Vasculititis Flashcards
Describe SLE
Multisystem autoimmune disorder that poses a challenge to the emergency clinician with its protean and occasionally dangerous manifestations.
Morbidity for SLE patients is typically mediated through organ inflammation and destruction of the consequences of therapeutic immunosuppression.
Further complicating lupus is the frequent association with antiphospholipid syndrome and its corresponding venous and arterial thromboses.
Discuss presentations with SLE
In general there are four broad presentations of a patient with SLE
1) symptoms related to SLE that is not yet diagnosed
- idiopathic pericarditis
- new rash
2) progression or acute deteriation due to known SLE (e.g progressive nephritis, lupus enterits)
3) Complciations of immunosupression
4) complaints unrelated to SLE
Discuss clinical features associated with SLE
Skin
- Malar butterfly rash
- discoid lesions in discoid lups
- ulcers on central portion of the palate and anterior nasal septu
- alopecia frequent during active disease
- purpura due to thrombocytopenia or necrotising angiitis of small vessels
- sub cut nodules
- Can have bullous lupus, toxic epidermal necrolysis variant
Haem
- anaemia
- leukopenia
- thrombocytopenia
- splenomegaly
CNS
- cerebral invovlement more common than PNS
- headaches
- personality chagnes
- psychoses
- sezirues
- organic brain sydnrome
Renal
- 5% develop CKD requiring dialysis
Cardiopulmonary
- pleurisy
- pericarditisi
- pleural effusion
- pulmonary hypertension
- significant increase in CAD - 52 fold increase in incidence
Raynauds
MSK
-arthritis - typically symmetrical and nonerosive may involve many joints
GIT
- Oral ulceration which occurs in nearly 1/3 of SLE patients
- lupus enteritis
Discuss ix of SLE
Not routinely done in the ED
if considered
BLoods
- ANA
- Anti dna
- ANti smith antibody
- aPL antibody
CXR - pleural effusion, pericardial disease, pneuonia,
ECHO
List medications commonly used in the treatment of an SLE flare
Methyl prednisone 1-2mg/kg
Predinsone 1-2mg/kg
Hydroxychloroquine 200-400 mg PO once daily
Cyclophosphamide 500-750mg/m2 IV once
Azathioprine 25-50mg/day
Describe antiphospholipid syndrome
Present in nearly 40% of SLE patients APS is considered when patients both have a clinical history of thrombosis and are carriers of any one of a particular set of aPL antibodies directed against three specific serum proteins. These include
1) lupus anticoagulant the most thrombogenic
2) anticardiolipin
3) anti-B2-glycoproetin 1 antibody.
Clinical features
- VTE
- Arterial thrombosis (including CVA and TIA)
- Recurrent miscarriage
- Livedo reticularis
- thrombocytopenia
Discuss IX of antiphospholipid syndrome
1) Spuriously elevated PTT in the setting of a normal PT/INR may reflect APS. - This can be confirmed by doing a mixing study - 50% of the of the patient and 50% control if aPL antibodies present PTT will remain elevated if for some other cause it will resolve
2) the VRDL assay test for syphilis contains cardiolipin and thus will be commonly falsely positive.
List vasculitis by vessel predominance
Large vessel involves the aorta and its immediate branches and in some cases the corresponding vessels from the venous system
Medium vessel involves the microvasculature downstream from the aorta
Small vessel involves the capillaries venules arterioles and glomeruli
LARGE VESSEL
- GCA - assymetrical blood pressure
- Takayasu’s – Claudication, aortic disease, renovascular HTN
MEDIUM VESSEL
- Polyarteritis nodusa - cutaneous nodules
- Buergers disease - ulcers
- Kawasaki disease
SMALL VESSEL
- Goodpasture
- Waegeners
- Churg-strauss
- Behcets
- HSP
Describe GCA
Large vessel arteritis with predominance for temporal and occipital artery
Mean onset at 70 and a range of 50-90
Smoking has a 6 fold increase in risk for the disease
Common presenting complaints
- headache - commonly located in the temporal area and with tenderness along the superficial temporal artery.
- visual symptoms
- jaw cluadication
- myalgias and polymyalgia rheumatica
Thoracic aortic aneurusms are 17 times more likley to occur in patients with GCA
Vetebral basilar arteries are affected in 75-100% of patients leading to sings of vertebrobasilar insufficiency including gait disturbance, dizziness or veritgo and vomiting.
Discuss diagnosis of GCA
One point for each
- Age >50
- New onset of localized headache
- temporal artery tenderness or decreased temporal artery pulse
- ESR >50
- Abnormal artery biopsy specimen characterized by mononuclear infiltration or granulomatous inflammation
Scores of 2 require biopsy to confirm - scores of 3 and higher adds little info beyond clinical and lab appraisal
Biopsy gold standard
Colour doppler remains controversial.
Describe Takayasu’s arteritis
Also known as pulseless disease or occlusive thromboaortopathy is a systemic large vessel vasculitis that primarily affects young women of japanese and southeast asian decent.
It is characterised by granulomatous inflammation of the aorta and its branches lading to massive intramural fibrosis and symptoms of large vessel stenosis thrombosis and aneurysm.
