Vasculitis and Autoimmune disease Flashcards
Sarcoidosis
Definition
Epidemiology
Symptoms and signs
Investigations
Management
Sarcoidosis
Definition
Multi system granulamtous inflammatory disease, characterised by non caseating granulomas
Epidemiology
Afro Carribeans
Scandanavians
Age- >50
Symptoms and signs
Lungs- dry cough, dyspnea [causes lung fibrosis]- Ninety percent of symptoms
General: fever, malaise and weight loss
Musc: arthralgia
Skin: erythema nodosum, lupus pernio
Eyes: uveitis, keratoconjuncitivis
Cardio: arrthymia, heart failure
Investigations
Bloods:
FBC
U + E
Ca- high
ACE- high
ESR- high
CXR:
Bilateral hilar lymphadenopathy
Pulmonary fibrosis
BIOPSY: non caseating granuloma
Management
Steroids
NSAIDS
Steroid sparing agents
SLE
Definition
Epidemiology
Symptoms and signs
Investigations
SLE
Definition
Multisystem autoimmune inflammatory disorder
Epidemiology
Females
Young
Other autoimmune disorders
Afro Carribean
Symptoms and signs
SOAP BRAIN MD
Serositis
Oral ulcers
Arthritis
Photosensitivity
Bloods- low blood cell counts
Renal failure
Antibodies - ANA
Immunological- other antibody- anti-ds DNA
Neurological symptoms- psychosis, seizures
Malar rash
Discoid rash
Investigations
- Bloods:
FBC
U and E
ESR - high
Autoantibodies:
ANA
Anti ds DNA
Anti cardiolipin
- Urine:
- casts
- proteinuria
- haematuria
- CXR
What are the three categories of vasculitides and which conditions come under them?
Large vessel vasculitides
Giant cell arteritis
Polymyalgia Rheumatica
Medium vessel vasculitides
Polyarteritis nodosa
Small vessel vasculitides
Behcet’s disease
Granulomatosis with polyangitis [Wegener’s]
Eosinophilic Granulomatosis with Polyangitis [Churg-Strauss]
Systemic Sclerosis [Scleroderma]
Definition
Types - definition, symptoms, antibody
Systemic Sclerosis [Scleroderma]
Definition
Rare connective tissue disease characterised by fibrosis in skin + organs + widespread blood vessel damage
Types
- Diffuse cutaneous systemic sclerosis
- Widespread, all over skin, affects multiple organs
Symptoms:
Skin changes - trunk
Heart, GI, renal disease
Early lung disease
Tendon friction
Raynaud’s phenomenon
Antibody:
anti- topoisomerase II / anti_Scl 70
- Limited cutaneous systemic sclerosis
- limited to certain systems
Also known as CREST syndrome
Symptoms:
Calcinosis- high calcium
Raynaud’s phenomenon
Esophageal dysmotility
Sclerodactyly {thick tight skin around fingers + hard bumps on fingers}Teleangiectasia
Antibody:
- Anti centromere
SBA 1
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
SBA 2
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
SBA 3
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
Features of anti phospholipiid syndrome?
+ antibody involved?
+ condition it’s related to?
- Triad
o Recurrent miscarriages
o Thromboembolism/VTE- PE/DVT
o Thrombocytopenia
- Antibody= anti cardiolipin
- Complication of SLE
Features of diffuse cutaneous systemic sclerosis
+ antibody involved
- Skin changes on trunk
- Heart, GI and renal disease
- Early lung disease
- Tendon friction
- Raynaud’s phenomenon
Antibody: anti-topoisomerase II or anti Scl-70
Features of Limited Cutaneous Systemic Sclerosis/CREST syndrome
+ antibody involved
- Calcinosis
- Raynaud’s phenomenon [hypoperfusion of fingers- go white, then red?]
- Esophageal dysmotility
- Sclerodactyly
- Telangiectasia
Antibody- anti centromere
Vasculitides [in general]
Definition
Aetiology
Types
Vascultides
Definition:
Inflammation of the walls of blood vessels.
Systemic Effects.
Distinctive features.
Aetiology
Autoimmune, antibody mediated?
Often unknown
Types
Large vessel
Medium vessel
Small vessel
Vasculitides SBA 1
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
Diagnosis likely temporal arteritis/GCA
Which condition is strongly associated with temporal arteritis?
