Vasculitides and autoimmune conditions Flashcards
What is sarcoidosis?
Multisystem granulomatous inflammatory disorder characterised by the presence of non-caseating granulomas
What is the epidemiology of sarcoidosis?
Afro-Caribbeans and Scandinavians
>50yrs
What are the clinical features of sarcoidosis by body system?
General: fever, malaise, weight loss (FLAWs)
Pulmonary: SOB, dry cough
Musc: arthralgia
Eyes: uveitis, keratoconjunctivitis
Skin: LUPUS PERNIO, erythema nodosum
Cardio: arrhythmia, heart failure
What are blood investigations would you do for sarcoidosis? What would you see?
- ↑ Ca (activated macrophages increase conversion of 1,25-dihydoxycholecalciferol –> calcitriol)
- ↑ACE (produced by recently activated macrophages in granulomas)
- ↑ ESR
What is the management for sarcoidosis?
Steroids
NSAIDs
Steroid-sparing agents
What is systemic lupus erythematosus?
A chronic multi-system autoimmune disorder
What is the epidemiology for SLE?
Young (20-40)
Afro-Caribbean
Female
What are the symptoms of SLE?
SOAP BRAIN MD
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
What are the investigations for SLE?
Bloods: ESR, U+E, FBC
Autoantibodies: anti-dsDNA, ANA, anti-cardiolipin
Urinalysis: casts, proteinuria, haematuria
CXR
Joint X-ray
What other condition can arise from SLE?
Anti-phospholipid syndrome
What is anti-phospholipid syndrome? Which antibody is present?
Triad of:
- thromboembolism
- recurrent miscarriage
- thrombocytopenia
Presence of ANTI-CARDIOLIPIN antibody
Define systemic sclerosis
A rare connective tissue disorder characterised by widespread small blood vessel damage + fibrosis in skin (scleroderma) and internal organs
What are the 4 types of systemic sclerosis? Which antibodies are implemented in each?
- DIFFUSE CUTANEOUS- anti-topoisomerase II (anti-Scl-70) antibodies
- LIMITED CUTANEOUS (CREST)- anticentromere
- PRESCLERODERMA- ANA antibodies
- SCLERODERMA SINE SCLERODERMA- ACA antibodies
What are the characteristics of diffuse cutaneous systemic sclerosis?
- Skin changes involving the trunk AND limbs
- Raynaud’s phenomenon
- Arthralgia/arthritis
- TENDON FRICTION RUBS
Anti-topoisomerase II antibody
What are the characteristics of limited cutaneous systemic sclerosis?
CREST: Calcinosis Raynaud's phenomenon oEsophageal dysmobility Sclerodactyly Telangectasia
Anti-centromere antibody
What is vasculitis?
Inflammation of the blood vessel walls, with often an unknown aetiology, systemic effects, and distinctive features.
What is giant cell arteritis?
Granulomatous inflammation of the large arteries affecting the external carotid artery, most commonly temporal artery.
What are the symptoms of GCA?
Unilateral headache Jaw claudication Scalp tenderness Visual disturbances + vision loss Systemic upset (fever, malaise, weight loss) Symptoms of PMR
What are the investigations/diagnostic criteria for GCA?
> 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)
What is the management for GCA?
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.
What is polymyalgia rheumatica?
Inflammatory rheumatological condition of unknown cause characterised by bilateral hip and shoulder girdle pain and morning stiffness,
NO weakness.
RF/aetiology of PMR
> 50
Females
15% develop GCA
How does PMR present?
Bilateral hip/shoulder girdle pain
Morning stiffness
Subacute onset (>2 weeks)
Systemic (FLAWS)
What is polyarteritis nodosa?
A vasculitis affecting medium-sized vessels (arterioles, capillaries, or venules)
What is the aetiology of PAN?
Idiopathic
Associated with HBV
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)
What can be seen in a renal angiogram in PAN?
Rosary sign
What is granulomatosis with polyangiitis?
aka Wegener’s
Triad of:
- URT (rhinitis, epistaxis)
- LRT (haemoptysis, heamorrhage)
- kidneys (glomerulonephritis)
Saddle nose
What are the investigations for GPA/Wegeners?
Serum cANCA
CXR- cavitating lesions
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
What are the investigations for eGPA?
pANCA
What is Behcet’s disease?
Triad of oral ulcers, genital ulcers, and uveitis
What is the background of Behcet’s disease?
Common in the Mediterranean (Greasy Turkeys)
Associated with HLA-B51
What are the clinical features of Behcet’s disease?
Triad of: -recurrent oral ulcers -genital ulcers -uveitis Also colitis, rash, arthritis, pericarditis (CRAP)
What are the investigations for Behcet’s disease?
Clinical diagnosis
Can confirm with Pathery test
What is the Pathergy test?
Needle skin prick forms a sterile pustule within 48h
What is Takuyasu’s arteritis?
A large vessel vasculitis causing occlusion of the aorta and its main branches
RF for Takuyasu’s arteritis
ASIAN
20-40yrs
Females
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
What is the background of Henoch-Schönlein purpura?
Affects 3-15 yr olds
IgA vasculitis
What are the symptoms of Henoch-Schönlein purpura?
Triad of:
- purpuric rash on buttocks and extensors of lower limbs
- abdo pain
- arthralgia
What is the background of Goodpasture’s syndrome?
Anti-GBM antibodies
What are the symptoms of Goodpasture’s syndrome?
Triad of:
- glomerulonephritis
- haemoptysis (pulmonary haemorrhage)
Anti-GBM antibodies