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Year 3 - MSK > Mini Symposium: Multi-system Autoimmune Disease > Flashcards

Mini Symposium: Multi-system Autoimmune Disease Flashcards

1
Q

what are some Connective Tissue Diseases?

A

◦Systemic Lupus Erythematosus

◦Scleroderma

◦Sjogren’s syndrome

◦Auto-immune myositis

◦Mixed connective tissue disease

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2
Q

what are examples of Systemic vasculitis?

A

◦Giant cell arteritis

◦Granulomatosis polyangiitis (Wegeners)

◦Microscopic polyangiitis

◦Eosinophilic granulomatosis polyangiitis (Churg-Strauss)

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3
Q

how is a diagnosis made?

A

Cardinal clinical features: History & Exam

Bedside investigations

Immunology

Imaging

Tissue diagnosis

Exclusion of differential diagnosis

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4
Q

What are some mimics?

A

Drugs - cocaine, minocyline, PTU

Infection - HIV, endocarditis, Hepatitis, TB

Malignancy - lymphoma

Cardiac myxoma

Cholesterol emboli

Scurvey

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5
Q

what is Systemic lupus erythematosus?

A

Lupus is a condition that affects the immune system. It can cause problems with your skin, joints, kidneys and other organs

Systemic lupus erythematosus (SLE) – lupus – is a long-term condition causing inflammation to the joints, skin and other organs. There’s no cure, but symptoms can improve if treatment starts early

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6
Q

what is the epidemiology of SLE?

A

UK Prevalence: 28/100,000

UK incidence: 4/100,000

Female:Male 9:1

Onset: 15-50 years

Significant ethnic diversity:

Afro-Caribbeans>Asian>Caucasian

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7
Q

what is SLE aetiology?

A

Genetic factors – high concordance rate of SLE in monozygotic twins, Sibling risk of developing SLE is 30-fold higher than in the general population, polygenic mode of inheritance

Hormonal factors

Environmental factors – ultraviolet light, drugs, infections

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8
Q

what is SLE pathogenesis?

A

Immune response against endogenous nuclear antigens (break in immunological tolerance)

Immune complex formation

Complement activation

Tissue injury

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9
Q

where can SLE effect?

A

Clinical presentation is varied

Different organs that may be involved in lupus

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10
Q

what is the classification criteria? (any 4)

A

1) Malar rash (butterfly rash)
2) Discoid rash (raised, scarring, permanent marks, alopecia)
3) Photosensitivity
4) Oral ulcers
5) Arthritis (2 joints at least) - Non erosive, bilateral
6) Serositis (pleurisy or pericarditis)
7) Renal (significant proteinuria or cellular casts in urine)
8) Neurological (unexplained seizures or psychosis)
9) Haematological (low WCC, platelets, lymphocytes, haemolytic anaemia)
10) Immunological (anti ds-DNA, SM, cardiolipin, lupus anticoagulant, low complement)
11) ANA

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11
Q

photos showing SLE: Skin manifestations

A

DDx butterfly: rosacea, mitral stenosis

Discoid: Scaly centre, dark rim

Malor rash and discoid rash

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12
Q

in someone with SLE what is one of the first things you should do?

A

urine analysis

use this to check renail involvement - make sure there isnt any

lupus nephritis

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13
Q

when would you consider a diagnosis of SLE?

A

Usually seen in women of child bearing age

Constitutional symptoms of fever, weight loss, malaise, severe fatigue

Skin rash and/or stomatitis

Arthritis

Pleuritic chest pain

Renal disease

cytopenia - Cytopenia occurs when one or more of your blood cell types is lower than it should be

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14
Q

what autoantibodies are involved in SLE?

A

ANA – antinuclear antibodies

Seen in 95% of SLE

Not specific for SLE

Seen in many inflammatory, infectious and neoplastic diseases

Seen in 5% to 15% of healthy population

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15
Q

what are ANA?

A

antinuclear antibodies (ANA)

our immune system normally makes antibodies to help you fight infection. In contrast, antinuclear antibodies often attack your body’s own tissues — specifically targeting each cell’s nucleus

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16
Q

what are some Conditions where +ve ANA
is unhelpful?

A
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17
Q

in different condiitons what % of ANA is present?

A
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18
Q

what are osme other autoantibodies that many be seen in SLE?

