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Multi System Autoimmune Diseases Flashcards

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1
Q

Progression of Wegener’s Granulomatosis

A

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

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2
Q

Progression of lupus

A

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

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3
Q

Progression of scleroderma

A

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

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4
Q

Progression of sjorgens syndrome

A

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

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5
Q

Connective tissue autoimmune diseases

A 
Systemic lupus erythematous (SLE)
Scleroderma
Sjogrens syndrome
Autoimmune myositis
Mixed connective tissue disease
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6
Q

Systemic vasculitis autoimmune diseases

A

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

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7
Q

Diagnosis of multi system autoimmune diseases

A 
Cardinal clinical features - history and exam 
Immunology 
Imaging
Tissue
Exclusion of differential diagnosis
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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
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9
Q

Who does SLE affect?

A

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

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10
Q

Why do more females get affected by SLE than males?

A

To do with oestrogen

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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

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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
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13
Q

Immunological features of SLE

A 
Anti ds-DNA
Anti-smith 
Cardiolipin 
Lupus anticoagulant 
Low complement 
ANA
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14
Q

Who gets scleroderma?

A

30-50 y/o onset

F > M 3:1

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15
Q

Presentation of scleroderma

A 
Morphea; constraints of the skin 
Thickening limited to hands and forearms 
calcium deposits 
Sausage fingers
Telectasia
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16
Q

Presentation of diffuse scleroderma

A

Thickening of the skin can go across the whole body

Mophrea; constriants of the skin

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17
Q

Types of scleroderma

A

Limited

Diffuse (less common)

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18
Q

Definition of telectasia

A

Visible blood vessels prominent

usually around neck or chest

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19
Q

Complications of scleroderma

A 
aesthetics 
Limited; pulmonary HTN
Diffuse; 
- pulmonary fibrosis
- renal crisis 
- small bower bacterial overgrowth
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20
Q

Who gets Sjorgen’s Syndrome?

A

Onset 40-50 years

F > M 9:1

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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

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22
Q

Complications of sjorgens syndrome

A 
Lymphoma
Neuropathy 
Purpura
Interstitial lung disease 
Renal tubular acidosis
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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)
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24
Q

