Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

MSK > Multi-System Autoimmune Diseases > Flashcards

Multi-System Autoimmune Diseases Flashcards

1
Q

What are connective tissue diseases?

A

Broad group of disorders that affect connective tissues (cartilage, BVs, bones, tendons etc.)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are examples of autoimmune connective tissue diseases?

A 
SLE
Slceroderma
Sjogren's syndrome
Autoimmune myositis
Mixed connective tissue disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is autoimmune vasculitis?

A

Autoimmune diseases characterised by inflammation of blood vessels (vasculitis) & subsequent ischaemia and damage to organs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the autoimmune vasculitides?

A

Giant cell arthritis
Granulomatosis polyangiitis (Wegner’s)
Microscopic polyangiitis
Eosinophilic granulomatosis polyangiitis (Churg-Strauss)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How do you diagnose an autoimmune vasculitis?

A

Cardinal clinical features (Ex & Hx)
Immunology
Imaging
Tissue - only way to get a proper diagnosis is via biopsy and microscopy
Exclusion of DDx

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What may mimic autoimmune vasculitis?

A 
Drugs
Infection 
Malignancy 
Cardiac myxoma 
Cholesterol emboli 
Scurvy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What drugs may mimic autoimmune vasculitis?

A

Cocaine (nausea, sweating, unstable HR and BP, chest pain, chills, resp depression)
Minocycline (nausea, diarrhoea, headache, fatigue, myalgia/arthralgia, rash, pruritus)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What infections may mimic autoimmune vasculitis?

A

HIV - myalgia/arthalgia, fever, generalised rash, generalised non-tender lymphadenopathy, headache

Endocarditis
Hepatitis
TB

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What symptoms do you get with endocarditis?

A

Inflammation of inner lining of heart and valves, usually caused by infections

Fever, chills, generalised malaise, weakness, night sweats, pleuritic chest pain, arthalgia/myalgia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What symptoms do you get with hepatitis?

A

Malaise, fever, myalgia/arthalgia, jaundice, pruritus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What symptoms do you get with TB?

A

Wt loss, fever, night sweats, lymphadenopathy, dyspnoea, productive cough

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is a cardiac myxoma and what symptoms do you get with one?

A

Benign heart tumour

SoB, fever, wt loss, palpitations

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What do you get with a cholesterol embolus?

A

Acute renal failure, purpura, GI and NS involvement, e.g. stroke

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is scurvy? What symptoms do you get with it?

A

Lack of vit C

Tiredness, sore arms, legs, gum disease, bleeding from skin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What age and gender tend to get SLE?

A

Young females (9x)
20-22 is peak
Onset: 15-50yo

Oestrogen has a lot to do with the disease (men present when middle aged with diff presentation)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

In which race is SLE more common?

A

Afrocarribeans > Asians >
Caucasians
Also worse in Afrocarribeans

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is the pathophysiology of SLE?

A

GENETICS & ENVIRONMENT

E.g. girl has susceptibility genes and exposed to UV radiation
UV –> cell DNA damage –> apoptosis –> apoptotic bodies (DNA, histones, proteins released from cells - as these are from the nucleus they are ‘nuclear antigens’)
Susceptibility genes mean immune cells more likely to think nuclear antigens are foreign & make Abs against the NA (anti-nuclear antibodies)
Susceptibility genes also –> less able to clear NAs so –> worse build up of NAs
ANAs present in lost SLE and form complex with NAs

Complexes enter BS and deposit/stick to vessel walls/organs (kidneys, joints, skin, heart)
Deposits cause local inflammation reaction in tissues –> damage via activation of complement system –> fully permeable channels in cells –> cells burst and die

Many patient’s also develop Ab against other cells (RBCs/WBC) and molecules (e.g. phospholipids) and its not fully understood why

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is the clinical presentation of SLE?

A

SLE can theoretically affect anywhere in the body

Classical presentation: woman, child bearing age, fever, rash, joint point and symptoms specific to where the complexes have deposited

FLARES and REMITTANCE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What classification criteria is used to diagnose lupus?

A

Don’t need to fufill a criteria to diagnose it

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is the classification criteria for lupus?

A

Any 4 of:
1. malar rash (butterfly) - sparing nasolabial folds, appears after sun exposure
2 - discoid rash (raised, dark, scaley, scarring, permanent marks, alopecia)
3 - photosensitivity
4 - oral ulcers
5 - arthritis (2 joints)
6 - serositis (pleurisy/pericarditis)
7 - renal (significant proteinuria/cellular casts in urine)
8 - neurological (unexplained seizures/psychosis)
9 - haematological (low WCC, platelets, lymphocytes, haemolytic anaemia)
10 - immunological (other Ab and low complement)
11 - ANA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What will SLE arthritis look like on Xray?

