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

Progression of Wegener’s Granulomatosis

A

Myalgia/arthralgia ->
Episcleritis ->
Sinusitis ->
Renal failure

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

Progression of lupus

A

Arthralgia/myalgia ->
Skin rash ->
Pleurisy ->
CVA

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

Progression of scleroderma

A

Arthralgia/myalgia ->
Skin thickening ->
GORD ->
Pulmonary HTN

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

Progression of sjorgens syndrome

A

Arthralgia/fatigue ->
Sicca ->
Skin rash ->
Neuropathy

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

Connective tissue autoimmune diseases

A 
Systemic lupus erythematous (SLE)
Scleroderma
Sjogrens syndrome
Autoimmune myositis
Mixed connective tissue disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Systemic vasculitis autoimmune diseases

A

GCA
Granulomatosis Polyangitis (Wegeners)
Microscopic polyangiitis
Eosinophilic granulomatosis polyangiitis (churg-straus)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Diagnosis of multi system autoimmune diseases

A 
Cardinal clinical features - history and exam 
Immunology 
Imaging
Tissue
Exclusion of differential diagnosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What can mimic the multi system autoimmune diseases?

A 
Drugs - cocaine, PTU, minocycline
Infection - HIV, Endocarditis, hepatitis, TB
Malignancy - lymphoma
Cardiac myxoma
Cholesterol emboli 
Scurvy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Who does SLE affect?

A

Disease of the young - 15-50 years
F > M; 9:1
Afrocarribeans > Asian > Caucasian

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

Why do more females get affected by SLE than males?

A

To do with oestrogen

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

Presentation of SLE

A

Face - butterfly rash, photosensitivity
Fever
Skin - vasculitis, purpura, urticaria (hives), discoid (scaley centre, dark rim)
Lungs - Pleurisy, Pleural effusion, Fibrosis (pleural)
Raynauds phenomenon
Kidney effects
Joints
- aseptic necrosis of the hips
- arthritis in small joints
Alopecia
Neuro - Fits, Hemiplegia, Ataxia, Peripheral neuropathy, Cranial nerve lesions
Heart - Pericarditis, Endocarditis, Aortic valve lesions
Abdominal pain
Myopathy
Haem - Anaemia, Leukopenia, Thrombocytopenia

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

Classification criteria for SLE; any 4 of….

A 
malar rash (butterfly rash)
Discoid rash (raised, scarring, alopecia)
Photosensitivity 
Oral ulcers 
Arthritis (at least 2 joints)
Serositis (pleurisy or pericarditis)
Renal (significant proteinuria or cellular casts in urine)
Neuro; unexplained seizures or psychosis 
Haematological; 
- low WCC
- platelets
- lymphocytes
- haemolytic anaemia
Immunological 
- Anti ds-DNA 
- SM
- cardiolipin 
- Lupus anticoagulant 
- low complement 
ANA
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Immunological features of SLE

A 
Anti ds-DNA
Anti-smith 
Cardiolipin 
Lupus anticoagulant 
Low complement 
ANA
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Who gets scleroderma?

A

30-50 y/o onset

F > M 3:1

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

Presentation of scleroderma

A 
Morphea; constraints of the skin 
Thickening limited to hands and forearms 
calcium deposits 
Sausage fingers
Telectasia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Presentation of diffuse scleroderma

A

Thickening of the skin can go across the whole body

Mophrea; constriants of the skin

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

Types of scleroderma

A

Limited

Diffuse (less common)

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

Definition of telectasia

A

Visible blood vessels prominent

usually around neck or chest

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

Complications of scleroderma

A 
aesthetics 
Limited; pulmonary HTN
Diffuse; 
- pulmonary fibrosis
- renal crisis 
- small bower bacterial overgrowth
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Who gets Sjorgen’s Syndrome?

A

Onset 40-50 years

F > M 9:1

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

Presentation of Sjorgen’s Syndrome

A

Primarily attacks salivary + lacrimal glands (struggle with QoL)
- dry mouth
- cannot cry
GI - dysphasia, abnormal oesophageal motility
Pulmonary - interstitial lung disease
Neuro - fits, hemiplegia, ataxia, cranial nerve lesions, sensory neuropathy
Renal - renal tubular acidosis
Skin - palpable purpura, raynauds syndrome
mononeuritis multiplex
Parotid gland enlargement
1/3 have systemic upset
- fatigue
- fever
- myalgia
- arthralgia

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

Complications of sjorgens syndrome

A 
Lymphoma
Neuropathy 
Purpura
Interstitial lung disease 
Renal tubular acidosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Presentation of autoimmune myositis

A 
tends to be proximal muscles affected 
NO PAIN 
weakness
- symmetrical 
- diffuse 
- proximal 
- polymyositis
- dermatomyositis (Gortons papules, helitrope rash)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Complications of autoimmune myositis

