AI diseases + Vasculitides

Question 1

Signs + sx for sarcoidosis

Answer 1

General
- malaise, wt loss, fever
Pulmonary (90%)
- SOB, dry cough
Cardio
- heart failure, arrhythmia
Musc
- arthralgia
Eyes
- uveitis, keratoconjuctivitis
Skin
- lupus pernio, erythema nodosum

Question 2

Ix for sarcoidosis

Answer 2

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

Question 3

Who is typically affected by sarcoidosis?

Answer 3

Afro-Car./Scandi
>50 years old

Question 4

Sx for SLE

Answer 4

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

Question 5

Ix for sarcoidosis

Answer 5

Bloods
- increased ESR, U&E, FBC
Auto-antibodies
- anti-dsDNA, ANA, anti-cardiolipin
Urine
- casts, proteinuria, haematuria
CXR

Question 6

How may anti-phospholipid syndrome present?

Answer 6

Complications due to anti-cardiolipin
- thromboembolism
- reccurent miscarriages
- thrombocytopaenia

Question 7

What are the different types of systemic sclerosis?

Answer 7

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

Question 8

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

Answer 8

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

Question 9

Give examples of vasculitides that affect:
a) large vessels
b) medium vessels
c) small vessels

Answer 9

a) Giant cell arteritis
b) Polyarteritis nodosa
c) Granulomatosis w/ polyangitis, Churg-Strauss syndrome, Behcet’s disease

Question 10

Giant cell arteritis sx based on which artery is affected

Answer 10

Unilateral headache (temporal artery affected)
Jaw claudication (facial artery affected)
Scalp tenderness (temporal artery affected)
Loss of vision (opthalmic artery affected)
B sx

Question 11

How do you diagnose and treat giant cell a?

Answer 11

Temporal artery biospy (not 100% sen. due to skip lesions)
Check ESR (raised)

High dose oral prednisolone (do not delay)

Question 12

Which condition is associated with giant cell a?

Answer 12

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

Question 13

Polyarteritis nodosa summary card

Answer 13

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

Question 14

Granulomatosis w/ polyangitis summary card

Answer 14

AKA Wegener’s syndrome
=> saddle nose, upper respiratory tract (rhinitis, nose bleeds), kidneys (glomerulonephritis), lower respiratory tract (haemoptysis)
=> cANCA
=> cavitating lesions on CXR

Question 15

Churg-Strauss syndrome summary card

Answer 15

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

Question 16

Behcet’s disease summary card

Answer 16

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)

Question 17

Takayasu’s arteritis summary card

Answer 17

Asian women, 20-40yo
=> FLAWS (inflammatory phase)
=> weak/absent UL/LL pulses, claudication (pulseless phase)

Question 18

Goodpasture’s syndrome summary card

Answer 18

TRIAD:
=> glomerulonephritis
=> haemoptysis (pulmonary haemorrhage)
=> anti-GBM antibodies

Question 19

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

Answer 19

C Calcium

Question 20

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

Answer 20

D Anti-cardiolipin antibody

Question 21

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

Answer 21

A Telangiectasia

Question 22

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

Answer 22

A Check ESR

Question 23

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

Answer 23

D Polymyalgia rheumatica

Question 24

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

Answer 24

C Polyarteritis nodosa

Question 25

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

Answer 25

C Granulomatosis with polyangiitis

Question 26

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

Answer 26

B Behcet’s disease

Question 27

Churg-Strauss syndrome is associated with:

A pANCA
B cANCA
C Anti-GBM antibodies
D Anti-LKM antibodies
E Anti-smooth muscle antibodies

Answer 27

A pANCA

Question 28

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

Answer 28

D Metastases

Question 29

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

Answer 29

E Muscle biospy

Question 30

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

Answer 30

C Dermatomyositis

She has Gottron’s papules