Vasculitis Flashcards
ANCA-associated Small Vessel Vasculitis, Henoch-Schonlein Purpura (HSP), Polymyalgia Rheumatica
What is the primary antibody associated with ANCA-associated vasculitis?
ANCA - IgG
What type of granulomatous inflammation is seen in Granulomatosis with Polyangiitis (GPA)?
Granulomatous inflammation affecting small and medium-sized vessels
Which organ systems are primarily affected in GPA?
Upper and lower respiratory tract, kidneys, eyes, and skin
What is the characteristic nasal deformity seen in GPA?
Saddle nose deformity
What type of ANCA is most commonly associated with GPA?
cANCA (anti-PR3)
What distinguishes Eosinophilic Granulomatosis with Polyangiitis (EGPA) from GPA?
- Late-onset asthma
- High eosinophil count
- ANCA specificity
Which antibody is most commonly associated with EGPA?
Anti-MPO and anti-PR3
How does MPA differ from GPA?
MPA does not affect the nasopharynx, whereas GPA does
What is the most common ANCA type associated with MPA?
pANCA (anti-MPO)
What blood markers are typically raised in ANCA-associated vasculitis?
ESR, PV, CRP
What is the first-line treatment for localised/early systemic ANCA vasculitis?
Steroids + methotrexate
What is the treatment for generalised/systemic ANCA vasculitis?
IV steroids + cyclophosphamide
What treatment is used for refractory ANCA vasculitis?
IV immunoglobulin + rituximab
Perinuclear anti-nuclear cytoplasmic antibody (pANCA) - positive
This suggests what?
Microscopic polyangiitis
Eosinophilic granulomatosis with polyangiitis (EGPA)
Cytoplasmic anti-nuclear cytoplasmic antibody (cANCA) - positive
This suggests what?
Granulomatosis with polyangitis (GPA) (Wegener’s)
A 58-year-old man who was recently referred to rheumatology is undergoing further investigations due to his two-month history of fatigue, weight loss, joint pain, skin ulcers and haemoptysis. He is found to have new renal impairment and a renal biopsy confirms a small vessel vasculitis. The patient is subsequently diagnosed with microscopic polyangiitis
.
What investigation finding is most associated with this condition?
p-ANCA positive
What type of vasculitis is Henoch-Schönlein Purpura (HSP)?
HSP is an IgA-mediated small vessel vasculitis affecting the
1. skin
2. GI tract
3. kidneys
4. joints
What is the most common age group affected by HSP?
affects children aged 2-11 years, though it is rare in infants.
What are the key clinical features of HSP?
(1) Purpuric rash (typically on buttocks and lower limbs)
(2) Colicky abdominal pain
(3) Bloody diarrhoea
(4) Joint pain and swelling
(5) Renal involvement
What is a common trigger for HSP?
A preceding infection (often Group A Streptococcus) occurs in 75% of cases, usually 1-3 weeks before symptom onset
How is the rash in HSP different from other rashes?
HSP rash is palpable purpura, meaning it is raised and does not blanch under pressure.
What investigation confirms renal involvement in HSP?
Urine dipstick to check for haematuria and proteinuria
What is the usual prognosis of HSP?
Self-limiting within 8 weeks, but relapses can occur
Which antibodies are found in the immune complexes deposited in the affected blood vessels in Henoch Schonlein Purpura?
IgA
A 3-year-old boy is brought to the paediatric emergency department by his father. His father is concerned as he has developed a pinprick purple rash on the back of his legs and has been complaining of stomach and knee pain for the last few days. Before this, he had a cold.
His vital signs are normal.
What is the most appropriate management of this condition?
NSAIDS
A 5-year-old boy presents with a 3-day history of severe abdominal pain, painful knees, and a purple rash on his legs and buttocks. He is drinking but not eating much, has normal bowel movements, and brownish urine. He denies vomiting, diarrhoea, headache, neck stiffness, or photophobia. He had a cold 2 weeks ago and is up to date on vaccinations.
He appears well but in pain. Vital signs: RR 20, O2 99%, HR 82, capillary refill 2 seconds, BP 122/76, temperature 37.1°C. A purpuric rash is present on the legs and buttocks. What is the mechanism of this child’s condition?
Small vessel vasculitis
What is polymyalgia rheumatica (PMR)?
(1) A chronic inflammatory condition affecting muscles around the shoulders, neck, and hips
(2) Seen in patients >50 years
(3) Often associated with giant cell arteritis
What are the key symptoms of PMR?