A

cancer

interstitial lung disease

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

An overlap syndrome and its features

A

MCTD

  • soft tissue swelling
  • Raynauds
  • myositis
  • arthralgia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Large vessel vasculitis examples

A

Takayasu arteritis
Giant cell arteritis (GCA)
Buergers disease

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

Medium vessel vasculitis examples

A

Polyarteritis nodose
Kawasaki disease
Wegeners

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

Small vessel vasculitis examples

A 
ANCA associated vasculitis
 - microscopic polyangiitis
- granulomatosis with polyangiitis
- eosinophilic granulomatosis with polyangiitis 
Immune complex SVV
- IgA vasculitis
- anti-GBM disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Classification criteria for GCA

A

3 of the following

  • age at onset > 50 years
  • new headache around the temporal artery
  • temporal artery tenderness/pulsation (occluded arteries
  • ESR > 50
  • abnormal temporal artery biopsy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What does GCA stand for?

A

Giant cell arteritis

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

What does ANCA AAV stand for?

A

ANCA associated vasculitis

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

What does ANCA stand for?

A

Antineutrophil cytoplasmic antibody

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Features of ANCA

A

Antibody
Neutrophils are activated and burst inappropriately when they come into contact with a blood vessel
This destroys the blood vessel

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

What is another name for Granulomatosis with Polyangitis?

A

Wegener’s granulomatosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

What are ANCA associated vasculitis?

A

Wegener’s (Graulomatosis with polyangiitis)
Microscopic polyangiitis
Eosinophilic granulomatosis with polyangiitis

36
Q

Features of Wegener’s granulomatosis

A

Necrotising granulomatous inflammation
Usually involving the upper and lower respiratory tract
Affecting predominately small to medium vessels
Necrotising glomerulonephritis common

37
Q

Features of microscopic granulomatosis

A

Necrotising vasculitis
Few or no immune deposits
Predominately affecting small vessels
necrotising arteries involving small and medium arteries may be present
necrotising glomerulonephritis very common
Granulomatous inflammation is absent

38
Q

Features of eosinophilic granulomatosis with polyangiitis

A

Eosinophil rich and necrotising granulomatous inflammation often involving the respiratory tract
Necrotising vasculitis predominately affecting small to medium vessels
Associated with asthma and eosinophilia
ANCA more frequent when glomerulonephritis is present

39
Q

What does ANA stand for?

A

Antinuclear antibody

40
Q

Conditions where ANA is raised

A 
SLE 99%
Systemic sclerosis 97%
Poly/dermatomyositis 40-80%
Sjorgrens syndrome 48-95%
MCTD 100% 
Drug induced lupus 100%
IBD (UC > CD) (usually pANCA)
Autoimmune hepatitis (usually pANCA)
41
Q

What does MCTD stand for?

A

Mixed connective tissue disease

42
Q

What is MCTD?

A

A broader category of rheumatoid “overlap” syndromes, used to describe when a patient has features of more than one classic inflammatory rheumatic disease

43
Q

Situations where ANA is raised “unhelpfully”

A

RA
MS
Infection variation

44
Q

Specific ANA profile of scleroderma

A

Scl-70

Centomere

45
Q

Specific ANA profile of SLE

A

dSDNA
Ro
Sm

46
Q

Specific ANA profile of polymyositis

A

Jo-1

47
Q

Specific ANA profiles of sjorgens disease

A

Ro

La

48
Q

Classes of lupus nephritis from biopsy

A 
I - minimal mesangial 
II - mesangial proliferative
III - focal 
IV - diffuse
V - membranous 
VI - advanced sclerosing
49
Q

Treatment of multisystem autoimmune diseases

A

Organ threat mild = hydroxychloroquine

Organ threat moderate

  • azathioprine
  • methotrexate
  • mycophenolate

Organ threat severe

  • cyclophosphamide
  • Rituximab
50
Q

What is polyarteritis nodosa?

A

Vasculitis affecting medium sized arteries with necrotising inflammation leading to aneurysm formation

51
Q

Who is polyarteritis nodosa common in?

A

Middle aged men

52
Q

What is polyarteritis nodosa associated with?

A

Hepatitis B infection

53
Q

Presentation of polyarteritis nodosa

A 
Fever
Malaise
Arthralgia 
HTN
Weight loss
Mononeuritis multiplex, sensorimotor polyneuropathy 
Testicular pain 
Livedo reticularis 
Hamaeturia, renal failure (renal disease seen in 70%)
Angiography can show saccular or fusiform aneurysms and arterial stenosis 
ANCA in 20%
Hep B serology +ve in 30%
54
Q

Treatment of raynauds

A

Nifedipine

55
Q

Investigations for sjogrens syndrome

A

RF +ve in 100%
ANA +ve in 70%
Primary disease - anti-Ro and anti-La
Schrimers test (filter paper near conjunctival sac to measure tear formation)
Focal lymphocytic infiltration on histology

56
Q

What is seen in drug induced lupus?

