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Vasculitides and autoimmune conditions Flashcards

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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
What is the aetiology of PAN?
Idiopathic | Associated with HBV
26
What are the clinical features of Polyarteritis Nodosa (PAN) by organ system?
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)
27
What can be seen in a renal angiogram in PAN?
Rosary sign
28
What is granulomatosis with polyangiitis?
aka Wegener's Triad of: - URT (rhinitis, epistaxis) - LRT (haemoptysis, heamorrhage) - kidneys (glomerulonephritis) Saddle nose
29
What are the investigations for GPA/Wegeners?
Serum cANCA | CXR- cavitating lesions
30
What is eosinophilic granulomatosis with polyangiitis?
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
31
What are the investigations for eGPA?
pANCA
32
What is Behcet's disease?
Triad of oral ulcers, genital ulcers, and uveitis
33
What is the background of Behcet's disease?
Common in the Mediterranean (Greasy Turkeys) | Associated with HLA-B51
34
What are the clinical features of Behcet's disease?
``` Triad of: -recurrent oral ulcers -genital ulcers -uveitis Also colitis, rash, arthritis, pericarditis (CRAP) ```
35
What are the investigations for Behcet's disease?
Clinical diagnosis | Can confirm with Pathery test
36
What is the Pathergy test?
Needle skin prick forms a sterile pustule within 48h
37
What is Takuyasu's arteritis?
A large vessel vasculitis causing occlusion of the aorta and its main branches
38
RF for Takuyasu's arteritis
ASIAN 20-40yrs Females
39
What are the symptoms of Takuyasu's?
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
40
What is the background of Henoch-Schönlein purpura?
Affects 3-15 yr olds | IgA vasculitis
41
What are the symptoms of Henoch-Schönlein purpura?
Triad of: - purpuric rash on buttocks and extensors of lower limbs - abdo pain - arthralgia
42
What is the background of Goodpasture's syndrome?
Anti-GBM antibodies
43
What are the symptoms of Goodpasture's syndrome?
Triad of: - glomerulonephritis - haemoptysis (pulmonary haemorrhage) Anti-GBM antibodies
44
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 ```
C. Calcium
45
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 ```
D. Anti-cardiolipin antibody
46
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. Telangiectasia
47
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. Check ESR
48
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 ```
D. Polymyalgia rheumatica
49
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 ```
C. Polyarteritis nodosa
50
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 ```
C. Granulomatosis with polyangiitis
51
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 ```
B. Behçet’s disease
52
Churg-Strauss syndrome is associated with: ``` A. pANCA B. cANCA C. Anti-GBM antibodies D. Anti-LKM antibodies E. Anti-smooth muscle antibodies ```
A. pANCA
53
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 ```
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
Which autoantibodies may be present in SLE?
- ANA: >95% are ANA +ve - Anti-dsDNA is more specific - Anti-cardiolipin --> anti-phospholipid syndrome
55
Pathophysiology and associations of SLE
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
State a specific complication of SLE
Antiphospholipid syndrome Characterised by triad of: - Thromboembolism - Recurrent miscarriage - Thrombocytopaenia
57
Antiphospholipid syndrome is positive for which antibody?
anti-cardiolipin
58
Define Sjogren's syndrome
A syndrome characterised by autoimmune destruction of the exocrine glands
59
Epidemiology sjogrens
Females | Associated with other AI conditions (secondary sjogrens)
60
How does Sjogren's present?
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
What are the investigations for Sjoren's?
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
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
SCHIRMER'S TEST Schober’s test= Ankylosing spondylitis Anti-dsDNA = SLE Anti-topoisomerase= diffuse cutaneous SS
63
Which antibodies may be present in Sjoren's?
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
Define polymyositis/dermatomyositis
Insidious onset, symmetrical proximal muscle weakness + striated muscle inflammation
65
How does polymyositis present?
Gradual onset (weeks/ months) Diffuse proximal muscle weakness Resp weakness Dysphagia, dysphonia
66
How does dermatomyositis present?
``` Gradual onset (weeks/ months) Proximal muscle weakness ``` RASH: - macular - heliotrope - GOTTRON'S PAPULES
67
compare presentations dermatomyositis and polymyositis
SAME: - Gradual onset (weeks/ months) - Proximal muscle weakness DIFFERENT: - dermatomyositis = rash - polymyositis = resp weakness, dysphagia/phonia
68
Which organs are most affected by poly/dermatomyositis?
