Connective Tissue Diseases and Vasculitis

Question 1

What is connective tissue?

Answer 1

• 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

Question 2

Name 4 heritable CTD

Answer 2

1. Marfans
2. Ehlers danlos
3. Osteogenesis imperfecta
4. Stickler syndrome

Question 3

Name 5 autoimmune CTDs

Answer 3

1. SLE
2. Sjogren’s
3. Systemic sclerosis - DCSSc, LCSSc
4. RA
5. Mixed CTD

Question 4

Some Signs and symptoms of systemic sclerosis

Answer 4

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

Question 5

3 important questions to ask in history?

Significance for FPE?

Answer 5

1. Do your hands change colour in the cold? Describe
2. SOB? (lung involvement is v bad prognosis indicator)
3. Bad indigestion/trouble swallowing?

Cold and painful hands (as opposed ot warm and painful for RA)

Question 6

What antibodies in SSc?

How manage? (think on systems involved)

Answer 6

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

Question 7

How does SLE present?

What are the symptoms?

Answer 7

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

Question 8

How manage SLE?

Answer 8

MDT

Immunosuppression

Hydroxychloroquine

Steroids

Biologicals

Regular monitoring - ? need for anticoagulation

Question 9

Another name for antiphospholipid syndrome?

How diagnose?

Sg and Sx

Management?

Answer 9

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

Question 10

What is Raynaud’s phenomenon?

Raynaud’s disease vs syndrome?

Answer 10

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

Question 11

What autoantibodies in Sjogren’s?

How common and what diseases secondary to?

Answer 11

Anti-RO and Anti-La

2nd most common CTD

Can be primary, but often seconadary in association with

CTD, PBC, Coeliac

Question 12

Sjogren’s Sg/Sx

Answer 12

Glandualar and extraglandular features

Parotid swelling

Xerostomia

Xeroophthalmia

Psychosexual - dry downstairs!

Arthralgia

Raynaud’s

Question 13

How test for sicca?

What diseases may mimic sjogrens?

How manage Sjogren’s?

Answer 13

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

Question 14

Examples of vasculitides

Criteria for Kawasaki’s?

Answer 14

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

Question 15

Vasculitis - Ix and Mg

DD - always consider?

Answer 15

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

Question 16

GCA

What is it?

What are signs/symptoms?

Answer 16

Medium/Large vessel immune mediated systemic vasculitis that often presents with temporal arteritis (GC is the immune cell):

Tenderness over temporal artery, jaw claudication, tenderness on combing, new onset headache, visual disturbance

+ Malalise, fever etc (as with all vasculitides)

Associated with polymyalgia rheumatics, so there may be proximal muscle weakness and pain.

Question 17

GCA

Investigations?

How Manage?

Answer 17

ESR should be raised (although start treatment if suspect clinically); ? need for temporal artery biopsy if ESR raised

High dose steroids - 40-60mg(if ischaemic symptoms); If visual disturbance start IV methylprednisolone in hospital

+ aspirin + PPI unless contraindicated and OP prophylaxis if on long term steroids

NB risk of large vessel disease e.g. thoracici aneurysm