[hint below- multiple choice]
A Takayasu’s aortitis
B Myalgic encephalomyelitis
C Fibromyalgia
D Polymyalgia rheumatica
E Polymyositis
D Polymyalgia rheumatica
Vasculitides SBA 3
47 y old man
Presents to GP
Skin rash
Feeling generally unwell for past three months
Abdo pain with rectal bleeding
On CT angiogram: Rosary sign
Regularly attends hospital for check ups since diagnosis of chronic hep B two 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
Giant cell arteritis
Definition
Aetiology
Symptoms [and signs]
Investigations
Treatment
Complications
Giant cell arteritis
Definition
Large vessel vasculitis
Associated with polymyalgia rheumatica
Red flag cause of headache
Aetiology
Idiopathic
Temporal artery inflammation
Epidemiology
Old
Female
Symptoms [and signs]
Unilateral headache - triggered by touching/brushing hair
Thickened temporal artery
Scalp tenderness
Jaw claudication- [pain worse on eating/talking]
Loss of vision [temporary-amaurosis fugax]
Systemic signs- malaise, fever, weight loss
Symptoms of polymyalgia rheumatica [bilateral morning shoulder and pelvic pain]
Investigations
Check ESR [before starting steroid treatment, but then start steroids ASAP]
Temporal artery biopsy
- do in multiple regions- because often damage only seen in certain parts, so not v sensitive- skip lesions not continuous
[biopsy shows giant cells]
Treatment
High dose oral prednisolone ASAP
[Don’t delay treatment- could lead to permanent loss of sight]
Complications
Permanent loss of sight
Polymyalgia Rheumatica
Definition
Background
Symptoms and signs
Investigations
Management
Polymyalgia Rheumatica
Definition
Joint pain disorder associated with GCA [fifteen % of patients will get GCA]- chronic
Symptoms and signs
Chronic
Bilateral shoulder pain
Bilateral pelvic pain
Morning stiffness
For more than 6 weeks
No weakness
Investigations
ESR- high
CRP- high
Management
Steroids- oral
Analgesia initially?
Polyarteritis Nodosa
Definition
Aetiology
Associated disease
Symptoms and signs
Polyarteritis Nodosa
Definition
Aetiology
Idiopathic
Associated disease
- Hep B
Symptoms and signs
- Rash
- Abdominal pain
- Rectal bleeding
- Renal failure
- Hypertension
- Peripheral neuropathy
- Fever, malaise, lethargy
Rosary sign
Vasculitides SBA 5
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
Vasculitides SBA 4
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 polyangitis [also known as Wegener’s]
Vasculitides SBA 6
Churg-Strauss syndrome is associated with:
A pANCA
B cANCA
C Anti-GBM antibodies
D Anti-LKM antibodies
E Anti-smooth muscle antibodies
B cANCA
Eosinophilic granulomatosis with polyangitis [Churg Strauss]
Definition
Antibody type
Symptoms and signs
Investigations
Eosinophilic granulomatosis with polyangitis [Churg Strauss]
Definition
Small vessel vasculitis which causes high eosinophil infiltration
Antibody type
pANCA
[EosinoPhil}
Symptoms and signs
Triphasic:
Allergic: asthma, rhinitis
Eosinophilic: tissue damage in lungs and GI tract
Vasculitis: widespread organ damage and death
Other symptoms:
Haemoptysis
Kidney damage
Rash
Local neuropathy
Investigations
pANCA
Granulomatosis with polyangitis [Wegener’s]
Definition
Symptoms and signs
Investigations
- antibody
Granulomatosis with polyangitis [Wegener’s]
Definition
Small vessel vasculitis, affects lungs and kidneys
Symptoms and signs
Upper resp: rhinitis, nose bleed
Lower resp: haemoptysis
Kidneys: haematuria, proteinuria
Saddle nose
Investigations
cANCA
CXR- cavitating lesions
Behcet’s disease
Definition and background
-
-
Epidemiology
-
Symptoms and signs
Investigations
Behcet’s disease
Definition and background
- Small vessel vasculitis
- HLA B51
Epidemiology
- Medittereanean- Greece and Cyprus
Symptoms and signs
TRIAD:
Oral ulcers
Genital ulcers
Uveitis
Also [a bunch of inflammation]:
Rash- eg erythema nodosum
Arthritis
Pericarditis
Colitis
Investigations
Clinical diagnosis
Pathergy test- needle prick becomes inflamed and sterile pustule forms in 48 hr
Large vessel vasculitis- fill in table
Medium vessel vasculitis table- fill in
Small vessel vasculitis tables- fill in
If in doubt for small vessel vasculitis: remember-
overall likely to affect:
Lungs
Kidneys
GI tract
Rash
Rhinitis
Unrelated difficult SBA courtesy of Laz
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
Melanoma tend to have brain mets
Just because neuro signs and skin lesions doesn’t mean neurofibromatosis
Symptoms suggest space occupying lesions - persistent headaches for 6 months + focal neurological signs: seizures, parasthesia, changes in speech, vision and hearing
Majority of brain tumours are mets
Skin lesion likely to be a melanoma
Gardener means likely to have risk factor of high UV exposure
Glioblastoma multiforme and meningoma tend to be single discrete lesions
Tuberous sclerosis is a hereditary condition that leads to the formation of multiple benign tumours across the body. It is an autosomal dominant condition so most people with tuberous sclerosis will be aware that they have it and will not be presenting at the age of 43.
