AI Diseases Flashcards
Define sarcoidosis
Multisystem granulomatous inflammatory disorder characterised by the presence of non-caseating granulomas
Epidemiology of sarcoidosis (age and area)
Afro-Caribbeans and Scandinavians
Usually > 50 years
General s/s of sarcoidosis (3)
fever, malaise, weight loss
Pulmonary s/s of sarcoidosis (2)
SOB, dry cough
Muscular s/s of sarcoidosis (1)
arthralgia
Eyes s/s of sarcoidosis (2)
uveitis, keratoconjunctivitis
Skin s/s of sarcoidosis (2)
lupus pernio, erythema nodosum
Cardio s/s of sarcoidosis (2)
arrhythmia, heart failure
Which are the main and most common symptoms of sarcoidosis
Pulmonary SOB and dry cough
Ix for sarcoidosis (3)
Bloods
CXR
Biopsy
What do you see in the bloods of sarcoidosis (3)
High calcium
High ACE
High ESR
What do you see in the CXR of sarcoidosis (2)
Bilateral hilar lymphadenopathy
Pulmonary infiltration/fibrosis
What do you see in the biopsy of sarcoidosis
Non-caseating granuloma
Epidemiology of SLE (age, sex, race)
Young
Afro-Caribbean
Female
Symptoms of SLE (think of the acronym 11)
Serositis - pleurisy, pericarditis Oral ulcers Arthritis Photosensitivity Bloods (low counts of everything) Renal (proteinuria, casts) ANA Immunological (anti-dsDNA) Neurological (psychosis, seizures) Malar rash Discoid rash
Which auto-antibodies are raised in SLE (3)
Anti-dsDNA
ANA
Anti-cardiolipin
What do you seen the urine of SLE
casts, proteinuria, haematuria
What are forms of serositis seen in SLE (2)
Pleurisy, pericarditis
What is seen in the urine of SLE
Casts, proteinuria, haematuria
Ix for SLE (4 and what do you see in the results)
Bloods: high ESR, U&E, FBC Autoantibodies Anti-dsDNA ANA Anti-cardiolipin Urine: casts, proteinuria, haematuria CXR
What is the main complication of SLE
Anti-phospholipid syndrome
What triad is anti-phospholipid
Thromboembolism
Recurrent miscarriage
Thrombocytopaenia
What antibody is seen in anti-phospholipid syndrome
anti-cardiolipin
What are the two types of systemic sclerosis
Diffuse Cutaneous Systemic Sclerosis
Limited Cutaneous Systemic Sclerosis (CREST Syndrome)
What is systemic sclerosis also known as
Scleroderma
Define systemic sclerosis
rare connective tissue disease characterised by widespread blood vessel damage and fibrosis in skin and internal organs
What happens in diffuse cutaneous systemic sclerosis (7)
Skin changes involving the trunk Raynaud’s phenomenon Tendon friction Early lung disease Heart, GI and renal disease
What happens in limited cutaneous systemic sclerosis (5)
(CREST Syndrome) Calcinosis Raynaud’s phenomenon Eosophageal dysmotility Sclerodactyly Telangiectasia
Which type of systemic sclerosis has a mnemonic to hell it
Limited cutaneous systemic sclerosis
CREST
Which disease is anti-centromere AB involved in
Limited cutaneous systemic sclerosis
Which disease is anti-topoisomerase II AB involved in
Diffuse cutaneous systemic sclerosis
Which antibody is involved in diffuse cutaneous systemic sclerosis
anti-topoisomerase II
Which antibody is involved in limited cutaneous systemic sclerosis
anti-centromere AB
What are the large 1, medium 1 and small cell vasculitis 3’
Large-vessel vasculitis
Giant cell arteritis (and Polymyalgia Rheumatica)
Medium-vessel vasculitis
Polyarteritis nodosa
Small-vessel vasculitis
Behcet’s disease
Granulomatosis with polyangiitis (Wegener’s)
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)
What is GCA associated with
Polymyalgia rheumatica
What is polymyalgia rheumatica associated with
shoulder/pelvic girdle pain associated with GCA
Symptoms of GCA (5)
Unilateral headache Jaw claudication Scalp tenderness Loss of vision Systemic upset (fever, malaise, weight loss)
Treatment of GCA
HIGH DOSE ORAL PREDNISOLONE
Do NOT delay treatment unnecessarily
Ix for GCA
Check ESR
Temporal artery biopsy
Define GCA
an inflammatory condition of unknown cause, characterised by severe bilateral pain and morning stiffness of the shoulder, neck and pelvic girdle. There is NO weakness.