The disease should be considered in women younger than 40 years old who present with signs and symptoms of large vessel occlusions including upper limb claudication, decreased or assymetrical pulses and unexplained HTN accompanied by constitutional symtpoms
CVA and aoritc insufficiency and visual symtpoms are presnet in 10%
IX
- ESR
- CTA
Management
- oral corticosteroidfs for 4-12 weeks followed by a taper
- it is self limiting in 20% of patients
List vasculitis that are typified by renal-pulmonary manifestation
These share a strong asscociated with antineutrophil antiobdoies
1) Wegeners granulomatosis
2) microscopic polyangitis
3) churg strauss syndrome
Good pastures does not have ANCA but is pulmnoary renal
Describe Wegeners granulomatosis
A c-ANCA associated systemic necrotizing vasculitis of small and medium sized vessles with predilection for the upper and lower resp tract and the kidneys
CLINICAL FEATURES
RESP
- Upper airway disease is the most common presenting symptoms including (serous OM, hearing loss, sinusisits, nasal mucosal ulceration and septal perforation, epistaxis)
-Subglottic stenosis - present in 16%
-Lower tract symptoms include (cough, dyspnoea, pleuritis and haemoptysis)
-A minority of patient present with diffuse alveolar haemorrhage
RENAL
- tends to be a later finding developing in a subset of patients with generalised disease
- once developed it may evolve rapidly leading to ESRD
OTHER
- Opthal (scleritis, episcleritis, uveitis)
- Cutaneous (palpable purpura, subcut nodules, ulcers)
- Neuro ( mono and polyneuropathy)
- Cardiac (peri and myocardiits)
Discuss management of Wegners
ABCD
-life threatening pulmonary haemorrhage and sequale of decompensated renal failure.
The combination of pulmonary haemorrhage and subglottic stenosis seen in Waegners represents a double hit for difficult airways and requires extreme caution. Although a large tube is preferred for pulmonary haemorrhage consideration and preparation for smaller tubes may be needed.
Combination therapy with corticosteorids and cyclophosphamide is considered teh standard of care
Describe microscopic polyangiits
Systemic small vessel vasculitis is the most common cause of the pulmonary renal syndrome leading to renal failure and pulmonary haemorrhage.
CLINCIAL
- Progressive renal failure in the context of pulmonary haemorrhage.
- glomerulonephritis with subsequent renal insuffiency is a nearly universal finding
- Necrotizing GM with RBC, cast, haematuria, and proteinuria.
Microscopic polyangitis probably exists on a spectrum with Waegners and it is not able to be differeniate with clinically.
Describe Churg strauss syndrome
Allergic granulomatosis is an autoimmune vasculitis of medium sized and small vessels associated with asthma and eosinophilia.
The mean onset is in the 4th decade of life and men are effected more commonly
CLINICAL
- most have asthma and allergic rhinitis several years before onset of vasculitis
-Pulmonary, renal and neuro manifestaitons are common.
PULMONARY
-lung infilitrateds
-pleural effusions
-alveolar haemorrhage
RENAL
-less common thatn other ANCA positive vasculitis
-prefer the lower urinary tract leading to obstructive uropathies.
CARDIAC
-Restrictive pericarditis
-CMX
-CCF
NERUO
-Asymmetrical neuropathy and CN neuropathies are the most common neuro manifestation
IX
- persistent elevated eosinophil count
- ANCA
Describe polyarteritis nodosa
Necrotizing vasculitis of unknown aetiology affected small and medium sized vessels.
Most commonly affects the skin, CNS and GIT> Necrosis occurs preferentially at arterial bifurcations and rbanch sites leading to microaneurysm formation, thrombosis, emboli, organ ischaemia and infarction.
CLINICAL
- Cutaneous lesions + adult onset HTN with evidence of systemic illness is suggestive of PAN.
- Cutaneous lesions occur in 1/3 of cases with palpable purpura with or without ulceration in the fingers, ankles malleoli and pretibial region.
- Digital cyanosis may be present due to ishcaemia.
- Splinter haemorrhages may be observed
- Renovascular arteritis may cause HTN which can be severe
- Mesenteric vasculitis can cause abdominal angina and in rare cases lead to frank infarction and perforation
Universally fatal without treatment which is steroids + second immunosuppressant
Describe Behcets disease
Compex chronic small vessel vasculitis that may affect the mucocatenous, ocular, CVS, renal, GIt, Pulmonary, urological, MSK and CVS
The triad of recurrent oral apthouls ulcers, genital ulcers and uveitis in young adults is highly suggestive. -Lesions have a yellow necrotic base and may appear alone or in crops of 3-10.
Ocular symptoms are common and consitute a major source of morbidity
CNS
- brainstem
- corticospinal syndromes
- asceptic meningoencephalitis
- ICP
- CVT
Steroids and other immunosuppressants
Describe Goodpastures syndrome
Describe the clinical triad of glomerulonephritis, pulmonary haemorrhage and circulating anti-glomerular basement membrane.
The syndrome is distinct from the disease which consist of glomerulonephritis and anti GBM antibodies without pulmonary haemorrahge.
It is not asscoiated with ANCA; anti GBM autoantibodies taret type 4 collagen found in the basement membrane of glomeruli and pulmonary alveoli, causing autoimmune damage through a type 2 hypersensitivity.
Bimodal peak 20-30 and 50-70
Discuss clinical features of goodpastures
Pulmonary or renal symptoms may exist alone or in combination.
PULMONARY
- range from dyspnoea and cough to hypoxia and frank pulmonary haemorrhage, which can be massive and life threatening.
-CXR may be normal or demonstrate hilar pulmonary infiltrates sparing the apicies and costophrenic angles.
RENAL
- can be insidious or manifest as RPGN
- haemoraturia, proteinuria and red cell cast.
Definitive diagnosis is made via tissue biopsy demonstrating linear deposition of antiGBM abs in the alveolar or GBMs respectively
Discuss management
Initial management is focused on securing a definitive airway if pulmonary haemorrhage is a feature.
High dose methypred and cyclophoshamide are the mainstay of immune supressive therapyu.
Plasma exchange is an option
