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Year 3 > Vasculitides and autoimmune conditions > Flashcards

Vasculitides and autoimmune conditions Flashcards

1
Q

What is sarcoidosis?

A

Multisystem granulomatous inflammatory disorder characterised by the presence of non-caseating granulomas

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2
Q

What is the epidemiology of sarcoidosis?

A

Afro-Caribbeans and Scandinavians

>50yrs

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3
Q

What are the clinical features of sarcoidosis by body system?

A

General: fever, malaise, weight loss (FLAWs)

Pulmonary: SOB, dry cough

Musc: arthralgia

Eyes: uveitis, keratoconjunctivitis

Skin: LUPUS PERNIO, erythema nodosum

Cardio: arrhythmia, heart failure

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4
Q

What are blood investigations would you do for sarcoidosis? What would you see?

A
  • ↑ Ca (activated macrophages increase conversion of 1,25-dihydoxycholecalciferol –> calcitriol)
  • ↑ACE (produced by recently activated macrophages in granulomas)
  • ↑ ESR
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5
Q

What is the management for sarcoidosis?

A

Steroids
NSAIDs
Steroid-sparing agents

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6
Q

What is systemic lupus erythematosus?

A

A chronic multi-system autoimmune disorder

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7
Q

What is the epidemiology for SLE?

A

Young (20-40)
Afro-Caribbean
Female

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8
Q

What are the symptoms of SLE?

SOAP BRAIN MD

A

Serositis
Oral ulcers
Arthritis
Photosensitivity

Bloods (low)
Renal (proteinuria, casts)
ANA
Immunological (Anti-dsDNA)
Neurological (psychosis, seizures)

Malar rash
Discoid rash

also: FLAWS, lymphadenopathy, raynauds, livedo reticularis, alopecia

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9
Q

What are the investigations for SLE?

A

Bloods: ESR, U+E, FBC
Autoantibodies: anti-dsDNA, ANA, anti-cardiolipin
Urinalysis: casts, proteinuria, haematuria
CXR
Joint X-ray

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10
Q

What other condition can arise from SLE?

A

Anti-phospholipid syndrome

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11
Q

What is anti-phospholipid syndrome? Which antibody is present?

A

Triad of:

  • thromboembolism
  • recurrent miscarriage
  • thrombocytopenia

Presence of ANTI-CARDIOLIPIN antibody

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12
Q

Define systemic sclerosis

A

A rare connective tissue disorder characterised by widespread small blood vessel damage + fibrosis in skin (scleroderma) and internal organs

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13
Q

What are the 4 types of systemic sclerosis? Which antibodies are implemented in each?

A
  • DIFFUSE CUTANEOUS- anti-topoisomerase II (anti-Scl-70) antibodies
  • LIMITED CUTANEOUS (CREST)- anticentromere
  • PRESCLERODERMA- ANA antibodies
  • SCLERODERMA SINE SCLERODERMA- ACA antibodies
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14
Q

What are the characteristics of diffuse cutaneous systemic sclerosis?

A
  • Skin changes involving the trunk AND limbs
  • Raynaud’s phenomenon
  • Arthralgia/arthritis
  • TENDON FRICTION RUBS

Anti-topoisomerase II antibody

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15
Q

What are the characteristics of limited cutaneous systemic sclerosis?

A 
CREST:
Calcinosis
Raynaud's phenomenon
oEsophageal dysmobility
Sclerodactyly
Telangectasia

Anti-centromere antibody

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16
Q

What is vasculitis?

A

Inflammation of the blood vessel walls, with often an unknown aetiology, systemic effects, and distinctive features.

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17
Q

What is giant cell arteritis?

A

Granulomatous inflammation of the large arteries affecting the external carotid artery, most commonly temporal artery.

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18
Q

What are the symptoms of GCA?

A 
Unilateral headache
Jaw claudication
Scalp tenderness
Visual disturbances + vision loss
Systemic upset (fever, malaise, weight loss)
Symptoms of PMR
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19
Q

What are the investigations/diagnostic criteria for GCA?

