Connective Tissue Diseases Flashcards
(28 cards)
What is SLE?
Multi-system relapsing & remitting autoimmune, inflammatory disorder w/auto-ab
How does tissue damage occur in SLE?
Deposition of immune complexes
Leads to complement activation (T3 hypersensitivity)
Resulting in tissue damage
What conditions can SLE we associated with?
Sjogrens
Antiphospholipid syndrome
What are the RFs for SLE?
Female
Afro-carribbean + Asian
Fix
Meds: Isoniazid, Hydrasalazine, phenytoin
How does SLE present? Can SLE be diagnosed on Sx?
Remitting & relapsing
Constitutional: Malaise, fatigue, fever
MYALGIA + ARTHRALGIA
Lymphadenopathy
4/11 of:
- Malar butterfly rash
- Discoid rash/macpap rash
- Photosensitivity
- Painless oral ulcers
- Non-erosive arthritis >2joints
- Serositis/pleuritis/pericarditis/effusion
- CNS: Seizure/psychosis
- ANA +ve
- Renal: Persistent proteinuria/cellular casts
- Haem: Leuko/thrombo/leukopenia
- Immune: Anti-dsDNA/Anti-Sm/ Antiphospholipid
How is SLE investigated?
1) Bloods: ↑ESR, CRP (N) FBC, U+Es, LFTs
2) Skin biopsy: Immune complexes at derma-epidermal junction
3) Immunology
What immunology may be +ve in a diagnosis of SLE?
ANA- 95% Anti-dsDNA-60% Anti-Sm (MOST specific) RF-40% Anti-phospholipid Abx Complement: ↓C3/4
How is SLE managed?
Cutaneous: TOP Steroids
Joint: NSAID + Hydroxychloroquine
DMARD
Suncream
How is SLE monitored?
Anti-dsDNA Ab titre
Complement: ↓during active disease
ESR
What are the complications of SLE?
Severe flare: Haemolytic anaemia, nephritis, pericarditis, CNS
Antiphospholipid syndrome
What are the causes of drug induced Lupus?
Hyralazine Procainamide Isoniazid Phenytoin Chlorpromazine Sx: Arthralgia, myalgia, malar rash, pleurisy
What is Scleroderma?
Multisystem AI disorder characterised by internal organ fibrosis & vascular damage
How does internal organ fibrosis & vascular damage occur in Scleroderma?
↑Fibroblast activity leads to ↑COLLAGEN DEPOSITION
What are the types of scleroderma?
1) LIMITED Cutaneous: Common, slow, mild, CREST syndrome
2) DIFFUSE Cutaneous: Rapid, high mortality
What are the RFs for Scleroderma?
FHx
Infection: CMV, EBV, Chlamydia
Vit D Deficiency
Drugs: Cocaine, Vit K, Bleomycin
How does Scleroderma present?
-Raynaud’s 90% 1st Sx
-CREST: Calcinosis, Raynaud’s, Oesophageal dysmotility, Sclerodactyly, Telangiectasia
-Joint swelling
-Constitutional: Fatigue, ↓weight, myalgia
Pulm: Fibrosis, HTN
Cardiac: Myocardial fibrosis, pericarditis, LVF
Renal: ANCA, GN
GU: Erectile dysfunction 90%
How is Scleroderma investigated?
Bloods: FBC, U&E, ESR, CRP Immunology: Anti-Scl70- DIAGNOSTIC for diffuse ACA- DIAGNOSTIC for limited ANA +ve, RF +ve (30%)
In terms of skin hardening how does this differ between limited & diffuse Scleroderma?
LIMITED: Face, distal limbs → up to knees & forearms
DIFFUSE: Trunk, proximal limbs (Upper arms/thighs)
How is Scleroderma managed?
- Physio & Conservative (stop smoking, exercise, OT)
- Raynaud’s: Nifedipine/Losartan
- Diffuse <3yr: Methotrexate
- Skin involved: Methotrexate OR Mycophenolate
What is Sjogren’s syndrome?
AI condition causing infiltration of exocrine glands by lymphocytes
What are the Sx of Sjogren’s?
Xerostomia: Altered taste, tongue sticks to roof of mouth, oral candida
Xeropthalmia: Corneal ulcers, blepharitis
Parotitis: B/L + recurrent
Fatigue: 80%
Chronic pain: Myalgia + polyarthralgia
Raynaud’s
Other: Dry cough, dry skin, reflux, vaginal dryness, malabsorption (pancreas)
How is Sjogren’s investigated?
Diagnosed according to Copenhagen criteria
Bloods:↑ESR, ↓Hb, RF (100%), Anti-Ro (70%), Anti-La
Shirmer test: Filter paper in lower conjunctiva >15mm wet = normal, <5mm wet = abnormal
How is Sjogren’s managed?
Artificial tears & saliva
DMARD
What are the complications of Sjogren’s?
↑Eye/Mouth/Parotid infections
↑ Risk of Hodgkin’s lymphoma
50% develop disease in other sites