Complications of autoimmune myositis

A

cancer

interstitial lung disease

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25
An overlap syndrome and its features
MCTD - soft tissue swelling - Raynauds - myositis - arthralgia
26
Large vessel vasculitis examples
Takayasu arteritis Giant cell arteritis (GCA) Buergers disease
27
Medium vessel vasculitis examples
Polyarteritis nodose Kawasaki disease Wegeners
28
Small vessel vasculitis examples
``` ANCA associated vasculitis - microscopic polyangiitis - granulomatosis with polyangiitis - eosinophilic granulomatosis with polyangiitis Immune complex SVV - IgA vasculitis - anti-GBM disease ```
29
Classification criteria for GCA
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
30
What does GCA stand for?
Giant cell arteritis
31
What does ANCA AAV stand for?
ANCA associated vasculitis
32
What does ANCA stand for?
Antineutrophil cytoplasmic antibody
33
Features of ANCA
Antibody Neutrophils are activated and burst inappropriately when they come into contact with a blood vessel This destroys the blood vessel
34
What is another name for Granulomatosis with Polyangitis?
Wegener's granulomatosis
35
What are ANCA associated vasculitis?
Wegener's (Graulomatosis with polyangiitis) Microscopic polyangiitis Eosinophilic granulomatosis with polyangiitis
36
Features of Wegener's granulomatosis
Necrotising granulomatous inflammation Usually involving the upper and lower respiratory tract Affecting predominately small to medium vessels Necrotising glomerulonephritis common
37
Features of microscopic granulomatosis
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
Features of eosinophilic granulomatosis with polyangiitis
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
What does ANA stand for?
Antinuclear antibody
40
Conditions where ANA is raised
``` 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
What does MCTD stand for?
Mixed connective tissue disease
42
What is MCTD?
A broader category of rheumatoid "overlap" syndromes, used to describe when a patient has features of more than one classic inflammatory rheumatic disease
43
Situations where ANA is raised "unhelpfully"
RA MS Infection variation
44
Specific ANA profile of scleroderma
Scl-70 | Centomere
45
Specific ANA profile of SLE
dSDNA Ro Sm
46
Specific ANA profile of polymyositis
Jo-1
47
Specific ANA profiles of sjorgens disease
Ro | La
48
Classes of lupus nephritis from biopsy
``` I - minimal mesangial II - mesangial proliferative III - focal IV - diffuse V - membranous VI - advanced sclerosing ```
49
Treatment of multisystem autoimmune diseases
Organ threat mild = hydroxychloroquine Organ threat moderate - azathioprine - methotrexate - mycophenolate Organ threat severe - cyclophosphamide - Rituximab
50
What is polyarteritis nodosa?
Vasculitis affecting medium sized arteries with necrotising inflammation leading to aneurysm formation
51
Who is polyarteritis nodosa common in?
Middle aged men
52
What is polyarteritis nodosa associated with?
Hepatitis B infection
53
Presentation of polyarteritis nodosa
``` 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
Treatment of raynauds
Nifedipine
55
Investigations for sjogrens syndrome
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
What is seen in drug induced lupus?
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
Immunology of drug induced lupus
ANA +ve in 100% dsDNA -ve Anti-histone antibodies in 80-90% anti-Ro, anti smith +ve in 5%
58
Most common causes of drug induced lupus
Procainamide | Hydralazine
59
Less common causes of drug induced lupus
Isoniazid Minocycline Phenytoin
60
What is Takayasu's arteritis and what gender gets it?
Inflammatory, obliterative arteritis affecting aorta and branches F > M
61
Presentation of takayasus arteritis
Upper limb claudication Diminished or absent pulses ESR affected in the acute phase
62
What is beurgers disease?
Segmental thrombotic occlusions of the small and medium lower limb vessels
63
Who is beurgers disease common in?
Young male smokers
64
Presentation of beurgers disease
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
What is antiphospholipid syndrome?
An acquired disorder characterised by predisposition to both venous and arterial thrombosis, recurrent foetal loss and thrombocytopenia
66
How may antiphospholipid syndrome occur?
Primarily | Secondary to other conditions (most commonly SLE)
67
What does antiphospholipid syndrome cause an increase in?
APTT
68
Presentation of antiphospholipid syndrome
``` Venous / arterial thrombosis recurrent foetal loss livedo reticularis thrombocytopenia prolonged APTT PET PHTN ```
69
Management of antiphospholipid syndrome
Initial VTEs - warfarin with target INR 2 - 3 for 6 months Recurrent VTEs - lifelong warfarin Arterial thrombosis - lifelong warfarin
70
What test can assist in diagnosing antiphospholipid syndrome?
+ve anti-cardiolipin antibody
71
Causes of +ve cANCA
``` Wegeners granulomatosis (90%) Microscopic polyangiitis (40%) ```
72
Causes of +ve pANCA
Microscopic polyangiitis (50-75%) Churg strauss syndrome (60%) PSC (60 - 80%) Wegeners (25%)
73
What is dermatomyositis?
An inflammatory disorder causing symmetrical, proximal muscle weakness and characteristic skin lesions
74
Causes of dermatomyositis
Idiopathic Connective tissue disease Malignancy
75
What is usually screened for after a diagnosis of dermatomyositis?
Malignancy
76
What is polymyositis?
A variant of dermatomyositis where the skin is not involved
77
Presentation of dermatomyoisitis
``` 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
What are gottrons papules?
Roughed red papules over extensor surfaces of fingers
79
Investigations of dermatomyoisitis
80% ANA +ve | 30% anti-synthetase Abs
80
Who gets still disease?
15 - 25 y/o | 35 - 46 y/o
81
Presentation of stills disease
``` 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
Criteria used to diagnose stills disease
Yamaguchi criteria
83
Management of stills disease
``` NSAIDs 1st line Steriods If persist - Methotrexate - IL-1 - anti-TNF therapy ```
84
Antibodies present in drug induced lupus
Anti-histone antibodies (95%)
85
Patients with sjrogens syndrome are at increased risk of what?
Lymphoid malignancies

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