diffuse
Organs affectedskin, fingers, kidney (renal failure), lungs (pulmonary fibrosis)
Lungs in diffuse vs limitedfibrosis vs PVH
Treatment Sclerodermastop smoking, avoid cold, ACEI (BP), PPI (dyspepsia)
Scleroderma prominent featureRAYNAUD'S PHENOMENON (all Scleroderma patients have raynaud's
RAYNAUD definecolour change extremities in response to cold
Colour changes in RAYNAUD'Swhite (ischemic)- blue(hypoxia)-red(re-perfusion)
RF RAYNAUD'Svinyl chloride
Causes of Raynaud's by incidenceidiopathic- Scleroderma-SLE-sjogren's
Rx RAYNAUD'Savoid smoking, cold, nifidepine (CaCB- dizzy/headaches)
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Section 4

Question Answer
Sjogren featuresdry eyes (keratoconjunctivitis sicca) + dry mouth (xerostomia)
Anti phospholipid featuresdvt/pe + pregnancy related mortality (spont abortions, premature birth)
Limited systemic sclerosis features pulmonäry hypertension + CREST = calcinosis, reynauds,eosphageal dysmobility, sclerodactyl, telangectasia
Diffuse systemic sclerosis featuresinterstial lung disease + scleroderma renal crisis (emergency=rapidly progressive renal failure)
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Section 5

Question Answer
large vessel vasculitisGCA + Takaysau
features of GCAcoexist with PMR- jaw claudication-headache -amurosis fugax
GCA is an emergencyblandness if untreated
Genetic component GCAHLA-DR4 (Scandinavian)
Bloods if suspect GCAESR, ALP raised
features of PMRsymmetrical proximal muscle weakness, morning stiffness, >2months
Rx PMRrapidly improves with steroids (ex-football coach with shoulder pain)
dd PMRRA (if arthralgia does not respond to steroids)
danger untreated GCAarteritis of posterior cilary artery- ischaemic optic neuritis
management GCA20mg prednisolone
features of takaysauyoung women-aortic arch-headache/claudication/BP difference in arms
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Section 4

Question Answer
medium vessel vasculitisPAN + Kawasaki
features of PANmen-hep B- bleeding skin/gut/kidneys
features of kawasakikids-strawberry tongue/red eyes/rash/shedding skin-cervical nodes- rx:low dose aspirin
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Section 5

Question Answer
small vessel vasculitiswegner's + churg strauss + henoch schonlein
wegner'sgranulomatous polyangitis - lungs/kidneys/SADDLE NOSE
churg strausseosinophilic granuloma- asthma/ rhinitis/ eosinophils
henoch schonleinkids lower limb rash
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Section 6

Question Answer
2 blood tests immunological complexesANA (anti-nuclear antibody-present in autoimmune disease) + ENA (extractable nuclear antibody-screens for the autoantibodies)
SLEANA-dsDNA-anti ro-anti Lo-anti-sM
drug induced SLEanti-histone
Anti-phospholipid syndromeanti-cardiolipins + anti-lupus
Myostisanti-Jo1 (anti hystidyl tRNA synthetase)
wegner'sANCA PR3
churg straussANCA MPO
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Scleroderma: ANA
Question Answer
Limited (CREST)anti-centromere
diffuse systemic sclerosisanti-toipomerase or anti-scl 70
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