Connective Tissue Disease Flashcards
What is SLE?
Systemic lupus erythematosus
Autoimmune inflammatory of the connective tissue
Systemic- affects multi body systems
Erythematosus- characteristic rash
Who gets SLE?
Anyone BUT
Most likely in adult women, of Afro-Caribbean, Hispanic or Asian descent
What is the pathophysiology of SLE?
the immune system attacks body’s own tissues instead of just infections.
Some ppl have genetic tendency to have an overactive immune system, and environmental triggers can set off the overactivity
Inflammation caused by deposition of immune complexes throughout body
How does SLE present?
Hallmark sign: photosensitive butterfly shaped rash
Can also get-
Fatigue and myalgia
Fever
Weight loss
Anaemia
SOB
Ulcers
Delirium
Raynauds
Jaccouds- temporary RA looking fingers
What can SLE cross over with?
Sjorgens syndrome
Systemic sclerosis
SLE autoantibodies
Non specific but always pos: ANA
Specific to SLE:
Anti dsDNA
AntiSM
What investigations would you do for SLE?
FBC- for anemia
CRP and ESR- for active inf
Low C3 and C4
Renal biopsy- for lupus induced nephritis
Autoantibodies
What is systemic sclerosis?
Autoimmune connective tissue disease- causes fibrosis of skin, tissues, and organs
What are the 2 types of systemic sclerosis?
Limited cutaneous and diffuse cutaneous
Key presentation of systemic sclerosis
CREST symptoms-
Calcinosis- subcutaneous calcium deposits
Raynauds- white fingers
oEsophogeal dysmotility- smooth muscle fibrosis
Scleordactyly- skin tightening around joints -> reduced range of motion
Telanglectasia- spider veins
How do lcSS and dcSS differ?
Limited have crest symptoms
Diffuse has crest features but also has systemic effects, ie CVD, lung problems, and renal crisis
lcSS antibodies
Anti centromere
ANA
dcSS antibodies
Anti-SCL-70
ANA
How is systemic sclerosis treated?
Treating symptoms eg NSAIDs, analgesia
PPIs for reflux
Steroids but be weary of risk of SRC
Anti hypertensives
Nifedipine for Raynauds
Lifestyle modification
What is Sjogrens?
Autoimmune condition affecting the exocrine glands, ie salivary and lacrimal
What is the classification for Sjogrens?
Primary- occurs on its own
Secondary- occurs secondary to rheumatoid conditions
How does Sjogrens present?
SICCA SYMPTOMS- umbrella term for dryness of mouth, eyes, and vagina
Tooth decay due to lack of saliva
Blepharitis- eyelid inf
Sjogrens antibodies
Anti RO anti LA
How is Sjogrens diagnosed?
Schirmer test- inserting folded filter paper under lower eyelid with it hanging out- moisture travel via diffusion is measured (expected to be >15mm)
+ antibodies and clinical diagnosis
How is Sjogrens treated?
Artificial tears (polyvinyl alcohol dye and carbomer)
Artificial saliva
Vaginal lubricants
Pilocarpine to stimulate tears and saliva
Hydroxychloroquine for those with joint pain
Complications of Sjogrens
Problems with eyes, mouth, and vagina due to dryness
Can cause complications in other organs like bronchiecstasis, non Hodgkin lymphoma, vascuitis, renal impairment
What is anti-phospholipid syndrome?
Autoimmune disorder that presents with recurrent VTE and/or foetal loss
What causes APS?
Genetic predisposition, associated with SLE
Antibodies associated with APS
Lupus anticoagulant
Anticardiolipin
Anti β2 glycoprotein 1
Key presentation of APS
Female patient with thrombosis (ie VTE or PE) with a history of frequent miscarriage
Treatment for APS
Lifetime anticoag-
Warfarin target INR 2-3
LWMH and aspirin in pregnancy (as warfarin is teratogenic)
How is SLE treated?
Hydoxychloroquine
NSAIDs
Steroids
Sun avoidance due to photosensitivity
DMARDs and biologics in more severe cases
How is Raynauds treated?
Nifedipine
Sildenafil for associated ulcers
