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Rhematology > Connective Tissue Diseases > Flashcards

Connective Tissue Diseases Flashcards

1
Q

Common connective tissue diseases

A 
SLE
Systemic sclerosis
Myosotis/ dermatmyositis
Sjogrens 
Mixed connective tissue diseases
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2
Q

What makes up mixed connective tissue disease

A

SLE + systemic sclerosis+ RNP Ab

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3
Q

Pathology of SLE

A

Pathogenic autoantibodies results in immune complex formation and complement mediated damage
IC = deposited in the tissues and are not cleared so this elicits inflammation via the classical pathway and activation of macrophages and neutrophils via surface FC receptors which bind to IgG.

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4
Q

Epidemiology of SLE

A

Women>men

Afrocaribean and south Asian > white

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5
Q

What are the systemic features of SLE

A 
Fatigue acute/chronic
Fever
Lymphadenopathy 
Weight loss 
Myalgia
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6
Q

Arthritis symptoms in SLE

A 
Symmetrical non-erosive synovitis 
Arthralgia - more common than synovitis 
Jacobs arthropathy - where the deformities are reducible by the patient or dr 
Nodules 
SimilarRA - small joint involvement
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7
Q

Skin Involvement in SLE

A

Photosensitivity - rash can appear only where sun exposure has been
Malar - butterfly rash on the face the nose and cheeks affected but the nasolabial folds are not affected
Discoid rash - can be scaring ans can occur in the absence of systemic features - cutaneous LE if ANA +ve more likely to progress to systemic
ALL can be made worse with UV light
Vasculitis rashes
Periungal erythema
Inflamm in the BV - infarction and necrosis

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8
Q

What is Raynaud

types of raynaud’s you get in SLE

A

Raynauds in the reflex vasoconstriction of BV in the extremities - finger, hands, feet and nose in response to a drop in temperature
If associated with SLE or another systemic disease it is secondary Raynauds - Raynaud’s phenomena
If not associated with other conditions- Raynauds diseases primary raynauds

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9
Q

What is livedo reticularis

A

Can get it wit SLE and vasculitis
It is blotches of purple/brown on the legs in a similar fishnet, distribution on the legs
In SLE they occur in patients who have anti-phospholipid syndrome who are more likely to clot

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10
Q

Alopecia inSLE

A

Can be areata or total is
Can be scaring or non scaring
The scaring can be associated with a discoid rash
The scaring form the hair will not grow back

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11
Q

Pulmonary features or SLE

A

Pleurisy - can be caused by SLE
But due to tx immunosuppression it may be caused by infection and due to the anti phospholipid syndrome it may be a PE
So it is important to rule these things out before attributing it to being a part of the illness
Fibrosis
Diffuse lung disease with a dry cough distinguish from infection again - inflam in the alveoli - pneumonitis which progresses to fibrosis
Pulmonary hypertension
- normalCXR it is a primary disorder can be characterised by a large heart reduced lung markings and a large pulmonary artery

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12
Q

What doesn’t rule out infection in SLE

A

Fever as it is part of SLE

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13
Q

Cardiac involvement in SLE

A

Pericarditis - inflamm of the lining of the heart
Myocarditis - cardiac failure and arrhythmia
Libman-sacks - non infectious vegetation of the heart can lead to emboli
CAD - early MI due to blood vessel inflammation

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14
Q

Muscles membrane affected in SLE

A

Oral/nasal/ genital ulcers
May be painful and painless
Apthous ulcers no different to normal ulcers
Sicca symptoms -2ndary Sjogrens dry eyes and mouth due to destruction of exocrine glands - salivary and lacrimal

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15
Q

Abdominal involvement SLE

A

Anorexia, N/V, pain, diarrhoea, vasculitis affecting intra ab organs
Ischaemic organs to anti phospholipid syndrome

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16
Q

Kidney involvement SLE

A 
Asymptomatic 
Proteinuria 
Blood cells 
RBC casts in the glomeruli 
Leading to ether a proliferative glomerulonephritis or a membranous glomerulonephritis
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17
Q

Neurological involvement SLE

A

Cerebrovascular disease
Seizure
Chorea
Cog dysfunction - dementia/ pseudo dementia from depression as these patients are living and coming to terms with this chronic condition
Psychosis
Mononeuropathy/ mono multi/poly - glove and stocking

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18
Q

Reproductive features of SLE

A

Recurrent miscarriages
Fetal growth restriction due to poor flow in the placenta
Neonatal lupus syndrome - due to the mothers anti-Ro and anti-La being passed to the Fetus this means the newborn could get a photosensitive rash if in the sun, may get congenital heart block
Premature menopause - auto ab against ovary or drug induced- cyclophosphamide

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19
Q

Features of anti-phospholipid syndrome

A 
Recurrent venous thrombosis
-DVT
-PE
Recurrent arterial thrombosis 
MI stroke 
Recurrent miscarriages 
Thrombocytopenia and interference with phospholipid dependent clotting tests
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20
Q

Lab result features of SLE

A

Haematology
- anaemia: chronic disease, haemolytic, blood loss - iron def
- leukopenia - lymph and neutropenia common - tx or disease
Thrombocytopenia
- ESR inc
-CRP dec or small rise in comparison to ESR
Renal function
Antibodies - own slide

