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MSK- Rheumatology > Connective Tissue Disorders > Flashcards

Connective Tissue Disorders Flashcards

1
Q

What are connective tissue diseases?

A

Spontaneous over activity of the immune system

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2
Q

What does SLE stand for

A

Systemic Lupus Erythematosis

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3
Q

Increased levels of which hormone increase the incidence of SLE

A

Oestrogen

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4
Q

What are some environmental factors predisposing you to SLE

A

silica dust Epstien-Barr virus UV light

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5
Q

What is the Pathogenesis of SLE

A

Loss of immune regulation: Increase apoptosis releases nuclear material. B and T cells are stimulated These produce Auto-antibodies

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6
Q

what are auto-antibodies

A

antibodies to the self rather than to bugs

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7
Q

Why do people with SLE also get Renal Problems?

A

Likely due to deposition of immune complexes in the mesangium

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8
Q

What are immune complexes made of?

A

nuclear antigens and anti-nuclear antibodies

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9
Q

How are immune complexes made?

A

Nuclear antigens join up with anti-nuclear antibodies during circulation and are then deposited in vessel walls. Especially in the mesangium

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10
Q

Why are immune complexes a problem?

A

Once they are deposited in a vessel wall the activate complement and attract neutrophils attacking the endothelial cell’s basement membrane

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11
Q

What systems are involved in Systemic Lupus Erythematosis

A

Mucocutaneous MSK Serositis Renal Neurological Haematological

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12
Q

What are the constitutional symptoms of SLE

A

Cancer like Malaise Poor appetite Weight loss Fatigue Fever

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13
Q

What are some of the mucocutaneous Features of SLE

A

Blad red faced guy with a sore mouth

Malar rash

Painless Mouth ulcers

alopecia

Photosensitvity

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14
Q

What are some of the MSK features of SLE

A

Non-deforming polyarthritis and Jaccoud’s arthritis and Myopathy

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15
Q

What is jaccoud’s arthritis

A

Frenchmen have strange hands Metacarpal phalangeal joints shift laterally

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16
Q

what the hell is Serositis?

A

Inflammation of the serous membranes

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17
Q

What might happen if you have serositis?

A

Pericarditis

Pleurisy

Pleural effusion

Pericardial effusion

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18
Q

What are some neurological features of SLE

A

Migranes Seizures Cranial or peripheral neuropathy Mononeuritis complex

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19
Q

what are the Haematological features of SLE?

A

Immunosuppressed , anaemic and clot easily

Lymphadenopathy

Leucoponeia

Lymphoponeia

Haemolytic anaemia

Thromobocytopenia

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20
Q

What Clinical features would you commonly find in Anti-phospholipid syndrome?

A

Venous and arterial thrombosis

Recurrent miscarriage

Lividio Reticularis

Thrombocytopenia

Prolonged APTT

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21
Q

What does Thrombocytopenia mean ?

A

low platelet count

22
Q

Why are people with SLE susceptible to infection? (intrinsic factors)

A

Thrombocytopenia

defective phagocytosis

Impaired cell mediated immunity

Low complement levels

23
Q

Why are people with SLE susceptible to infection? (extrinsic factors)

A

They are usually on steroids

24
Q

What conditions is ANA found in?

A

Systemic Lupus Erythematous

Rheumatoid Arthritis

HIV

Hepatitis C

25
Q

C3 and C4 levels correlate with systemic Lupus Erythematosus disease activity positively , negatively or not at all?

A

Negatively Bad disease = low complement levels

26
Q

What in particular do you measure to monitor SLE activity?

A

Anti-dsDNA

and

c3/c4 levels

27
Q

What is Livido Reticularis?

A

Funny pattern, cold legs at basketball

28
Q

What two things should people with SLE avoid?

A

excessive sun exposure

Pregnancy

29
Q

what drugs are used to treat SLE? (classes)

A

NSAIDs Immunosuppressives Steroids Biologics

30
Q

What is used to treat mild SLE?

A

Hydrochloroquine Topical steroids NSAIDs

31
Q

What is used to treat moderate SLE?

A

Oral Steroids - Azathioprine or Methotrexate

32
Q

What is used to treat severe SLE?

A

IV steroids Cyclophosphamide

Biologics Rituximab

33
Q

What blood tests (along with clinical signs) are diagnostic of Anti-phospholipid Syndrome?

A

Positive anti-cariolipin antibodies

Lupus anticoagulant activity

Anti-beta2-glycoprotein

twice, 12 weeks apart.

34
Q

What clinical signs ( along with blood tests) are diagnostic for Anti-phospholipid Syndrome?

A

Arterial or Venous thrombosis

Recurrent pregnancy loss with no other explanation

35
Q

How do you treat Anti-Phoslipid Syndrome?

A

Life long anti-coagulation Aspirin and Heparin during pregnancy

Attention to CVD risk factors

36
Q

What Is the story about Sjorgen’s syndrome?

A

Sjorgen RoLa lives in the desert so he gets dry eyes and lymphomas

37
Q

Why does Sjorgen Rola have dry eyes?

A

Lymphocyte infiltration of the exocrine glands causing xerostomia and keratoconjunctivitis sicca.

38
Q

What it is in the primary Sjorgen’s syndrome classification criteria?

A

need 4 out of the 6 and at least one test

  1. Subjective dry eyes for three months
  2. Dry eyes on examination
  3. Subjective dry mouth for three months
  4. Dry mouth on examination
  5. Immunology - either Ro or La or RoLa
  6. Biopsy evidence on Lymphocyte infiltrate.
39
Q

What is the peak age range for Primary Sjogren’s Syndrome?

A

40-60yrs

40
Q

How do you treat Sjogren’s syndrome?

A

Eye drops Synthetic Slaiva Hydrolochloroquine Steroids and immunosupression

41
Q

What is Systemic Sclerosis?

A

Autoimmune disease with excess deposition of collagen in the skin and internal organs

42
Q

What clinical features do you commonly find in Systemic Sclerosis?

A

CREST

Calconosis

Raynaud’s syndrome

Esophageal Dymotility

Sclerodactyly

Telangiectasia

43
Q

What does CREST stand for?

A

Calconosis Raynaud’s syndrome Esophageal Dymotility Sclerodactyly Telangiectasia

44
Q

Whats the story about Raynaud

A

Captain Raynaud is always crashing his vessles

45
Q

Whats the story with Scperodactyly

A

Teradactyles have really hard roguh tight skin

46
Q

What antibodies are Characteristic of Systemic Sclerosis?

A

Anti-Scl-70 antibodies

47
Q

what Gastrointestinal symptoms do people with systemic sclerosis get?

A

Hypo-motility of EVERYTHING

48
Q

What respiratory problems do people with systemic sclerosis get?

A

Interstitial lung disease pulmonary hypertension Chest wall restriction

49
Q

what CVS problems do people with systemic sclerosis they get?

A

Raynauds Atherosclerotic disease Hypertensive cardiomyopathy

50
Q

What do you use to treat Systemic Sclerosis?

A

Calcium channel blockers

Prostacyclin

ACE inhibitors

Prednisolone

Immunosupression

51
Q

Who gets Systemic Lupus erythematosus?

A

Woman of child bearing age

52
Q
A

MSK- Rheumatology (8 decks)

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