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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.)

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2
Q

What are examples of autoimmune connective tissue diseases?

A 
SLE
Slceroderma
Sjogren's syndrome
Autoimmune myositis
Mixed connective tissue disease
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3
Q

What is autoimmune vasculitis?

A

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

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4
Q

What are the autoimmune vasculitides?

A

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

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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

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6
Q

What may mimic autoimmune vasculitis?

A 
Drugs
Infection 
Malignancy 
Cardiac myxoma 
Cholesterol emboli 
Scurvy
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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)

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8
Q

What infections may mimic autoimmune vasculitis?

A

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

Endocarditis
Hepatitis
TB

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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

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10
Q

What symptoms do you get with hepatitis?

A

Malaise, fever, myalgia/arthalgia, jaundice, pruritus

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11
Q

What symptoms do you get with TB?

A

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

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12
Q

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

A

Benign heart tumour

SoB, fever, wt loss, palpitations

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13
Q

What do you get with a cholesterol embolus?

A

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

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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

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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)

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16
Q

In which race is SLE more common?

A

Afrocarribeans > Asians >
Caucasians
Also worse in Afrocarribeans

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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

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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

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19
Q

What classification criteria is used to diagnose lupus?

A

Don’t need to fufill a criteria to diagnose it

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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

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21
Q

What will SLE arthritis look like on Xray?

A

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

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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

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23
Q

Why do you get haematological anomalies in SLE?

A

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

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24
Q

What are the other antibodies as mentioned in 10?

A

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

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25
What is anti-smith an Ab against?
Small ribonucleoproteins
26
What is anti-dsDNA an Ab against?
Double stranded DNA
27
What are anti-phospholipids an Ab against?
Proteins attached to phospholipids
28
Are ANA only found in SLE?
NO
29
What other things may cause a butterfly like rash?
Rosacea and mitral stenosis
30
How do you Mx SLE?
Avoid sunlight Corticosteroids Immunosupressants
31
What is scleroderma?
Chronic dx caused by abnormal growth of connective tissue which leads to diffuse thickening and hardening of the skin & often inner organs
32
What does sclerosis mean?
Thickening
33
What is characteristic of scleroderma?
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
In which gender/age group is scleroderma most common?
3x F and 30-50yo
35
What is the pathogenesis of scleroderma?
Inflammatory synthesis of ECM - fibroblast proliferation and synthesis of normal collagen + non-inflammatory vasculopathy if more severe dx
36
What are morphea?
Localised patches of hardened skin Usually on trunk Don't come with over symptoms/pain
37
What are the types of scleroderma?
Localised and systemic sclerosis
38
What does localised scleroderma involve?
Morphea/linear scleroderma
39
What are the types of systemic sclerosis?
Limited | Diffuse
40
What does systemic sclerosis involve?
Internal organs & skin involved
41
What does limited systemic sclerosis present like?
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
What does diffuse systemic sclerosis tend to present like?
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
What extracutaneous manifestations may be present in systemic sclerosis?
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
What is CREST syndrome?
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
What is Raynaud's phenomena?
Vasospasm in BVs of fingers/toes usually due to exposure to cold/stress
46
What are the complications of limited systemic sclerosis?
Pulmonary hypertension
47
What are the complications of diffuse systemic sclerosis?
Pulmonary fibrosis Renal crisis Small bowel bacterial overgrowth (improper peristalsis)
48
In which age group/gender is Sjogren's most common?
9x F | 40-50yo
49
What is Sjogren's syndrome?
Autoimmune dx characterised by destruction of the lacrimal and salivary glands
50
What are the clinical features of Sjogren's?
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
What are the complications of Sjogren's?
``` Lymphoma Neuropathy Purpura Interstitial lung dx Renal tubular acidosis ```
52
What is autoimmune myositis?
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
What are the clinical features of auto-immune myositis?
Muscle weakness - symmetrical, diffuse, proximal Affecting ADLs
54
What are the types of auto-immune myositis?
Polymyositis | Dermatomyositis
55
What is dermatomyositis?
Muscle wasting + Gottron's papules (80%) and heliotrope rash (30-60%)
56
What are Gottron's papules?
Erythematous papules on the extensor surfaces of the hand
57
What is a heliotrope rash?
Erythematous rash on upper eye lids & face
58
What are the complications of DM/PM?
Cancer/quite often a paraneoplastic syndrome | Interstitial lung disease
59
What is MCTD?
Overlapping symptoms of systemic sclerosis, SLE, polymositis Soft tissue swelling Raynaud's Myositis Arthalgia
60
What is giant cell arteritis?
Autoimmune vasculitis that causes chronic inflammation of large and medium sized vessels
61
What are the clinical features of giant cell arteritis?
Temporal arteritis - elderly age, new onset headache around temple, hardened and tender temple artery, jaw claudication
62
How do you Rx temporal arteritis?
Medical emergency as can lead to loss of vision | Glucocorticoids
63
What is the GCA classification criteria?
``` 3 of: age at onset 50y+ new headache temporal artery tenderness/reduced pulsation ESR 50+ Abnormal temporal biopsy ```
64
What is ANCA?
Antineutrophil cytoplasmic Ab Target antigens in cytoplasm of neutrophils Burst inappropriately and when they come into contact with BVs they destroy BVs
65
What are the ANCA associated vasculitides?
Granulomatosis with polyangiitis (wegner's) Microscopic polyangiitis Eosinophilic granulomatosis with polyangiitis These affect small BVs
66
What is involved in Granulomatosis with polyangiitis?
Necrotising granulomatous inflammation Usually involving upper and lower RT Affecting small--> med essels Necrotising GN is common
67
If someone presents with sinus disease, chest cavities and kidney disease what should you think about?
GPA
68
What is microscopic polyangiitis?
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
What is Eosinophilic granulomatosis with polyangiitis?
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
What are some of the major complications with ANCA associated vasculitis?
``` Nerve damage Malignancy Lung damage ESRD Hearing loss ```
71
What conditions are assoc with ANA?
``` 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
What ANA antibodies are present in SLE?
Anti-dsDNA Anti-Ro Anti-Sm
73
What ANA antibodies are present in scleroderma?
Anti-Scl-70 | Anti-centromere
74
What ANA antibodies are present in polymositis?
Anti-Jo1
75
What ANA antibodies are present in Sjogren's?
Anti-Ro | Anti-La
76
What Ix might you do in multi-system autoimmune dx?
Radiology (PET, MRI, CT) | Biopsy, e.g. of kidneys, temporal arteries
77
What are the classes of lupus nephritis?
``` 1 - minimal mesangial 2 - mesangial proliferation 3 - focal 4 - diffuse 5 - membranous 6 - advanced sclerosing ```
78
What are the treatments for multi-system autoimmune diseases?
Mild - hydroxychloroquine Mod - azathioprine, methotrexate, mycophenolate Severe - cyclophosphamide, rituximab

MSK (27 decks)

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