SLE and Co Flashcards
define systemic lupus erythematosus?
Chronic multi system autoimmune inflammatory disorder
causes of SLE?
- idiopathic
- sex hormone status (pre-menopausal women + men with kleinfelters XXY)
- genetics (HLA-B8 D2 & 3)
- UV lights can cause skin flare
- EBV exposure may be a trigger
What drugs can cause SLE? why is this presentation of SLE different from others?
- transient SLE that resolves on drug removal
- hydrasalazine, phneytonin, penicillamine, isonizad
Clinical features of SLE?
- joints - small joint arthralgia/arthritis
- systemic - tiredness/fatigue
- skin - buttefly rash, photosensitivty
- blood - anaemia
- lungs - pleural effusion, fibrotic alveolitis
- CVS - VTE, vasculitis, raynauds, pericarditis
- kidney - glomerulonephritis
- neuro- depression, epilepsy, psychosis
- other - miscarriage, mucosal ulcer
Most useful anti-bodies for SLE?
- Anti-dsDNA
- Anti-ANA
-less useful RF, smith, Ro, La
Other useful blood test for ACTIVE SLE disease?
- C3 and C4 are reduced in active disease because of complex formation
- ESR high
- CRP normal
- regular urinalysis - IgG nephritis
Tx for SLE?
dependent on disease severity
- education and RF reduction (smoking cessation)
- NSAIDs - mild arthralgia
- hydroxychloroquine - mild disease when NSAIDS don’t work
- Corticosteroids
- steroid sparing agents - azothioprine, MTX
- rituximab - severe disease
What is anti-phospholipid syndrome?
acquired autoimmune condition commonly associated with SLE with these common features:
- VTE
- prolonged PTT
- recurrent miscarriages
- persistently +ve antiphospholipid antibodies
- net-like rash - livedo reticularis
- pre-eclampsia
- thrombocytopenia
- pulmonary HTN
Commonly presents as clots in either arteries, veins or placenta. What would be there respective presentations?
- artery = Stroke, TIA, ACS
- Vein = DVT, PE, budd-chairri syndrome (hepatic microclots)
- placenta = recurrent miscarriage
Mnemonic for anti-phospholipid tings?
CLOT Clots of the blood Livedo reticularis Obsetric complications/haemorrhage Thrombocytopenia
1 or more of the antiphospholipid antibodies must be persistently present for a diagnosis of APS. What are they? (3)
- anticardiolipin
- lupus anti-coagulant antibodies
- beta-glycoprotein antibodies
What is the treatment for antiphospholipid syndrome if: first VTE? Recurrent VTE? clot whilst on anticoagulant? Pregnancy? syndrome present but no VTE?
first VTE - warfarin for 6 months INR 2-3
recurrent VTE - life long warfarin INR 2-3
clot whilst on warfarin - increased target INR to 3-4
pregnancy - LMWH injections
no VTE Hx - aspirin or clopidogrel
Sjorgens: Cause? symptoms? Investigation? Treatment?
cause - autoimmune destruction of exocrine cells/glands
symptoms - dry anything that can be (tears, saliva (+enlarged glands), vagina, joints (arthralgia))
Investigations - auto-antibodies (RF +ve (100%), ANA, Ro, La) + schimer test paper gets less tear wet
Treatment - replacement saliva, tears, pilocarpine may stimulate tears/saliva
There’s diffuse scleroderma and limited scleroderma. What is limited scleroderma otherwise known as? what does it’s acronym stand for?
limited scleroderma = CREST syndrome or limited cutaneous scleroderma
Calcinosis - Ca2+ deposits in the skin
Raynaud’s phenomena
Esophageal dysfunction -GORD
Sclerodactyly - thickening and tightening of the hand skin
Telangiectasia - dilation of superficial vessels causing red spots
There’s diffuse scleroderma and limited scleroderma. What’s the Symptoms of diffuse? Other than distribution of symptoms how does it differ from limited?
Skin symptoms happen much more rapidally in diffuse scleroderma and involve other organs early too
- Skin - as per cutaneous scleroderma
- GI - CORD
- renal - AKI and CKD
- lung - fibrosis + icnreased vascular resistance = PHTN
- arrhythmias + conduction blocks