GI lung cardiac disease
69
Which autoantibodies are present in dermatomyositis and polymyositis?
Polymyositis: Anti-Jo Dermatomyositis: ANA, Anti-Mi-2, anti-Jo
70
Which 3 rashes are present in dermatomyositis?
Macular rash= shawl sign Gottron’s papules= rough red papules over knuckles and elbows Heliotrope rash= lilac rash of eyelids + oedema
71
What investigations would you do for dermatomyositis/polymyositis?
- 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
What non-blood investigations would you do for sarcoidosis, what would you see?
CXR: - bilateral hilar lymphadenopathy - pulmonary infiltrates/fibrosis BIOPSY - non-caseating granuloma
73
lupus pernio is associated with which condition?
sarcoidosis
74
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 ```
CREST syndrome = anti-centromere limited cutaneous systemic sclerosis (lcSSc)
75
anti-CCP is associated with which condition?
Rheumatoid arthritis
76
anti-topoisomerase is associated with which condition?
Diffuse systemic sclerosis
77
anti-cardiolipin is associated with which condition?
Antiphospholipid syndrome - thromboembolism - recurrent miscarriage - thrombocytopenia
78
What are the RF for GCA?
>50-years-old Female Associated with Polymyalgia Rheumatica
79
What investigations would you do for PMR? What would you see?
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
Management of PMR
Low dose prednisolone (2-4 weeks till ESR normalises) Osteoporosis prophylaxis: - calcium (calcium carbonate) - vit D (cholecalciferol) - bisphosphonates (alendronic acid)
81
Ix for Takayasu’s Arteritis
↑ESR/CRP | Angiography (CTA or MRA)
82
What are the 2 main large vessel vasculitides?
Temporal arteritis | Takayasu’s arteritis
83
What are the 2 main medium vessel vasculitides?
Polyarteritis nodosa | Kawasaki disease
84
What dermatological condition may be indicative of polyarteritis nodosa?
livedo reticularis | = mottled net-like appearance of the skin caused by spasms of the blood vessels
85
What investigations would you do for polyarteritis nodosa?
- BLOODS: FBC- high WCC, anaemia, HBV, deranged U+Es/LFTs - BIOPSY--> arteritis - ARTERIOGRAM (CTA or MRA) --> ROSARY SIGN
86
What would you see on CTA/MRA in polyarteritis nodosa?
Rosary sign | bead-like appearance of blood vessels due to multiple micro-aneurysms
87
What are the 3 categories of small vessel vasculitides?
1. ANCA POSITIVE - Churg Strauss - Wegener’s - MPA 2. IMMUNE COMPLEX 3. ANCA NEGATIVE - HPA - Goodpasture’s
88
How does Granulomatosis with polyangitis (Wegener’s Granulomatosis) classically present?
triad of: URT: - Rhinitis - Epistaxis LRT: - Haemoptysis - SOB RENAL - Haematuria - Dysuria
89
What is granulomatosis with polyangiitis (Wegener’s Granulomatosis) classically present?
An autoimmune necrotising granulomatous vasculitis characterised by a triad of organ involvement (URT, LRT, renal)
90
Ix for granulomatosis with polyangiitis (Wegener’s Granulomatosis)- what would you see?
cANCA Urinalysis: RBCs casts CXR- cavitating lesions
91
What is Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome)?
Systemic autoimmune vasculitis causing blood vessel inflammation, characterised by asthma and eosinophilia
92
How does Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome) present?
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
What is Microscopic Polyangitis?
small-vessel ANCA-associated vasculitis
94
How does microscopic polyangitis present?
Similar to wegeners but without the URT involvement
95
Ix for microscopic polyangitis + results
- Bloods: pANCA, U+Es, ↑Cr - Urinalysis: haematuria, proteinuria - CXR: cavitating lesions - Renal Biopsy (glomerulonephritis)
96
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 ```
Granulomatosis with polyangiitis (Wegener's) Rhinitis + nosebleeds = URT Haemoptysis = LRT Proteinuia + haemature = Renal cANCA
97
What is Bechet's disease?
Multisystem disorder causing vasculitis of small, medium and large sized vessels
98
Epidemiology Bechet's
Eastern Mediterranean Men 20-40yrs
99
How does Bechet's present?
Can’t see, can’t pee, can’t eat spicy - Anterior uveitis - Genital Ulcers - Oral Ulcers also: Thrombophlebitis + DVT, arthritis, erythema Nodosum
100
genetic aetiology Bechet's
Associated with HLA B51 (strong genetic predisposition)
101
BUZZWORD: Rosary sign, Hep B infection
Polyarteritis nodosa
102
BUZZWORD: Uveitis, oral and genital ulcers
Bechet's Syndrome
103
BUZZWORD: pANCA, eosinophilia, asthma
Churg-strauss syndrome
104
BUZZWORD: Bilateral hip and shoulder girdle pain
Polymyalgia rheumatica
105
BUZZWORD: Asian females
Takayasu’s arteritis
106
BUZZWORD: pANCA, glomerulonephritis
Microscopic Polyangitis
107
BUZZWORD: Scalp tenderness, headache
GCA
108
BUZZWORD: cANCA, URT, LRT, GN
Wegener’s disease

Year 3 (38 decks)

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