Connective Tissue Disease

Question 1

What is SLE?

Answer 1

Systemic lupus erythematosus

Autoimmune inflammatory of the connective tissue

Systemic- affects multi body systems
Erythematosus- characteristic rash

Question 2

Who gets SLE?

Answer 2

Anyone BUT
Most likely in adult women, of Afro-Caribbean, Hispanic or Asian descent

Question 3

What is the pathophysiology of SLE?

Answer 3

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

Question 4

How does SLE present?

Answer 4

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

Question 5

What can SLE cross over with?

Answer 5

Sjorgens syndrome
Systemic sclerosis

Question 6

SLE autoantibodies

Answer 6

Non specific but always pos: ANA

Specific to SLE:
Anti dsDNA
AntiSM

Question 7

What investigations would you do for SLE?

Answer 7

FBC- for anemia
CRP and ESR- for active inf
Low C3 and C4
Renal biopsy- for lupus induced nephritis
Autoantibodies

Question 8

What is systemic sclerosis?

Answer 8

Autoimmune connective tissue disease- causes fibrosis of skin, tissues, and organs

Question 9

What are the 2 types of systemic sclerosis?

Answer 9

Limited cutaneous and diffuse cutaneous

Question 10

Key presentation of systemic sclerosis

Answer 10

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

Question 11

How do lcSS and dcSS differ?

Answer 11

Limited have crest symptoms

Diffuse has crest features but also has systemic effects, ie CVD, lung problems, and renal crisis

Question 12

lcSS antibodies

Answer 12

Anti centromere
ANA

Question 13

dcSS antibodies

Answer 13

Anti-SCL-70
ANA

Question 14

How is systemic sclerosis treated?

Answer 14

Treating symptoms eg NSAIDs, analgesia
PPIs for reflux
Steroids but be weary of risk of SRC
Anti hypertensives

Nifedipine for Raynauds
Lifestyle modification

Question 15

What is Sjogrens?

Answer 15

Autoimmune condition affecting the exocrine glands, ie salivary and lacrimal

Question 16

What is the classification for Sjogrens?

Answer 16

Primary- occurs on its own
Secondary- occurs secondary to rheumatoid conditions

Question 17

How does Sjogrens present?

Answer 17

SICCA SYMPTOMS- umbrella term for dryness of mouth, eyes, and vagina
Tooth decay due to lack of saliva
Blepharitis- eyelid inf

Question 18

Sjogrens antibodies

Answer 18

Anti RO anti LA

Question 19

How is Sjogrens diagnosed?

Answer 19

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

Question 20

How is Sjogrens treated?

Answer 20

Artificial tears (polyvinyl alcohol dye and carbomer)
Artificial saliva
Vaginal lubricants
Pilocarpine to stimulate tears and saliva
Hydroxychloroquine for those with joint pain

Question 21

Complications of Sjogrens

Answer 21

Problems with eyes, mouth, and vagina due to dryness

Can cause complications in other organs like bronchiecstasis, non Hodgkin lymphoma, vascuitis, renal impairment

Question 22

What is anti-phospholipid syndrome?

Answer 22

Autoimmune disorder that presents with recurrent VTE and/or foetal loss

Question 23

What causes APS?

Answer 23

Genetic predisposition, associated with SLE

Question 24

Antibodies associated with APS

Answer 24

Lupus anticoagulant
Anticardiolipin
Anti β2 glycoprotein 1

Question 25

Key presentation of APS

Answer 25

Female patient with thrombosis (ie VTE or PE) with a history of frequent miscarriage

Question 26

Treatment for APS

Answer 26

Lifetime anticoag-

Warfarin target INR 2-3
LWMH and aspirin in pregnancy (as warfarin is teratogenic)