A

No damage to the bone, ligaments have softened up - it is fully reversible!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Why might people with SLE and renal involvement have normal U&Es?

A

Dipstick will better show protein/blood in urine which are early signs of kidney disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Why do you get haematological anomalies in SLE?

A

WCC/platelets/lymphocytes/RCs etc are targeted by immune cells and destroyed

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What are the other antibodies as mentioned in 10?

A

Anti-smith
Anti-dsDNA
Antiphospholipids - anti-cardiolipin, lupus anticoagulant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What is anti-smith an Ab against?

A

Small ribonucleoproteins

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What is anti-dsDNA an Ab against?

A

Double stranded DNA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

What are anti-phospholipids an Ab against?

A

Proteins attached to phospholipids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Are ANA only found in SLE?

A

NO

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What other things may cause a butterfly like rash?

A

Rosacea and mitral stenosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

How do you Mx SLE?

A

Avoid sunlight
Corticosteroids
Immunosupressants

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What is scleroderma?

A

Chronic dx caused by abnormal growth of connective tissue which leads to diffuse thickening and hardening of the skin & often inner organs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

What does sclerosis mean?

A

Thickening

33
Q

What is characteristic of scleroderma?

A

Top layer of skin becomes very keratinised and 5x as thick as it should be & loss of skin softness

May also get swelling of hands/feet, joint pain, BV damage –> overreaction to cold/stress (Raynauds)

34
Q

In which gender/age group is scleroderma most common?

A

3x F and 30-50yo

35
Q

What is the pathogenesis of scleroderma?

A

Inflammatory synthesis of ECM - fibroblast proliferation and synthesis of normal collagen
+ non-inflammatory vasculopathy if more severe dx

36
Q

What are morphea?

A

Localised patches of hardened skin
Usually on trunk
Don’t come with over symptoms/pain

37
Q

What are the types of scleroderma?

A

Localised and systemic sclerosis

38
Q

What does localised scleroderma involve?

A

Morphea/linear scleroderma

39
Q

What are the types of systemic sclerosis?

A

Limited

Diffuse

40
Q

What does systemic sclerosis involve?

A

Internal organs & skin involved

41
Q

What does limited systemic sclerosis present like?

A

90% time Raynauds precedes onset of other symptoms
Skin manifestations usually involve hands, fingers, face - DOES NOT PASS ELBOWS
Extracutaneous organ involvement may occur (e.g. difficulty swallowing, heartburn etc.)
May manifest as crest syndrome

42
Q

What does diffuse systemic sclerosis tend to present like?

A

Raynaud’s coinciding/following onset of other symptoms
Skin manifestations spread proximally from trunk –> elbow (thickening and tightening of skin across whole body)
Extracutaneous manifestations common

43
Q

What extracutaneous manifestations may be present in systemic sclerosis?

A

Arthalgia/myalgia
GI
Oesophageal dysmotility - dysphagia/reflux
Small bowel dysmotility - bloating, gas, constipation, cramping
Pulmonary dx - pulmonary HTN, interstitial lung dx
Heart dx - myocarditis, pericarditis
Renal - abnormal collagen deposition –> decreased renal BF & therefore function
ETC

44
Q

What is CREST syndrome?

A

C- calcinosis (calcium deposits, really painful)
R - Raynaud’s
E - oesophageal dysmotility (gullet thickens, peristalsis is abnormal & can affect sphincter, GORD common complaint)
S - sclerodactyly (sausage fingers)
T - telectangasia (small visible BVs around face & Chest)

ASSOC with limited systemic sclerosis

45
Q

What is Raynaud’s phenomena?

A

Vasospasm in BVs of fingers/toes usually due to exposure to cold/stress

46
Q

What are the complications of limited systemic sclerosis?

A

Pulmonary hypertension

47
Q

What are the complications of diffuse systemic sclerosis?

A

Pulmonary fibrosis
Renal crisis
Small bowel bacterial overgrowth (improper peristalsis)

48
Q

In which age group/gender is Sjogren’s most common?

A

9x F

40-50yo

49
Q

What is Sjogren’s syndrome?

A

Autoimmune dx characterised by destruction of the lacrimal and salivary glands

50
Q

What are the clinical features of Sjogren’s?

A

Dry eyes and mouth
Parotid enlargement

1/3 have systemic upset - fatigue, fever, myalgia, arthalgia

Rarely other organs are involved, e.g. nerves/lungs

51
Q

What are the complications of Sjogren’s?