A

Not all features of lupus have to be present
Renal and nervous system involvement UNUSUAL
Athralgia
Myalgia
Skin e.g. malar rash
Pulmonary involvement e.g. pleurisy common

57
Q

Immunology of drug induced lupus

A

ANA +ve in 100%
dsDNA -ve
Anti-histone antibodies in 80-90%
anti-Ro, anti smith +ve in 5%

58
Q

Most common causes of drug induced lupus

A

Procainamide

Hydralazine

59
Q

Less common causes of drug induced lupus

A

Isoniazid
Minocycline
Phenytoin

60
Q

What is Takayasu’s arteritis and what gender gets it?

A

Inflammatory, obliterative arteritis affecting aorta and branches
F > M

61
Q

Presentation of takayasus arteritis

A

Upper limb claudication
Diminished or absent pulses
ESR affected in the acute phase

62
Q

What is beurgers disease?

A

Segmental thrombotic occlusions of the small and medium lower limb vessels

63
Q

Who is beurgers disease common in?

A

Young male smokers

64
Q

Presentation of beurgers disease

A

Proximal pulses usually present but pedal pulses are lost
Acute hypercellular occlusive thrombus usually present
Tortous corkscrew shaped collateral vessels may be seen on angiography

65
Q

What is antiphospholipid syndrome?

A

An acquired disorder characterised by predisposition to both venous and arterial thrombosis, recurrent foetal loss and thrombocytopenia

66
Q

How may antiphospholipid syndrome occur?

A

Primarily

Secondary to other conditions (most commonly SLE)

67
Q

What does antiphospholipid syndrome cause an increase in?

A

APTT

68
Q

Presentation of antiphospholipid syndrome

A 
Venous / arterial thrombosis
recurrent foetal loss
livedo reticularis
thrombocytopenia
prolonged APTT 
PET
PHTN
69
Q

Management of antiphospholipid syndrome

A

Initial VTEs - warfarin with target INR 2 - 3 for 6 months
Recurrent VTEs - lifelong warfarin
Arterial thrombosis - lifelong warfarin

70
Q

What test can assist in diagnosing antiphospholipid syndrome?

A

+ve anti-cardiolipin antibody

71
Q

Causes of +ve cANCA

A 
Wegeners granulomatosis (90%)
Microscopic polyangiitis (40%)
72
Q

Causes of +ve pANCA

A

Microscopic polyangiitis (50-75%)
Churg strauss syndrome (60%)
PSC (60 - 80%)
Wegeners (25%)

73
Q

What is dermatomyositis?

A

An inflammatory disorder causing symmetrical, proximal muscle weakness and characteristic skin lesions

74
Q

Causes of dermatomyositis

A

Idiopathic
Connective tissue disease
Malignancy

75
Q

What is usually screened for after a diagnosis of dermatomyositis?

A

Malignancy

76
Q

What is polymyositis?

A

A variant of dermatomyositis where the skin is not involved

77
Q

Presentation of dermatomyoisitis

A 
Photosensitive
Macular rash over back and shoulder
Heliotrope rash in periorbital region 
Gottrons papules 
Mechanic hands - extremely dry and scaley hands with linear cracks on palmar and lateral aspects of fingers
Nail fold capillary dilatation 
Proximal muscle weakness +/- tenderness
Raynauds
Resp muscle weakness 
ILD
Dysphagia / dysphonia
78
Q

What are gottrons papules?

A

Roughed red papules over extensor surfaces of fingers

79
Q

Investigations of dermatomyoisitis

A

80% ANA +ve

30% anti-synthetase Abs

80
Q

Who gets still disease?

A

15 - 25 y/o

35 - 46 y/o

81
Q

Presentation of stills disease

A 
Arthalgia
Elevated serum ferritin 
Salmon pink maculopapular rash 
Pyrexia (rises in late afternoon / early evening in a daily pattern and accompanies the joint pain and rash)
Lymphadenopathy 
RF and ANA -ve
82
Q

Criteria used to diagnose stills disease

A

Yamaguchi criteria

83
Q

Management of stills disease

A 
NSAIDs 1st line
Steriods
If persist 
- Methotrexate
- IL-1
- anti-TNF therapy
84
Q

Antibodies present in drug induced lupus

A

Anti-histone antibodies (95%)

85
Q

Patients with sjrogens syndrome are at increased risk of what?

A

Lymphoid malignancies

MSK (25 decks)

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