Connective tissue diseases Flashcards

1
Q

what are connective tissue diseases

A

spontaneous over activity of immune system

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2
Q

What parts of the body canSLE affect?

A

EVERYWHERE

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3
Q

Is there a genetic link with SLE?

A

YES

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4
Q

Is there an increased risk with increased oestrogen?

A

yup

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5
Q

What is th primary pathogenies of SLE?

A

loss of immune regulation, increased and defective apoptosis, autoantibodies are produced

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6
Q

What is the criteria for clinical lupus?

A

arthritis, cutaneous lupus, oral or nasal ulcers, alopecia (non scarring), serositis, renal, leukopenia, neurological, ANA, antiDNA, antiSm, low complement

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7
Q

What are some symptoms of SLE

A

fever, malaise, WL, fatigue, poor appetite, photosensitivity, malaria rash, butterfly rash, raynauds, fibrosis of lungs, hypertension, infarct, pleurisy, pericarditis, cardiomyopathy,

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8
Q

What investigations would you do for SLE?

A

FBC, renal function, ANA, antiDNA, complement levels,

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9
Q

When should positive ANA test be taken seriously?

A

When other autoantibodies are positive also…

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10
Q

What drugs would you use in SLE?

A

NSAIDS and analgesia, hydroxychloroquine. Steroids (prednisolone small dose) for skin rashes, high dose for severe SLE, immunosuppressives.

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11
Q

What do patients with lupus die of?

A

Cardiovascular disease and infection primarily

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12
Q

What are signs of anti- phospholipid syndrome?

A

positive anti-cardiolipin antibodies, thrombosis, unexplained pregnancy loss/ recurrent pregnancy loss, thrombophlebitis, thrombocytopenia,

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13
Q

How do you treat anti- phospholipid syndrome

A

thrombosis- lifelong anti coag
pregnancy loss- aspirin and heparin during pregnancy
watch out for vascular risk factors

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14
Q

What is sjogrens syndrome?

A

autoimmune, lymphocyte infiltration of exocrine glands causing keratoconjunctivits and xerostomia

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15
Q

What symptoms do you get with sjogrens

A

oral and ocular symptoms daily for >3 months, eye dryness, Ro, La, biopsy of infiltrate and salivary gland involvement

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16
Q

How do you treat Sjogrens

A

eye drops, saliva replacement, pilocarpine, hydroxychloroquine, steroids/ immunosuppression

17
Q

What is systemic sclerosis

A

hardening of the skin due to collagen deposition

18
Q

What does crest stand for in systemic sclerosis

A

Calconosis, Raynauds, oesophagel dysmotility, scerodactyly, telangiectasia

19
Q

What type of antibody is associated with systemic sclerosis?

A

Anti centromere antibodies

20
Q

What is a respiratory manifestation of systemic sclerosis

A

interstital lung disease

21
Q

How would you treat systemic sclerosis

A

CCBWhat is undifferentiated connective tissue disorder called?, prostacyclin, ACE inhibitors, prednisolone, immunosuppression

22
Q

What is the undifferentiated connective tissue disorder called?

A

connective tissuish

23
Q

What is the autoantibody for mixed connective tissue disease

A

Anti RNP