AI diseases + Vasculitides Flashcards
Signs + sx for sarcoidosis
General
- malaise, wt loss, fever
Pulmonary (90%)
- SOB, dry cough
Cardio
- heart failure, arrhythmia
Musc
- arthralgia
Eyes
- uveitis, keratoconjuctivitis
Skin
- lupus pernio, erythema nodosum
Ix for sarcoidosis
Bloods
- increased ESR, U&Es, increased Ca2+, increased ACE (released by epithelial cells derived from active macrophages due to formation of granulomas)
CXR
- bilateral hilar lymphadenoapthy, pulmonary infiltration/fibrosis
Biospy
*non-caseating granuloma
Who is typically affected by sarcoidosis?
Afro-Car./Scandi
>50 years old
Sx for SLE
SOAPBRAINMD
S erositis
O ral ulcers
A rthritis
P hotosensitivity
B loods (decreased count)
R enal (proteinuria, casts)
A NA
I mmno (anti-dsDNA)
N euro (psychosis, seizures)
M alar rash
D iscoid rash
Ix for sarcoidosis
Bloods
- increased ESR, U&E, FBC
Auto-antibodies
- anti-dsDNA, ANA, anti-cardiolipin
Urine
- casts, proteinuria, haematuria
CXR
How may anti-phospholipid syndrome present?
Complications due to anti-cardiolipin
- thromboembolism
- reccurent miscarriages
- thrombocytopaenia
What are the different types of systemic sclerosis?
Rare connective tissue disease characterised by widespread blood vessel damage and fibrosis in skin and internal organs
Diffuse cutaeneous
- skin changes involving the trunk
- Raynaud’s phenomenen
- tendon friction
- early lung disease
- heart, GI, renal disease
Limited cutaneous (CREST syndrome)
- C alcinosis
- R aynaud’s phenomenen
- E sophageal dysmotility
- S clerodactyly
- T elangiectasia
Which disease is associated with the following auto-antibodies?
a) anti-topoisemerase II antibody (anti-Scl-70)
b) anti-centromere antibody
c) anti-cardiolipin
d) anti-dsDNA
e) cANCA
f) pANCA
g) anti-GBM
a) Diffuse cutaneous systemic sclerosis
b) Limited cutaneous systemic sclerosis
c) Anti-phospholipid syndrome
d) SLE
e) Granulomatosis w/ polyangitis
f) Eosinophilic granulomatosis w/ polyangitis
g) Goodpasture’s syndrome
Give examples of vasculitides that affect:
a) large vessels
b) medium vessels
c) small vessels
a) Giant cell arteritis
b) Polyarteritis nodosa
c) Granulomatosis w/ polyangitis, Churg-Strauss syndrome, Behcet’s disease
Giant cell arteritis sx based on which artery is affected
Unilateral headache (temporal artery affected)
Jaw claudication (facial artery affected)
Scalp tenderness (temporal artery affected)
Loss of vision (opthalmic artery affected)
B sx
How do you diagnose and treat giant cell a?
Temporal artery biospy (not 100% sen. due to skip lesions)
Check ESR (raised)
High dose oral prednisolone (do not delay)
Which condition is associated with giant cell a?
Polymyalgia rheumatica
=> >50yo, 15% get GCA
=> increased ESR/CRP
=> bilateral shoulder and girdle pain, morning stiffness, chronic, no weakness O/E
=> treat with oral steroids
Polyarteritis nodosa summary card
Idiopathic, associated with Hep B.
Sx => renal failure, hypotension, abdo pain, skin rash, rectal bleeding, peripheral neuropathy, constitutional upset
Ix => Rosary sign (pearl necklace) in CT abdo due to adenomyomatosis of gallbladder
Granulomatosis w/ polyangitis summary card
AKA Wegener’s syndrome
=> saddle nose, upper respiratory tract (rhinitis, nose bleeds), kidneys (glomerulonephritis), lower respiratory tract (haemoptysis)
=> cANCA
=> cavitating lesions on CXR
Churg-Strauss syndrome summary card
Eosinophilic granulomatosis w/ polyangitis
=> haemoptysis, rash, kidney damage, focal neuroapthy
=> allergic (asthma/rhinitis), eosinophilic (high eosinophils damage lungs and GIT), vasculitic (widespread organ damage and death)
=> pANCA
Behcet’s disease summary card
Affects pts from Mediterranean; Greece/Cyprus
=> pericarditis, colitis, arthritis, rash
=> recurrent oral ulcers, genital ulcers, uveitis*
=> clinical diagnosis, can do pathergy test (needle prick inflamed and sterile pustule develops within 48hrs)
Takayasu’s arteritis summary card
Asian women, 20-40yo
=> FLAWS (inflammatory phase)
=> weak/absent UL/LL pulses, claudication (pulseless phase)
Goodpasture’s syndrome summary card
TRIAD:
=> glomerulonephritis
=> haemoptysis (pulmonary haemorrhage)
=> anti-GBM antibodies
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
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
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
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
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
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
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
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 Behcet’s disease
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
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
A 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
E Muscle biospy
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
She has Gottron’s papules
