Connective Tissue Disorders Flashcards
What are connective tissue diseases?
Spontaneous over activity of the immune system
What does SLE stand for
Systemic Lupus Erythematosis
Increased levels of which hormone increase the incidence of SLE
Oestrogen
What are some environmental factors predisposing you to SLE
silica dust Epstien-Barr virus UV light
What is the Pathogenesis of SLE
Loss of immune regulation: Increase apoptosis releases nuclear material. B and T cells are stimulated These produce Auto-antibodies
what are auto-antibodies
antibodies to the self rather than to bugs
Why do people with SLE also get Renal Problems?
Likely due to deposition of immune complexes in the mesangium
What are immune complexes made of?
nuclear antigens and anti-nuclear antibodies
How are immune complexes made?
Nuclear antigens join up with anti-nuclear antibodies during circulation and are then deposited in vessel walls. Especially in the mesangium
Why are immune complexes a problem?
Once they are deposited in a vessel wall the activate complement and attract neutrophils attacking the endothelial cell’s basement membrane
What systems are involved in Systemic Lupus Erythematosis
Mucocutaneous MSK Serositis Renal Neurological Haematological
What are the constitutional symptoms of SLE
Cancer like Malaise Poor appetite Weight loss Fatigue Fever
What are some of the mucocutaneous Features of SLE
Blad red faced guy with a sore mouth
Malar rash
Painless Mouth ulcers
alopecia
Photosensitvity
What are some of the MSK features of SLE
Non-deforming polyarthritis and Jaccoud’s arthritis and Myopathy
What is jaccoud’s arthritis
Frenchmen have strange hands Metacarpal phalangeal joints shift laterally
what the hell is Serositis?
Inflammation of the serous membranes
What might happen if you have serositis?
Pericarditis
Pleurisy
Pleural effusion
Pericardial effusion
What are some neurological features of SLE
Migranes Seizures Cranial or peripheral neuropathy Mononeuritis complex
what are the Haematological features of SLE?
Immunosuppressed , anaemic and clot easily
Lymphadenopathy
Leucoponeia
Lymphoponeia
Haemolytic anaemia
Thromobocytopenia
What Clinical features would you commonly find in Anti-phospholipid syndrome?
Venous and arterial thrombosis
Recurrent miscarriage
Lividio Reticularis
Thrombocytopenia
Prolonged APTT
What does Thrombocytopenia mean ?
low platelet count
Why are people with SLE susceptible to infection? (intrinsic factors)
Thrombocytopenia
defective phagocytosis
Impaired cell mediated immunity
Low complement levels
Why are people with SLE susceptible to infection? (extrinsic factors)
They are usually on steroids
What conditions is ANA found in?
Systemic Lupus Erythematous
Rheumatoid Arthritis
HIV
Hepatitis C
C3 and C4 levels correlate with systemic Lupus Erythematosus disease activity positively , negatively or not at all?
Negatively Bad disease = low complement levels
What in particular do you measure to monitor SLE activity?
Anti-dsDNA
and
c3/c4 levels
What is Livido Reticularis?
Funny pattern, cold legs at basketball
What two things should people with SLE avoid?
excessive sun exposure
Pregnancy
what drugs are used to treat SLE? (classes)
NSAIDs Immunosuppressives Steroids Biologics
What is used to treat mild SLE?
Hydrochloroquine Topical steroids NSAIDs
What is used to treat moderate SLE?
Oral Steroids - Azathioprine or Methotrexate
What is used to treat severe SLE?
IV steroids Cyclophosphamide
Biologics Rituximab
What blood tests (along with clinical signs) are diagnostic of Anti-phospholipid Syndrome?
Positive anti-cariolipin antibodies
Lupus anticoagulant activity
Anti-beta2-glycoprotein
twice, 12 weeks apart.
What clinical signs ( along with blood tests) are diagnostic for Anti-phospholipid Syndrome?
Arterial or Venous thrombosis
Recurrent pregnancy loss with no other explanation
How do you treat Anti-Phoslipid Syndrome?
Life long anti-coagulation Aspirin and Heparin during pregnancy
Attention to CVD risk factors
What Is the story about Sjorgen’s syndrome?
Sjorgen RoLa lives in the desert so he gets dry eyes and lymphomas
Why does Sjorgen Rola have dry eyes?
Lymphocyte infiltration of the exocrine glands causing xerostomia and keratoconjunctivitis sicca.
What it is in the primary Sjorgen’s syndrome classification criteria?
need 4 out of the 6 and at least one test
- Subjective dry eyes for three months
- Dry eyes on examination
- Subjective dry mouth for three months
- Dry mouth on examination
- Immunology - either Ro or La or RoLa
- Biopsy evidence on Lymphocyte infiltrate.
What is the peak age range for Primary Sjogren’s Syndrome?
40-60yrs
How do you treat Sjogren’s syndrome?
Eye drops Synthetic Slaiva Hydrolochloroquine Steroids and immunosupression
What is Systemic Sclerosis?
Autoimmune disease with excess deposition of collagen in the skin and internal organs
What clinical features do you commonly find in Systemic Sclerosis?
CREST
Calconosis
Raynaud’s syndrome
Esophageal Dymotility
Sclerodactyly
Telangiectasia
What does CREST stand for?
Calconosis Raynaud’s syndrome Esophageal Dymotility Sclerodactyly Telangiectasia
Whats the story about Raynaud
Captain Raynaud is always crashing his vessles
Whats the story with Scperodactyly
Teradactyles have really hard roguh tight skin
What antibodies are Characteristic of Systemic Sclerosis?
Anti-Scl-70 antibodies
what Gastrointestinal symptoms do people with systemic sclerosis get?
Hypo-motility of EVERYTHING
What respiratory problems do people with systemic sclerosis get?
Interstitial lung disease pulmonary hypertension Chest wall restriction
what CVS problems do people with systemic sclerosis they get?
Raynauds Atherosclerotic disease Hypertensive cardiomyopathy
What do you use to treat Systemic Sclerosis?
Calcium channel blockers
Prostacyclin
ACE inhibitors
Prednisolone
Immunosupression
Who gets Systemic Lupus erythematosus?
Woman of child bearing age