A

> 3 OF THE FOLLOWING:

  • Age of onset >50years
  • New headache
  • ↑ESR >50mm/hr
  • Temporal artery abnormality (tender, non-pulsatile)
  • Positive artery biopsy- see multinucleated giant cells (NOT sensitive- skip lesions)
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20
Q

What is the management for GCA?

A

HIGH DOSE PREDNISOLONE (straight after ESR)

Start therapy if high suspicion even if unconfirmed.

  • prevent irreversible visual loss.
  • taper the dose as ESR normalises

Once GCA confirmed:

  • aspirin to reduce risk of stroke + vision loss
  • PPI, bisphosphonates- reduce osteoporosis risk from chronic glucocorticoid use.
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21
Q

What is polymyalgia rheumatica?

A

Inflammatory rheumatological condition of unknown cause characterised by bilateral hip and shoulder girdle pain and morning stiffness,
NO weakness.

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22
Q

RF/aetiology of PMR

A

> 50
Females
15% develop GCA

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23
Q

How does PMR present?

A

Bilateral hip/shoulder girdle pain
Morning stiffness
Subacute onset (>2 weeks)
Systemic (FLAWS)

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24
Q

What is polyarteritis nodosa?

A

A vasculitis affecting medium-sized vessels (arterioles, capillaries, or venules)

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25
Q

What is the aetiology of PAN?

A

Idiopathic

Associated with HBV

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26
Q

What are the clinical features of Polyarteritis Nodosa (PAN) by organ system?

A

Necrotizing inflammation of the vessels within organs –> ischaemia and infarction:

  • Kidneys: haematuria, renal failure, HTN
  • Abdo: mesenteric ischaemia –> abdo pain + PR bleeding
  • Skin: livedo reticularis
  • Nerves: peripheral neuropathy
  • Arthralgia
  • Systemic (FLAWS)
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27
Q

What can be seen in a renal angiogram in PAN?

A

Rosary sign

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28
Q

What is granulomatosis with polyangiitis?

A

aka Wegener’s

Triad of:

  • URT (rhinitis, epistaxis)
  • LRT (haemoptysis, heamorrhage)
  • kidneys (glomerulonephritis)

Saddle nose

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29
Q

What are the investigations for GPA/Wegeners?

A

Serum cANCA

CXR- cavitating lesions

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30
Q

What is eosinophilic granulomatosis with polyangiitis?

A

Tri-phasic:
-allergic: asthma/rhinitis
-eosinophilic: eosinophils damage the lungs/gut
-vasculitic: can lead to widespread vessel damage and death
Other features: haemoptysis, rash, focal neuropathy, kidney damage

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31
Q

What are the investigations for eGPA?

A

pANCA

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32
Q

What is Behcet’s disease?

A

Triad of oral ulcers, genital ulcers, and uveitis

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33
Q

What is the background of Behcet’s disease?

A

Common in the Mediterranean (Greasy Turkeys)

Associated with HLA-B51

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34
Q

What are the clinical features of Behcet’s disease?

A 
Triad of:
-recurrent oral ulcers
-genital ulcers
-uveitis
Also colitis, rash, arthritis, pericarditis (CRAP)
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35
Q

What are the investigations for Behcet’s disease?

A

Clinical diagnosis

Can confirm with Pathery test

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36
Q

What is the Pathergy test?

A

Needle skin prick forms a sterile pustule within 48h

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37
Q

What is Takuyasu’s arteritis?

A

A large vessel vasculitis causing occlusion of the aorta and its main branches

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38
Q

RF for Takuyasu’s arteritis

A

ASIAN
20-40yrs
Females

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39
Q

What are the symptoms of Takuyasu’s?

A

Presentation depends on which branches of the aorta are stenosed/ occluded

SUBCLAVIAN

  • Limb claudication = pain on exertion- sign of limb ischaemia
  • absent pulse
  • bruits
  • unequal BP (>10mmHg difference between the 2 arms)

CAROTIDS

  • Headache
  • TIA/Stroke
  • Carotid bruits

Aortic root dilation –> AR murmur

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40
Q

What is the background of Henoch-Schönlein purpura?

A

Affects 3-15 yr olds

IgA vasculitis

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41
Q

What are the symptoms of Henoch-Schönlein purpura?