(1) Proximal muscle pain and stiffness (2) Especially in the morning (>45 mins) (3) Difficulty getting out of bed
(4) Systemic symptoms (fatigue, weight loss)
(5) muscle strength is preserved
How is PMR diagnosed?
Raised ESR/CRP but normal CK (differentiates from polymyositis)
What is the first-line treatment for PMR?
Prednisolone 15mg daily, tapered gradually over 18 months - 2 years, with a dramatic response to steroids
What is the treatment for organ-threatening PMR?
Steroids + Cyclophosphamide
What age group does Polymyalgia Rheumatica (PMR) most commonly appear in?
over 60 years old
If a patient over 60 develops new-onset diabetes mellitus along with weight loss, what diagnosis should you consider?
Pancreatic Cancer
Which disease is polymyalgia rheumatica (PMR) highly associated with?
Giant cell arteritis
A 59-year-old woman comes to the GP complaining of 3 weeks of shoulder and hip pain and stiffness. She thinks she has lost around 2kg in the last month unintentionally. She reports no neurological symptoms, headache or visual loss.
The GP sends off FBC, U+Es, ESR, CRP and TFTs.
What other investigations would you send the first line?
Bone profile, protein electrophoresis
A 63-year-old gentleman attends the GP following a month’s pain in his hips and shoulders. He finds he has stiffness in his shoulders that improves after an hour of waking. He has had neck pain since last week.
What condition is most commonly associated with the condition described above?
Giant cell arteritis
A 67-year-old woman presents with a few days’ history of pain and stiffness in her shoulders and upper arms. She used to be independent with her activities of daily living (ADLs). However, she has been struggling with dressing, bathing and cooking as her movements of the upper arms are limited by pain. There are mild crepitations on the passive movement of her shoulders and active movement is limited by pain in the muscles. She is denying any ocular or dermatological symptoms. She is not on any medications.
What investigation is essential in confirming the most likely diagnosis?
ESR
An 85-year-old woman presents to the GP with a six-week history of pain and early morning stiffness in the shoulders and hips. She was diagnosed with polymyalgia rheumatica and started on steroids.
What further scan should the GP consider doing?
DEXA SCAN - assess fracture risk
A 70-year-old woman complains of severe bilateral shoulder and hip girdle pain and stiffness for the past 2 weeks.
Given the most likely diagnosis, which blood test is most likely to be elevated?
ESR
A 45-year-old patient presents with bloody nasal discharge, joint pain, and rapidly progressive glomerulonephritis; given the most likely diagnosis, what autoantibody is most likely to be positive?
c-ANCA (PR3-ANCA)
What are the key respiratory features of Granulomatosis with Polyangiitis (GPA)?
- Upper respiratory symptoms
= sinusitis, nasal crusting, nasal discharge - Lower respiratory symptoms
= shortness of breath, haemoptysis, chest pain
Which antibody is classically associated with eosinophilic granulomatosis with polyangiitis?
p-ANCA
A 36-year-old gentleman presents to a Rheumatology Clinic for a review. Over the last year, his health declined significantly. He has a history of asthma and later rhinorrhoea and nasal obstruction, which required surgical polyp removal. Subsequently, it was noted that his eosinophil count was 14% and he complained of fevers and malaise. Finally, he was seen in a neurology clinic and was diagnosed with peripheral neuropathy.
Given the likely diagnosis, what is most likely to be elevated in this patient?
Eosinophilic Granulomatosis with Polyangiitis
= therefore p-ANCA raised
A 45-year-old man is referred by his GP to rheumatology due to late-onset asthma, persistent sinusitis and eosinophilia. He goes on to have further blood tests which show that he is positive for perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA), and a tissue biopsy, the results of which are also in line with the suspected diagnosis.
Which renal biopsy finding is most expected?
Granuloma
A 40-year-old man presents to the General Practice with shortness of breath and wheeze particularly noticeable on running for the bus in the morning.
He is also complaining of a persistent blocked nose and paraesthesia in both feet. What findings are most likely to support the diagnosis?
MPO
P-ANCA
positive antibodies
A 40-year-old man presents to the GP with fatigue and unintentional weight loss. He reports recurrent sinusitis and rashes of unknown cause. His only medical history consists of asthma which was diagnosed last year.
His blood shows very high eosinophil levels.
The GP suspects eosinophilic granulomatosis with polyangiitis (Churg-Strauss).
What is the most appropriate initial treatment option?
Corticosteroids