A

Anti -ds DNA:

Seen in 60% of patients with SLE

Highly specific for SLE

Low titre rarely seen in other inflammatory conditions

Strongest clinical association is with nephritis

Anti -Sm (Smith):

Seen in 10% to 30% of SLE patients

Highly specific for SLE

Anti–Ro:

Risk of foetal congenital heart block

Neonatal lupus

Antiphospholipid antibodies:

Anti-cardiolipin, lupus anticoagulant

arterial/venous thrombosis

miscarriages

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19
Q

what is Systemic sclerosis?

A

Systemic scleroderma, or systemic sclerosis, is an autoimmune rheumatic disease characterised by excessive production and accumulation of collagen, called fibrosis, in the skin and internal organs and by injuries to small arteries

connective tissue disease characterised by fibrosis of the skin and internal organs

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20
Q

what are the different Classification of scleroderma?

A

Localised scleroderma

Systemic sclerosis (SSc):

  • Limited cutaneous systemic sclerosis (CREST)
  • Diffuse cutaneous systemic sclerosis
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21
Q

what si the difference between localised and systemic scleroderma?

A

Localised scleroderma (also known as morphoea or morphea) only affects the skin. In some cases it can spread to the tissues underneath the skin, such as muscles and bones. Systemic sclerosis affects the skin but may also involve the body’s internal organs

(pictures showing localised scleroderma)

22
Q

what is the epidemiology of systmeic scleroderma?

A

UK Prevalence: 24/100,000

UK Incidence: 10/1,000,000

Onset: 30-50 years

Female : Male 3:1

23
Q

what is the aetiology of systemic scleroderma?

A

Environmental:

  • Silica
  • Solvents
  • Viral infections

Genetic predisposition

24
Q

What is the pathogenesis of scleroderma?

A

Vascular damage (microcirculation)