Neurofibromatosis type 1 tends to cause fewer central nervous system lesions and has more widespread peripheral features, such as neurofibromas, café au lait macules (> 5 is significant), axillary freckling, Lisch nodules and spinal scoliosis.
SBA
51-year-old secretary visits her GP complaining that her shoulders are painful and that she has found it difficult to lift herself out of her chair at work. She has not, however, experienced any difficulty typing on her computer. An upper limb neurological examination is performed, shoulder abduction is 3/5 bilaterally. She has no other symptoms.
Which investigation is most likely to provide a definitive diagnosis?
A Autoantibody screen
B Creatine kinase
C EMG
D CRP
E Muscle biopsy
What is the likely condition?
E Muscle biopsy
Polymyositis
What is the difference between dermatomyositis and polymyositis?
SBA
A 46 year old woman presents with symmetrical PIP and DIP finger swelling and erythematous scaly plaques over the same joints. She has also noticed a purple coloured swelling/ rash on her upper eyelids. Lab results reveal raised ESR, raised CRP, and raised CK (Creatine Kinase). ANA positive, Anti-Mi2 antibodies positive
What is the most likely diagnosis?
A.Rheumatoid Arthritis
B.SLE
C.Dermatomyositis
D.Haemochromatosis
E.Polymyositis
C Dermatomyositis
Picture shows: Gottron’s papules
Rash around eyes is called: Heliotrope rash
Non ANCA related small vessel vasculitis- fill in table
Anti GBM= antibody against glomerular basement membrane
Dermatomyositis
Definition
Symptoms/Main features
Dermatomyositis
Definition
Idiopathic inflammatory myopathy characterised by cutaneous skin lesions
Symptoms/key features
Heliotrope rash- purple rash on eye
Gottron’s papules- [see other flashcards for picture]
Skin lesions are frequently the initial presenting complaint, with muscle involvement developing later.
Polymyositis
Definition
a connective tissue disease characterised by inflammation of the muscles.
Differs from dermatomyositis in that polymyositis does not cause a rash.
Patients experience diffuse weakness in the proximal muscles (distal muscles tend to be spared).
Takayasu’s arteritis
Definition
Aetiology/risk factors
Epidemiology
Symptoms
Signs
Investigations
Takayasu’s arteritis
Definition
Large vessel vasculitis in aortic arch
Epidemiology
Female
< Forty years old
Asian
Genetic predisposition
Symptoms
Limb claudication
Chest pain
Systemic: fever, lethargy, myalgia etc.
Signs
Bruits
Unequal blood pressure in both arms
High ESR/CRP
Absent/weak pulses
Investigations
Microscopic polyangitis
Definition
Aetiology/risk factors
Epidemiology
Symptoms
Signs
Investigations
Microscopic polyangitis
Definition
Small vessel vasculitis
Aetiology/risk factors
Unknown
Epidemiology
50 years average onset
More males but both sexes affected
Symptoms and Signs
Glomerulonephritis
Haemoptysis
Skin rash
Joint pain
Investigations
pANCA
What does this guy have?
wegner’s granulomatosis
what does this guy have
wegner’s granulomatosis
What does this guy have?
Behcet’s
What is this sign?
What is it indicative of?
Rosary sign
polyarteritis nodosa
What does this guy have?
What is it associated with
polymyaligia rheumatica
temporal arteritis
what does this guy have?
sarcoidosis
What does this lady have?
What is she at higher risk of getting?
Malar rash - SLE
Antiphospholipid syndrome
What are these antibodies prominent in which condition