A

Triad of:

  • purpuric rash on buttocks and extensors of lower limbs
  • abdo pain
  • arthralgia
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42
Q

What is the background of Goodpasture’s syndrome?

A

Anti-GBM antibodies

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43
Q

What are the symptoms of Goodpasture’s syndrome?

A

Triad of:

  • glomerulonephritis
  • haemoptysis (pulmonary haemorrhage)

Anti-GBM antibodies

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44
Q

A 40-year-old Afro-Caribbean man has suffered from shortness of breath and a dry cough for the last 3 months. He also complains of some ‘sore lumps on his shins’. Closer inspection reveals tender violet nodules on both shins. A chest X-ray is requested, which shows bilateral hilar lymphadenopathy. Blood tests are also requested, including U&Es – which parameter would you expect to be raised?

A. Sodium
B. Potassium 
C. Calcium
D. pH
E. Urea
A

C. Calcium

45
Q

A 33-year-old female with SLE presents to the fertility clinic complaining that she has been desperately trying to start a family but has had repeated miscarriages. She has had 3 miscarriages in the past 5 years. She has a past medical history of asthma and two DVTs. Given the likely diagnosis, which of the following antibodies is associated with this disease?

A.  Anti-CCP antibody
B.  Anti-Jo-1 antibody
C.  Anti-centromere antibody
D.  Anti-cardiolipin antibody
E.  Anti-smooth muscle antibody
A

D. Anti-cardiolipin antibody

46
Q

A 58-year-old woman presents to her GP complaining of difficulty swallowing which started 6 months ago. On examination, the skin on her hands appears thickened and tight around her fingers. Furthermore, a hard lump is felt under the skin on her left thumb. Which of the following additional clinical features would support a diagnosis of limited cutaneous systemic sclerosis?

A.  Telangiectasia
B.  Thickened skin on the chest 
C.  Macroglossia
D.  Buccal pigmentation 
E.  Cherry haemangioma
A

A. Telangiectasia

47
Q

A 63-year-old woman presents to A&E with a headache, mainly affecting the left half of her forehead, that has gradually been getting worse over the past week. She has been eating less as she experiences pain in her jaw when she chews her food. She has, generally speaking, been healthy aside from experiencing some stiffness and pain in her shoulders over the past 6 months. What is the first step in her management?

A.Check ESR 
B.Temporal artery biopsy 
C.IV hydrocortisone 
D. Oral prednisolone 
E.IV antibiotics
A

A.Check ESR

48
Q

Which of the following conditions is strongly associated with temporal arteritis?

A. Takayasu’s aortitis
B. Myalgic encephalomyelitis
C. Fibromyalgia
D. Polymyalgia rheumatica
E. Polymyositis
A

D. Polymyalgia rheumatica

49
Q

A 47-year-old man visits his GP having developed a skin rash. He has been feeling ‘generally unwell’ for the past 3 months, and has suffered from abdominal pain accompanied by some rectal bleeding. An angiogram reveals ‘Rosary sign’. He regularly attends hospital for check-ups since he was diagnosed with chronic hepatitis B, 2 years ago. What is the most likely diagnosis?

A. Giant cell arteritis 
B. Dermatomyositis 
C. Polyarteritis nodosa 
D. Granulomatosis with polyangiitis 
E. Behcet’s disease
A

C. Polyarteritis nodosa

50
Q

A 52-year-old man has suffered from rhinitis and recurrent nosebleeds for the past 5 months. Initially, he did not think much of it, until he began coughing up a small about of blood about 3 weeks ago. A urine dipstick reveals proteinuria and haematuria. Blood tests reveal:
ESR: 72 mm/hr (< 20 mm/hr)
cANCA: positive
What is the most likely diagnosis?

A.Microscopic polyangiitis 
B.Goodpasture’s syndrome 
C.Granulomatosis with polyangiitis 
D.Churg-Strauss syndrome 
E. Behçet’s disease
A

C.Granulomatosis with polyangiitis

51
Q

A 45-year-old man from Cyprus presents with recurrent ulcers on his penis. He has not noticed any discharge from his penis or pain whilst urinating. He adds that he has also developed mouth ulcers several times over the past year. During the consultation, you notice that his eyes are quite red. When questioned, he says that his eyes have been itchy recently, and thinks that he might have hay fever. What is the most likely diagnosis?

