Connective Tissue Diseases and Vasculitis Flashcards
What is connective tissue?
- Mesoderm derived
- Most abundant tissue in the body
- Binds, supports and strengthens other tissues
- Protects and insulates internal organs
- Compartmentalizes structures such as skeletal muscles
- Stores energy in adipose tissue
- Transports molecules as blood
Name 4 heritable CTD
- Marfans
- Ehlers danlos
- Osteogenesis imperfecta
- Stickler syndrome
Name 5 autoimmune CTDs
- SLE
- Sjogren’s
- Systemic sclerosis - DCSSc, LCSSc
- RA
- Mixed CTD
Some Signs and symptoms of systemic sclerosis
Sclerodactyly - tight, cold, waxy, skin on fingers with telangiectasia; ? calcification
Facial features - tethereing of skin on forehead, telangiectasia, beaking of nose, furrows around mouth and microstomia
CREST - calcinosis, raynauds’, oesophageal dysmotility, sclerodactlyly, telangiectasia
3 important questions to ask in history?
Significance for FPE?
- Do your hands change colour in the cold? Describe
- SOB? (lung involvement is v bad prognosis indicator)
- Bad indigestion/trouble swallowing?
Cold and painful hands (as opposed ot warm and painful for RA)
What antibodies in SSc?
How manage? (think on systems involved)
Anti-Scl70 for DCSSc
Anti-centromere for LCSSc
? immunosuppression…
Manage complications:
Skin - emollients, methotrexate
Gut - ABx prophylaxis, osmotic laxatives?
Vascular and Raynauds’ - vasodilators, iloprost (prostacyclin analogues), bosentan (smooth muscle dilator)
Lungs - LTOT, Cyclophosphamide, CCB or sildenafil if PulHTN
Renal - ACE-I
Cardiovascualr - pacemaker if associated heart block
How does SLE present?
What are the symptoms?
Malaise, lethargy, fever, arthralgia…
SOAP BRAIN MD
Serositis, Oral ulcers, ANA (anti-dsDNA), Photosensitive rash
Blood dyscrasia, Renal crises, Arthralgia, Immune, Neuropsychotic (seizures, psychosis)
Malar rash, Discoid rash
How manage SLE?
MDT
Immunosuppression
Hydroxychloroquine
Steroids
Biologicals
Regular monitoring - ? need for anticoagulation
Another name for antiphospholipid syndrome?
How diagnose?
Sg and Sx
Management?
Hughes’ syndrome; may be primary or secondary e.g. to SLE
Lupus anticoagulant and Anti-cadiolipin (only need one with appropriate history). 2 tests at least 6 weeks apart
CLot risk - DVT, Miscarriage, Stroke; Livedo reticularis
Anticoagulation - warfarin, aspirin, ? clopidogrel; may need heparin if pregnant, but can’t be on long term because of risk OP
What is Raynaud’s phenomenon?
Raynaud’s disease vs syndrome?
vasospasm leading to stereotypical colour change of fingers/toes from white to purple to red (reactive hyperaemia). Can hurt on reperfusion
Disease - in isolation
Syndrome - when part of a systemic disease
What autoantibodies in Sjogren’s?
How common and what diseases secondary to?
Anti-RO and Anti-La
2nd most common CTD
Can be primary, but often seconadary in association with
CTD, PBC, Coeliac
Sjogren’s Sg/Sx
Glandualar and extraglandular features
Parotid swelling
Xerostomia
Xeroophthalmia
Psychosexual - dry downstairs!
Arthralgia
Raynaud’s
How test for sicca?
What diseases may mimic sjogrens?
How manage Sjogren’s?
Schirmer’s test (filter paper in eyes to measure tear production)
HIV, Sarcoid, TB, Lymphoma, GVHD, anti-ACh drugs, previous radiotherapy
Artifical saliva and tears, hydroxychloroquine, immunosuppression, muscarinics e.g. piilocarpine
Examples of vasculitides
Criteria for Kawasaki’s?
Giant cell arteritis (temporal)
Wegener’s - sinus, lungs and kidneys; c-ANCA (PR3)
microscopic Polyangitis - c-ANCA (MPO)
PAN - p-ANCA
Takayasus
Kawasakis’s
- Fever +
- Coronary artery aneurysm or 4 of:
- cervical LA > 1.5cm
- mucosal irritation - strawberry tongue, bucal mucosal injection,
- erythema and oedema, ? desquamation
- Widespread non-vesicular rash
- Bilateral dry conjunctivitis
Vasculitis - Ix and Mg
DD - always consider?
Ix - Full profilel, especially CRP/ESR
IC formation, reduced C3/4, RF, ANA, cryoglobulins; ? angiography
High dose steroids, cytotoxic drugs - ? need for gamete sparing?
TB and HIV