Signs and symptoms of PMR (4)
Chronic
Bilateral shoulder or pelvic pain
Morning stiffness
NO weakness
Ix of PMR
ESR/CRP - raised
Mx of PMR
Oral steroids
What is Takayasu’s aortitis
a large vessel vasculitis that affects the aorta and its branches. q
What is the typical demographic of Takayasu’s aortitis
Typically affects Middle-aged women of Asian descent
What is Myalgic Encephalomyelitis also known as
Chronic fatigue syndrome
What is chronic fatigue syndrome also known as
Myalgic encephalomyelitis
Takayasu’s Aortitis tends to present with… (4)
constitutional upset, absent/weak upper/lower limb pulses and claudication
What does Rosary sign appear in and what imaging modality
Polyarteritis nodosa and CT angiogram
What is polyarteritis nodosa associated with
Hepatitis B
Symptoms of polyarteritis nodosa
Constitutional upset Skin rash Abdominal pain Rectal bleeding Peripheral neuropathy Renal failure Hypertension
Definition of polyarteritis nodosa
a rare type of vasculitis that affects medium-sized vessels.
What triad is seen in granulomatosis with polyangitis
Upper respiratory tract (rhinitis, nose bleeds)
Lower respiratory tract (haemoptysis)
Kidneys (glomerulonephritis)
What is Wegener’s disease also known as
Granulomatosis with polyangitis
What triad is seen in Wegener’s disease
Upper respiratory tract (rhinitis, nose bleeds)
Lower respiratory tract (haemoptysis)
Kidneys (glomerulonephritis)
What big sign suggests Wegener;’s granulomatosis
Saddle nose
Ix for Wegener’s granulomatosis (2)
cANCA
CXR - cavitating lesions
Three phases of eosinophilic granulomatosis with polyangitis
Allergic – asthma/rhinitis
Eosinophilic – high eosinophils can cause tissue damage (mainly in the lungs and GIT)
Vasculitic – can lead to widespread organ damage and death
Features of eosinophilic granulomatosis with polyangitis (4)
haemoptysis, rash, focal neuropathy, kidney damage
Which antibody is Wegener’s associated with
Cytoplasmic ANCA
Which antibody is Churg-Strauss associated with
Perinuclear ANCA
Which disease is pANCA associated with
Churg strauss syndrome
What is Churg Strauss syndrome also known as
Eosinophilic granulomatosis with polyangitis
What is eosinophilic granulomatosis with polyangitis also known as
Churg Strauss syndrome
What is Behcets disease associated with
HLA-B51
Triad seen in Behcets disease
Recurrent oral ulcers
Genital ulcers
Uveitis
S/s of Behcet’s disease (7)
Recurrent oral ulcers
Genital ulcers
Uveitis
also Rash (e.g. erythema nodosum) Arthritis Pericarditis Colitis
Ix for Behcets disease
Clinical diagnosis
Pathergy test – needle prick becomes inflamed and sterile pustule develops within 48 hrs
Triad in Goodpasture’s syndrome
Glomerulonephritis
Haemoptysis (pulmonary haemorrhage)
Anti-glomerular basement membrane antibodies
Triad in Heinloch Schonlein Purpura
Purpuric rash on buttocks and extensors of lower limbs
Abdominal pain
Arthralgia
Demographic of Henoch Schonlein Purpura
Affects 3-15 yr old children
Which Ig is involved in Henoch Schonlein purpura
IgA vasculitis
Which anti-body is involved in good pasture’s syndrome
Anti-glomerular basement membrane antibodies
Which antibody is involved in dermatomyositis
Anti-Mi2
What is the difference between polymyositis and dermatomyositis
Dermatomyositis involves the skin usually of the hands and eyelids
Which antibody is usually raised in both types of scleroderma
ANA
What is scleroderma usually associated with
Sjogrens syndrome