A.Inflammatory bowel disease 
B.Behçet’s disease 
C.Herpes simplex virus 
D.Syphilis 
E.Reactive arthritis
A

B.Behçet’s disease

52
Q

Churg-Strauss syndrome is associated with:

A. pANCA
B. cANCA
C. Anti-GBM antibodies
D. Anti-LKM antibodies
E. Anti-smooth muscle antibodies
53
Q

A 43-year-old gardener is brought into A&E after having a seizure. His seizure lasts about 2 mins and disappears by itself. He says that this has never happened before and his only health problem has been a persistent headache that has developed over the past 6 months. On examination, a single heavily pigmented skin lesion is noticed on his right forearm. An MRI head scan reveals several lesions in both hemispheres.
What is the most likely diagnosis?

A. Neurofibromatosis Type 1 
B. Meningioma
C. Tuberous sclerosis 
D. Metastases 
E. Glioblastoma multiforme
A

D. Metastases

New onset seizure, chronic persistent headache
Indicates SOL
Heavily pigmented skin lesion indicates malignant melanoma
Several lesions in both hemispheres leads to a suspicion of metastases

54
Q

Which autoantibodies may be present in SLE?

A
  • ANA: >95% are ANA +ve
  • Anti-dsDNA is more specific
  • Anti-cardiolipin –> anti-phospholipid syndrome
55
Q

Pathophysiology and associations of SLE

A

Tissue damage caused by immune complex deposition in organs (skin, joints, kidneys, brain)

HLA B8, DR2 and DR3 associated

Associated with other auto-immune conditions (sjorens, AI thyroid conditions)

56
Q

State a specific complication of SLE

A

Antiphospholipid syndrome

Characterised by triad of:

  • Thromboembolism
  • Recurrent miscarriage
  • Thrombocytopaenia
57
Q

Antiphospholipid syndrome is positive for which antibody?

A

anti-cardiolipin

58
Q

Define Sjogren’s syndrome

A

A syndrome characterised by autoimmune destruction of the exocrine glands

59
Q

Epidemiology sjogrens

A

Females

Associated with other AI conditions (secondary sjogrens)

60
Q

How does Sjogren’s present?

A

DRY EYES –> keratoconjunctivitis sicca  corneal ulceration
DRY MOUTH –> Recurrent oral infections
recurrent episodes of parotitis

  • vaginal dryness –> dyspareunia
  • Raynaud’s, myalgia, arthralgia
  • Vasculitis - sensory polyneuropathy
  • Kidney disease - renal tubular acidosis (usually subclinical)
61
Q

What are the investigations for Sjoren’s?

A

SCHIRMER’S TEST- filter paper near conjunctival sac to measure tear formation

Antibodies- ANA, anti-Ro, anti-La, RF

Biopsy = focal lymphocytic infiltration

FBC- may show low CD4

62
Q

A 31-year-old lady presented with. She is generally healthy and plays tennis daily but over the past couple of months she has felt increasingly tired with muscle aches. On further questions she reports having a dry mouth with a gravelly sensation in her eyes.

Which of the following would be the most appropriate investigation?
A. Muscle biopsy
B. Schirmer’s test
C. Schober’s test
D. Check for anti-topoisomerase antibodies
E. Check for anti-dsDNA

A

SCHIRMER’S TEST

Schober’s test= Ankylosing spondylitis
Anti-dsDNA = SLE
Anti-topoisomerase= diffuse cutaneous SS

63
Q

Which antibodies may be present in Sjoren’s?

A

Rheumatoid factor(RF) - 50% PTs
ANA - 70% PTs

anti-Ro(SSA) antibodies = 70% of patients with PSS
anti-La(SSB) antibodies = 30% of patients with PSS

64
Q

Define polymyositis/dermatomyositis

A

Insidious onset, symmetrical proximal muscle weakness + striated muscle inflammation

65
Q

How does polymyositis present?