Immune system activation/Inflammation

Fibrosis

25
what is Limited cutaneous SSc?
Limited cutaneous systemic sclerosis (lcSSc) is a subtype of systemic sclerosis (SSc; see this term) characterized by the association of Raynaud's phenomenon with skin fibrosis limited to the hands, face, feet and forearms
26
What is Diffuse cutaneous SSc?
Diffuse cutaneous systemic sclerosis (dcSSc) is a subtype of Systemic Sclerosis (SSc; see this term) characterized by truncal and acral skin fibrosis with an early and significant incidence of diffuse involvement (interstitial lung disease, oliguric renal failure, diffuse gastrointestinal disease, and myocardial involvement).
27
SSc subsets - what may limited and diffuse cause?
Limited: Anti centromere antibodies Pulmonary hypertension Gastrointestinal Diffuse: Anti Scl70 antibodies Pulmonary fibrosis Renal crisis Small bowel bacterial overgrowth
28
what is Sjogren’s syndrome?
Systemic autoimmune disease Characterised by lymphocytic infiltrates of the salivary and tear glands leading to oral and ocular dryness and autoantibody secretion Sjögren's (pronounced show-grins) syndrome is a condition that affects parts of the body that produce fluids, like tears and spit (saliva) It usually starts in people aged 40 to 60 and is much more common in women than men It's a long-term condition that can affect your daily life, but there are treatments to help relieve the symptoms
29
What is the epidemiology of Sjorgens syndrome?
Prevalence: 1 in 100 Incidence: 4 in 100,000 Onset: 40-50yrs Female : Male 9:1
30
what are the symptoms and sigsn of Sjogrens syndrome?
Dry eyes and mouth Anti Ro (SSA), anti La (SSB) antibodies Salivary gland biopsy Parotid gland enlargement 1/3 have systemic upset - fatigue, fever, myalgia, arthralgia, dry skin
31
What are some complications of Sjogrens syndrome?
Lymphoma Neuropathy Cutaneous vasculitis Interstitial lung disease Renal tubular acidosis
32
what is Auto-immune myositis?
Myositis is a rare type of autoimmune disease that inflames and weakens muscle fibers Autoimmune myositis causes inflammation and weakness in the muscles (polymyositis) or in the skin and muscles (dermatomyositis)
33
what is the epidemiology of Polymyositis and dermatomyositis?
Rare: 6/million incidence More common in females (ratio 3:1) Peak incidence is in 50 – 60 year olds Increased risk of malignancy
34
what does myositis cause and how is it investigated?
Muscle weakness - symmetrical, proximal Raised CK level EMG, MRI, muscle biopsy Interstitial lung disease – anti Jo1 antibodies Cutaneous - Gottron’s papules (80%), Heliotrope rash (30-60%)
35
what is an overlap syndrome?
Mixed Connective Tissue Disease
36
what is mixed conncetive tissue disease?
Raynaud’s Soft tissue swelling/sclerodactyly Myositis Arthralgia Mixed connective tissue disease is a term used by some doctors to describe a disorder characterized by features of systemic lupus erythematosus, systemic sclerosis, and polymyositis. Raynaud syndrome, joint pains, various skin abnormalities, muscle weakness, and problems with internal organs can develop
37
The vasculitides - what are they and how are they classified?
The term vasculitides refers to heterogeneous disorders characterized by vascular inflammation affecting vessels of different sizes from large arteries to capillaries or tiny venules. Vasculitis leads to diminished blood flow or vessel occlusion resulting in ischemia, necrosis, and subsequent tissue damage Inflammation of the blood vessels Classified depending on size of vessel involved
38
what are different exmaples of the vasculitides?
39
what is Giant cell arteritis?
Most common type of vasculitis Mainly effects white individuals Incidence increases with age More common in women Temporal arteritis is a serious condition where the arteries at the side of your head (the temples) become inflamed Almost all patients who develop giant cell arteritis are over the age of 50. GCA commonly causes headaches, joint pain, facial pain, fever, and difficulties with vision, and sometimes permanent visual loss in one or both eyes
40
what is the GCA classification criteria?
3 of the following (classic temporal arteritis): Age at onset ≥50 years New headache Temporal artery tenderness/reduced pulsation ESR≥50 Abnormal temporal biopsy
41
what is the gold standard investigaitong for GCA?
Temporal artery biopsy Shows inflammatory infiltrate in the artery wall Intimal hyperplasia Luminal exclusion Can also do Ultrasound doppler
42
what is shown here?
CT angiogram, MR angiogram CT angiogram and MR angiogram good to investigate large vessel involvement
43
what is FDG PET good for diagnosing?
inflammation of the aorta
44
what are the complciations of GCA?
Irreversible visual loss Aortic aneurysms Arterial stenosis and limb ischaemia Stroke
45
what is the treatment and management of GCA?
Urgent initiation of high dose Prednisolone 40-60mg per day, gradually tapered PPI Bone protection Steroid sparing medication
46
what is ANCA associated vasculitis?
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of diseases (granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis), characterized by destruction and inflammation of small vessels Granulomatosis with polyangiitis (Wegener’s) Microscopic polyangiitis Eosiniphilic granulomatosis with polyangiitis Overall incidence 15/million Overall prevalence 150/million
47
what is Granulomatosis with Polyangiitis (Wegener’s) (GPA) and what does it cause?
Necrotising granulomatous inflammation Usually involving the upper and lower respiratory tract Hearing loss, sinusitis, hemoptysis Necrotising glomerulonephritis is common cANCA, anti PR3 antibodies Collapse of nasal bridge Can get investigated for cancer but biopsy shows it is this
48
what is Microscopic polyangiitis (MPA) and what does it cause?
Necrotising vasculitis, with few or no immune deposits, predominantly affecting small vessels Granulomatous inflammation is absent Renal and pulmonary involvement common pANCA, anti MPO antibodies
49
what is Eosinophilic Granulomatosis with Polyangiitis (Churg Strauss) (EGPA) and what does it cause?
Eosinophil rich and necrotising granulomatous inflammation often involving the respiratory tract Late onset asthma, nasal polyps and eosinophilia Necrotising vasculitis predominantly affecting small to medium vessels Neurological involvement Cardiac, gastrointestinal – poor prognosis 40-60% anti MPO antibodies positive
50
Case scenario: 45 year old female patient 3-day history of breathlessness and right sided chest pain worse with deep inspiration CXR confirms right pleural effusion Treatment with antibiotics makes no difference FBC showed persistently low WCC of 3.0 then 3.2 and low platelets of 100 Over the last year she has been experiencing intermittent pain and swelling in her joints and recurrent facial rash after sun exposure What is the likely diagnosis: A. Scleroderma B. SLE C. Giant Cell Arteritis D. None of the above
Lupus in a women with evidence of cytopenia, arthritis, photosensitivity, pleuritic chest pain So it is B
51
What bedside assessment would you prioritise? A. Blood pressure B. Fundoscopy C. Auscultation for carotid bruits D. Urine dipstick
D – make sure they don’t have any renal involvement
52
(3) Which of the following immunological tests would support your clinical suspicion A. Positive anti dsDNA antibodies B. Reduced complement levels C. Positive anti Smith antibodies D. Positive anti-nuclear antibodies
All answers are true here

Year 3 - MSK (45 decks)

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