A 
Lymphoma 
Neuropathy 
Purpura 
Interstitial lung dx
Renal tubular acidosis
52
Q

What is autoimmune myositis?

A

Inflammation and degenerative changes in the muscle
Leading to symmetrical muscle weakness and fibrous replacement of muscle
Usually in the limb girdles/proximal thighs

53
Q

What are the clinical features of auto-immune myositis?

A

Muscle weakness - symmetrical, diffuse, proximal

Affecting ADLs

54
Q

What are the types of auto-immune myositis?

A

Polymyositis

Dermatomyositis

55
Q

What is dermatomyositis?

A

Muscle wasting + Gottron’s papules (80%) and heliotrope rash (30-60%)

56
Q

What are Gottron’s papules?

A

Erythematous papules on the extensor surfaces of the hand

57
Q

What is a heliotrope rash?

A

Erythematous rash on upper eye lids & face

58
Q

What are the complications of DM/PM?

A

Cancer/quite often a paraneoplastic syndrome

Interstitial lung disease

59
Q

What is MCTD?

A

Overlapping symptoms of systemic sclerosis, SLE, polymositis

Soft tissue swelling
Raynaud’s
Myositis
Arthalgia

60
Q

What is giant cell arteritis?

A

Autoimmune vasculitis that causes chronic inflammation of large and medium sized vessels

61
Q

What are the clinical features of giant cell arteritis?

A

Temporal arteritis - elderly age, new onset headache around temple, hardened and tender temple artery, jaw claudication

62
Q

How do you Rx temporal arteritis?

A

Medical emergency as can lead to loss of vision

Glucocorticoids

63
Q

What is the GCA classification criteria?

A 
3 of:
age at onset 50y+
new headache
temporal artery tenderness/reduced pulsation 
ESR 50+
Abnormal temporal biopsy
64
Q

What is ANCA?

A

Antineutrophil cytoplasmic Ab
Target antigens in cytoplasm of neutrophils
Burst inappropriately and when they come into contact with BVs they destroy BVs

65
Q

What are the ANCA associated vasculitides?

A

Granulomatosis with polyangiitis (wegner’s)
Microscopic polyangiitis
Eosinophilic granulomatosis with polyangiitis

These affect small BVs

66
Q

What is involved in Granulomatosis with polyangiitis?

A

Necrotising granulomatous inflammation
Usually involving upper and lower RT
Affecting small–> med essels
Necrotising GN is common

67
Q

If someone presents with sinus disease, chest cavities and kidney disease what should you think about?

A

GPA

68
Q

What is microscopic polyangiitis?

A

Necrotising vasculitis with few/no immune deposits affecting small vessels
Necrotising arteritis involving small and medium arteries may be present
Necrotising GN v common
Pulmonary capillaritis often occurs
Granulomatous inflammation absent

similar to GPA without nasopharynx involvement!

69
Q

What is Eosinophilic granulomatosis with polyangiitis?

A

Eosinophil rich and necrotising granulomatous inflammation often involving the resp tract
Necrotising vasculitis predominantly affecting small–> med vessels
Assoc with asthma & eosinophilia
ANCa more frequent when GN present

70
Q

What are some of the major complications with ANCA associated vasculitis?

A 
Nerve damage
Malignancy 
Lung damage
ESRD
Hearing loss
71
Q

What conditions are assoc with ANA?

A 
SLE - 99%
Systemic sclerosis - 97%
PM/DM - 40-80%
Sjogrens - 48-96%
MCTD - 100%
Drug induced lupus - 100%

Asymptomatic people may also have ANA, may also be present in RA, MS and infection

72
Q

What ANA antibodies are present in SLE?

A

Anti-dsDNA
Anti-Ro
Anti-Sm

73
Q

What ANA antibodies are present in scleroderma?

A

Anti-Scl-70

Anti-centromere

74
Q

What ANA antibodies are present in polymositis?

A

Anti-Jo1

75
Q

What ANA antibodies are present in Sjogren’s?

A

Anti-Ro

Anti-La

76
Q

What Ix might you do in multi-system autoimmune dx?

A

Radiology (PET, MRI, CT)

Biopsy, e.g. of kidneys, temporal arteries

77
Q

What are the classes of lupus nephritis?

A 
1 - minimal mesangial 
2 - mesangial proliferation 
3 - focal 
4 - diffuse
5 - membranous
6 - advanced sclerosing
78
Q

What are the treatments for multi-system autoimmune diseases?

A

Mild - hydroxychloroquine
Mod - azathioprine, methotrexate, mycophenolate
Severe - cyclophosphamide, rituximab

MSK (27 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions