VASCULAR Flashcards
What are the small vessel Vasculitides?
P-ANCA +ve:
- Goodpasture’s
- 40% Microscopic polyangitis
- 60% Churg-Strauss
- 25% Granulomatosis w/ polyangitis
- Polyarteritis Nodosa
C-ANCA +ve:
- 90% Granulomatosis w/ polyangitis
- 30% of Microscopic polyangitis
ANCA -ve:
IgA ICs
Henoch Schonlein Purpura
What is Microscopic polyangitis?
RENAL impairment: Raised creatinine, Haemautria and Proteinuria
P-ANCA
LUNGS: cough, haemoptysis and dyspnoea
SYSTEMIC: lethargy , myalgia, weight loss, fever
PALPABLE PURPURIC RASH
MONEURITIS MULTIPLEX
What is Churgg-Strauss?
Eosinophilic granulomatosis with polyangitis
P-ANCA
a-2-globulin
Stage 1:
- Allergy
- Bronchial ASTHMA (may be precipitated by Leukotrine Receptor Antagonists) CXR shows pulmonary infiltrates
- Allergic rhinitis: inflammation of the nasal passage ->polyps
- Non-specific fever, malaise, anorexia and weight loss
Stage 2: Eosinohillia >10%
Extravascular granuloma formation
Stage 3: Necrotising Vasculitis
- Organ damage
- Kidney failure
- Petechial rash: palpable purpura and cut/subcut nodules
- Mononeuritis Multiplex: tingling in hands and feet
What is Mononeuritis Multiplex?
Peripheral neuropathy, isolated damge to >2 nerve areas, sensory and motor, painful
What is Granulomatosis with Polyangitis?
Necrotising granulomatous vasculitis
C-ANCA
Classic triad: U+L respiratory tract and renal involvement
RESPIRATORY tract: Upper: - Epistaxis - Sinusitis (mucosal thickening, air-fluid levels) - Nasal crusting SADDLE-SHAPED NOSE
Lower:
- Dyspnoea
- Haemoptysis
- CXR- Ill-defined nodules with cavitation (central lucency) air and fluid
CUTANEOUS: Vascular lesions: - ulceration - nodules - purpura
EYE: proptosis
CRANIAL NERVE LESIONS
Manage with:
- steroids
- cyclophosphamide
- plasma exchange
What is Henock Schonlein Purpura?
Children after infection
- Palpable purpuric rash with localised oedema over buttocks and extensors
- abdominal pain
- Polyarthritis and arthralgia
- IgA nephropathy -> haematuria and renal failure
Treat: analgesia, support kidneys
Usually self-limiting by 1/3 relapse
What are the types of Mesenteric Ischaemia?
Acute Mesenteric Ischameia
Chronic Mesenteric Ischaemia
Ischaemic Colitis
What are the predisposingfactors for Mesenteric Ischaemia?
Age AF Other causes of emboli: - Endocarditis - Malignancy CVD risk factors: - Smoking - HTN - Diabetes Cocaine
What are the features of Mesenteric Ischaemia?
Abdominal pain
Rectal bleeding
Diarrhoea
Fever
Raised WBC with Lactic Acidosis
ECG consistent with AF
What is Acute Mesenteric Ischaemia?
Occlusive embolism of an artery which supplies the small bowel e.g. Superior Mesenteric Artery
Often have a history of AF
Severe onset of pain out-of-keeping with exam findings
Urgent surgery, poor prognosis
What is Chronic Mesenteric Ischaemia?
Relatively rare, non-specific ’intestinal angina’
Causes colicky, intermittent abdominal pain
What is Ischaemic Colitis?
Acute but transient compromise in blood supply to the large bowel leading to:
- Inflammation
- Ulceration
- Haemorrhage
Occurs in ‘watershed’ areas such as splenic flexure that are located at the borders of the territory supplied by superior and inferior mesenteric arteries
AXR: ‘Thumb printing’ due to mucosal oedema/haemorrage
Supportive management
?surgery if peritonitic/ perforated/ haemorrhage
What is the Aetiology of Aortic Dissection?
CHAT
CT disease Congenital e.g. coarctation HTN Aortic atherosclerosis/ aortitis (Takayasu's aortitis, tertiary syphilis) Trauma
How does Aortic Dissection present?
SCAR
Sudden tearing, central pain radiating to back
Coronary artery occlusion-MI or angina or
Carotid obstruction-TIA (hemiparesis, dysphasia, blackouts)
Anterior spinal artery can lead to paraplegia
Renal artery (left) (anuria or renal failure)
How does Aortic dissection present?
CHAMP
COLLAPSING PULSE and an early DIASTOLIC murmur above the aortic area: AORTIC REGURG
Hypertension and a discrepancy in the blood pressure between the arms of >20 mmHg
Arm pulses are unequal
Murmur on the back inferior to left scapula, descends to abdomen
Palpable abdominal mass
What are the Medium Vessel Vasculitides?
Buerger’s
Polyarteritis Nodosa
Kawasaki’s
What is Buerger’s disease?
THROMBOANGITIS OBLITERANS diagnosed by angiogram which shows ACUTE HYPERCELLULAR OCCLUISIVE THROMBOSIS
Affects young male smokers
Ischaemia of extremities: ulceration, claudication, Raynaud’s, absent foot pulses
Superficial thrombophlebitis
What is Polyarteritis Nodosa?
Affects middle aged men ASSOCIATED WITH HEP B
P-ANCA
Systemic features (fever, malaise, arthralgia, weight loss) HTN Testicular pain Renal 70% HAEMATURIA and MICROANEURYSMS NECROTISING VASCULITIS
LIVEDO RETICULARIS mottling, purple-lace, vascular pattern
Can cause pancreatitis
What is Kawasaki’s disease?
Children
HIGH GRADE FEVER RESISTANT TO ANTIPYRETICS
Conjunctival infection Redness Adenopathy (cervical lymphadenopathy) Strawberry tongue red and cracked lips Hands (palms and soles of feet)
CORNONARY ARTERY ANEURYSM (ECHO)
Give HIGH-DOSE ASPIRIN (usually contraindictaed in children due to Reyes) and IV Ig
What are the Large Vessel Vasculitides?
Takayasu’s
Giant Cell Arteritis
What is Takayasu’s disease?
Young (20-40), Asian, Female
Aortic Arch syndrome or pulseless disease:
Occlusion of the aorta and bracnhes-granulomatous thickening of the aortic arch
Aortic Regurgitation in 20%
Obliterative: absent limb pulses (U. Limbs mainly) claudication
- Systemic (fever, malaise, raised ESR in acute)
- Unequal BP in upper limbs
- Carotid bruit
- Intermittent claudication
- Pulselessness-absent radial pulse
- Massive intimal fibrosis: stenosis of renal artery, thrombosis and aneurysms
Steroids
What is Giant Cell Arteritis?
Overlaps with Polymyalgia Rheumatica
CRANIAL/TEMPORAL arteritis affects CERTAIN SECTIONS - SKIP LESIONS GRANULOMATOUS
> 60 F
- Rapid onset
- Headache
- Jaw claudication
- Visual distrubances (anterior ischaemic optic neuropathy)
- Tender, palpable temporal artery (biopsy for skip lesions)
- Systemic: lethargy, depression, low grade fever, anorexia, night sweats
Raised ESR and CRP
Dramatic response to high dose prednisolone
What is Polymyalgia Rhuematica?
GIANT CELL VASCULITIS w SKIP LESIONS, MUSCLE BED ARTERIES most affected
>60
- Rapid onset (<1 month)
- Morning stiffness of proximal muscles with aching and mild polyarthralgia
- Systemic: lethargy, depression, low grade pyrexia, anorexia, night sweats
High ESR (>40) Low CDG+ T cells Dramatic response to high dose prednisolone
What is Behcet’s syndrome?
Complex, MULTI-SYSTEM autoimmune of ARTERIES and VEINS
East mediterranean young (20-40) men, FHx
Traid of:
- GENITAL ULCERS (painful)
- ORAL ULCERS
- ANTERIOR UVEITIS
- Thrombophlebitis
- Arthritis
- Neurological: ASEPTIC MENIGITIS
- GI-Colitis, diarrhoea and pain
- ERYTHEMA NODOSUM
- DVT
PATHERGY TEST: needle prick becomes inflamed with pustule