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21
Q

Antibodies in SLE

A 
ANA. Sensitive but not specific 
Anti-dsDNA -60% 
Ani - ENA - extractable nucleotide antibody 
Ro, La, Sm, RNP
Antiphospholipid ab
APTT prolonged 
Anti-cardiolipin ab 
Lupus anticoagulant
22
Q

Complement levels in SLE why are they this way

A

Low
C3/4
They are low because they are being used up quickly

23
Q

At is a marker of SLE disease activity

A

How Low complement is

How high do-DNA is

24
Q

What are Ro and La associated with

A

Neonatal lupus

25
Q

What are RNP ab associated with

A

SLE and systemic sclerosis

Mixed connective tissue disease

26
Q

What are ant-smith ab associated with

A

No definite

? Nephritis and CNS involvement

27
Q

Mx of SLE

A

Con - sunblock, education, infection risk, fertility - contraception and what to do to ensure safe pregnancy
Med - NSAIDS - not a lot due to the si - PPI, renal impairment - not use, CV risk and hypertension so used sparingly
Corticosteroid - IM, IV, PO
Hydroxychloroquine
Introduce a immunosuppression
Azathioprine
Methotrexate, cyclophosphamide
Mycofenalate
Refractory disease - rituximab or anti CD22mab
Use cyclosporine in leukopenia
Mycophenolate and cyclophosphamide in renal impairment

28
Q

What is systemic sclerosis

A

Generalised disorder of connective tissue affecting the skin and internal organs
It is characterised by fibrotic arteriosclerosis or peripheral and visceral vasculature
Extracellular matrix accumulation (collagen) in the skin and viscera
Associated with auto ab

29
Q

What is localised sclerosis

A 
CREST 
Calcinosis 
Raynauds
Oesophageal dysmotility
Sclerodactyly
Telangectasia
30
Q

Systemic sclerosis effects on the skin

A

Starts inflammatory -oedema, tightening, thicken and waxy but then progresses to atrophy where the skin thins more collagen deposition and muscle decreases

31
Q

What happens to the joints in systemic sclerosis

A

Skin may pull the joints into an abnormal position

Face hand and feet and then Arms legs and chest involvement

32
Q

How does systemic sclerosis affect the face

A

Small tight mouth
Vitiligo
Hyperpigmentation
Telangectasia

33
Q

Systemic sclerosis affect on the gut

A

Hiatus hernia
Poorly coordinated muscle activity
Dysphagia
Strictures - which inc risk of perf on endoscopy so test recommended is barium swallow

34
Q

Pulmonary involvement systemic sclerosis

A 
Intestitial fibrosis 
-breathless 
-dry cough 
Exclude infection 
Tx with immunosuppression 
- pulmonary hypertension
35
Q

Cardiac affects of systemic sclerosis

A

CCF and arrhythmia

36
Q

Nail involvement systemic sclerosis

A

Under nailfold capillaroscopy

See dilated capillaries which are tortuous and a dec vessel number

37
Q

Renal involvement systemic sclerosis

A

Accelerated hypertension
Renal crisis- blood vessel arterosclerosis
Shrink the glomeruli

38
Q

Ix systemic sclerosis

A 
BP monitoring 
Renal function 
Pulm function
ECG
CXR
Barium swallow 
ANA
Anti-ENA
Anti-centromere 
Anti-SC
39
Q

Mx systemic sclerosis

A

Symp - raynauds - con and med
Skin - moisturise
Immunosuppression or anti-fibrotic
Tx aimed at organ

40
Q

What is myosotis

A 
Chronic inflam of striated muscle 
There are 4 types 
-myositis 
- dermatomyositis 
- mixed CT
- malignancy associated
41
Q

What antibody associated with myositis

A

Anti-Jo1

42
Q

Clinical features of myositis

A

Pain stiff and tender muscles with progressive weakness mainly in the proximal arms and legs
Progressive facial muscle involvement - facial, bulbar, speech and language, resp - breathing
Lung fibrosis
And heart involvement

43
Q

Skin involvement - myositis

A

Heliotrope rash around the eyes
Rash on the skin of the chest
Gettron papules on the knuckles MCP, PIP, DIP
MAchnists hand -dry and cracked

44
Q

Ix myositis

A 
Blood- CPK, LDH, AST, ALT, ESR
ANA
Anti Jo1
ECG
CXR
MRI
Biopsy of the muscle
Ix - malignancy
45
Q

What cancer are associated with dermatomyosits

A

Breast
Lung
Ovarian
Dermatomyositis can result from these cancers presumably as an autoimmune response

46
Q

Therapy myositis

A

Steroids - but not for long as they can accuse muscle weakness
Immunosuppressants - methotrexate, azathioprine
Severe and lung - cyclophosphamide
Refractory - cyclosporine mycophenalate
Rituximab
IV Ig
Monitor disease progress

47
Q

Sjogrens what is it

A

Slowly progressive inflammatory disease affecting the exocrine glands
- lacrimal
- salivary
Overlap SLE
Lymphocyte infiltration
Ab - Ro and La
Mucosal dryness and eye dry, genital tract dry

48
Q

Signs and test for Sjogrens

A

Schirmer tear test

Parotid swelling

49
Q

Xtra glandular disease in Sjogrens

A 
Polyarthritis
Lung 
Kidney
Liver
BV
50
Q

When in association with other Autoimmune disease sjogrens is
And what other AI diseases are they like

A

RA, SLE, systemic sclerosis

2ndary sjogrens

Rhematology (6 decks)

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