A

Gradual onset (weeks/ months)
Diffuse proximal muscle weakness
Resp weakness
Dysphagia, dysphonia

66
Q

How does dermatomyositis present?

A 
Gradual onset (weeks/ months)
Proximal muscle weakness

RASH:

  • macular
  • heliotrope
  • GOTTRON’S PAPULES
67
Q

compare presentations dermatomyositis and polymyositis

A

SAME:

  • Gradual onset (weeks/ months)
  • Proximal muscle weakness

DIFFERENT:

  • dermatomyositis = rash
  • polymyositis = resp weakness, dysphagia/phonia
68
Q

Which organs are most affected by poly/dermatomyositis?

A

GI
lung
cardiac disease

69
Q

Which autoantibodies are present in dermatomyositis and polymyositis?

A

Polymyositis: Anti-Jo
Dermatomyositis: ANA, Anti-Mi-2, anti-Jo

70
Q

Which 3 rashes are present in dermatomyositis?

A

Macular rash= shawl sign
Gottron’s papules= rough red papules over knuckles and elbows
Heliotrope rash= lilac rash of eyelids + oedema

71
Q

What investigations would you do for dermatomyositis/polymyositis?

A
  • CK- most sensitive muscle enzyme test, can be elevated up to 50fold (cereal to monitor)
  • Muscle biopsy- DIAGNOSTIC inflammation, atrophy
  • EMG
  • Autoantibodies- ANTI-JO
72
Q

What non-blood investigations would you do for sarcoidosis, what would you see?

A

CXR:

  • bilateral hilar lymphadenopathy
  • pulmonary infiltrates/fibrosis

BIOPSY
- non-caseating granuloma

73
Q

lupus pernio is associated with which condition?

A

sarcoidosis

74
Q

A 56-year-old woman presents with cold, pale fingers and difficulty swallowing. On examination she has puffy, swollen, tight fingers.

Given the likely diagnosis, which of the following autoantibodies is associated with this condition?
A. Anti-CCP antibodies
B. Anti-topoisomerase antibodies
C. Anti-nuclear antibodies
D. Anti-cardiolipin
E. Anti-centromere antibodies
A

CREST syndrome = anti-centromere

limited cutaneous systemic sclerosis (lcSSc)

75
Q

anti-CCP is associated with which condition?

A

Rheumatoid arthritis

76
Q

anti-topoisomerase is associated with which condition?

A

Diffuse systemic sclerosis

77
Q

anti-cardiolipin is associated with which condition?

A

Antiphospholipid syndrome

  • thromboembolism
  • recurrent miscarriage
  • thrombocytopenia
78
Q

What are the RF for GCA?

A

> 50-years-old
Female
Associated with Polymyalgia Rheumatica

79
Q

What investigations would you do for PMR? What would you see?

A

Diagnosis is made via history and with supportive laboratory tests

Bloods:

  • ↑ESR/ CRP
  • normal CK

Other:

  • USS shows bursitis/effusion in joint
  • rapid improvement with low dose prednisolone
80
Q

Management of PMR

A

Low dose prednisolone (2-4 weeks till ESR normalises)

Osteoporosis prophylaxis:

  • calcium (calcium carbonate)
  • vit D (cholecalciferol)
  • bisphosphonates (alendronic acid)
81
Q

Ix for Takayasu’s Arteritis

A

↑ESR/CRP

Angiography (CTA or MRA)

82
Q

What are the 2 main large vessel vasculitides?

A

Temporal arteritis

Takayasu’s arteritis

83
Q

What are the 2 main medium vessel vasculitides?

A

Polyarteritis nodosa

Kawasaki disease

84
Q

What dermatological condition may be indicative of polyarteritis nodosa?

A

livedo reticularis

= mottled net-like appearance of the skin caused by spasms of the blood vessels

85
Q

What investigations would you do for polyarteritis nodosa?

A
  • BLOODS: FBC- high WCC, anaemia, HBV, deranged U+Es/LFTs
  • BIOPSY–> arteritis
  • ARTERIOGRAM (CTA or MRA) –> ROSARY SIGN
86
Q

What would you see on CTA/MRA in polyarteritis nodosa?

A

Rosary sign

bead-like appearance of blood vessels due to multiple micro-aneurysms

87
Q

What are the 3 categories of small vessel vasculitides?

A
  1. ANCA POSITIVE
    - Churg Strauss
    - Wegener’s
    - MPA
  2. IMMUNE COMPLEX
  3. ANCA NEGATIVE
    - HPA
    - Goodpasture’s
88
Q

How does Granulomatosis with polyangitis (Wegener’s Granulomatosis) classically present?

A

triad of:

URT:

  • Rhinitis
  • Epistaxis

LRT:

  • Haemoptysis
  • SOB

RENAL

  • Haematuria
  • Dysuria
89
Q

What is granulomatosis with polyangiitis (Wegener’s Granulomatosis) classically present?

A

An autoimmune necrotising granulomatous vasculitis characterised by a triad of organ involvement
(URT, LRT, renal)

90
Q

Ix for granulomatosis with polyangiitis (Wegener’s Granulomatosis)- what would you see?

A

cANCA
Urinalysis: RBCs casts
CXR- cavitating lesions

91
Q

What is Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome)?

A

Systemic autoimmune vasculitis causing blood vessel inflammation, characterised by asthma and eosinophilia

92
Q

How does Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome) present?

A

Tri-phasic presentation:

  1. ALLERGIC
    - asthma
    - rhinitis
  2. EOSINOPHILIC
    - tissue damage in lungs/GIT
    - Lung damage –> haemoptysis
  3. VASCULITIC
    - widespread organ damage
    - Systemic vasculitis –> peripheral neuropathies + purpura/ petechiae
93
Q

What is Microscopic Polyangitis?

A

small-vessel ANCA-associated vasculitis

94
Q

How does microscopic polyangitis present?

A

Similar to wegeners but without the URT involvement

95
Q

Ix for microscopic polyangitis + results

A
  • Bloods: pANCA, U+Es, ↑Cr
  • Urinalysis: haematuria, proteinuria
  • CXR: cavitating lesions
  • Renal Biopsy (glomerulonephritis)
96
Q

A 63-year-old lady presents with a 3-month history of rhinitis and recurrent nosebleeds. She has recently started to cough up blood. She reports having less energy and has lost over 5kg over the past 2 months.
Urinalysis: proteinuria and haematuria
Bloods: ↑ESR and cANCA +ve

What is the most likely diagnosis?
Goodpasture’s syndrome
Granulomatosis with polyangiitis
Eosinophilic granulomatosis with polyangiitis
Microscopic polyangiitis
SLE
A

Granulomatosis with polyangiitis (Wegener’s)

Rhinitis + nosebleeds = URT
Haemoptysis = LRT
Proteinuia + haemature = Renal
cANCA

97
Q

What is Bechet’s disease?

A

Multisystem disorder causing vasculitis of small, medium and large sized vessels

98
Q

Epidemiology Bechet’s

A

Eastern Mediterranean
Men
20-40yrs

99
Q

How does Bechet’s present?

A

Can’t see, can’t pee, can’t eat spicy

  • Anterior uveitis
  • Genital Ulcers
  • Oral Ulcers

also:
Thrombophlebitis + DVT, arthritis, erythema Nodosum

100
Q

genetic aetiology Bechet’s

A

Associated with HLA B51 (strong genetic predisposition)

101
Q

BUZZWORD: Rosary sign, Hep B infection

A

Polyarteritis nodosa

102
Q

BUZZWORD: Uveitis, oral and genital ulcers

A

Bechet’s Syndrome

103
Q

BUZZWORD: pANCA, eosinophilia, asthma

A

Churg-strauss syndrome

104
Q

BUZZWORD: Bilateral hip and shoulder girdle pain

A

Polymyalgia rheumatica

105
Q

BUZZWORD: Asian females

A

Takayasu’s arteritis

106
Q

BUZZWORD: pANCA, glomerulonephritis

A

Microscopic Polyangitis

107
Q

BUZZWORD: Scalp tenderness, headache

108
Q

BUZZWORD: cANCA, URT, LRT, GN

A

Wegener’s disease

Year 3 (38 decks)

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