Vasculitis Flashcards
Features of polymyalgia rheumatic
generally >50
subacute onset (<2w)
Bilateral aching and/or tenderness + morning stiffness in shoulders, hips and proximal limb muscles
Fatigue, fever, decreased weight, anorexia and depression
NO weakness
Mild polyarthritis, tensynovitis and carpal tunnel syndrome
Investigations for PMR
High CRP
ESR typically >40 may be normal
CK levels are normally (exclude myositis/myopathies)
Mx for PMR
Prednisolone 15mg a day PO
Dramatic response <1w, consider alternative if not
Decrease prednisolone dose slowly according to sx and ESR
Addition of MTX under specialist supervision for those at risk of relapse/prolonged therapy
Advise patient seek urgent review if sx of temporal arteritis develop
What is polyarteritis nodosa?
necrotising vasculitis that causes aneurysms and thrombosis in medium-sized arteries
Leads to infarction in affected organs and severe systemic sx
2x common in men
Features of polyarteritis nodosa
skin: rash, “punched out” ulcers, nodules
fever, malaise, arthralgia
weight loss
HTN
mononeuritis multiplex, sensorimotor polyneuropathy
testicular pain
livedo reticularis
haematuria, renal failure
Investigations for polyarteritis nodosa
Increased WCC
mild eosinophilia
anaemia
increased ESR and CRP
Rule out other organ involvement: LFTs, Hep B and C serology, CK, creatinine, urine analysis
CXR - exclude other forms of vasculitis, as typically lung sparing
Biopsy - of affected organ to confirm diagnosis
Imaging: arteriography (mesenteric or renal) - alternative to diagnose show aneurysms and irregular constrictions in vessels
CT/MRI: wedge shaped renal infarctions (less specific)
Acute mx for polyarteritis nodosa
Induction of remission: Prednisolone 1mg/kg/day tapered up until improve and gradually when sx improve taper down
Add cyclophosphamide 2mg/kg/day PO or 15mg/kg IV every 2-3w
Mx of remission and complications of polyarteritis nodosa
Remission:
- Azathioprine or MTX: can replace cyclophosphamide with these as maintenance
Steroids: continue lower dose and taper gradually
Cx:
- anti-HTNs
- avoid nephrotoxic drugs
- dialysis
- peripheral neuropathy - gabapentin or amitriptyline
What is granulomatosis with polyangiitis
multi-system disorder of unknown cause characterised by necrotising granulomatous inflammation and vasculitis of small and medium vessels
commonly affects upper resp tract, lungs and kidneys
Features of granulomatosis with polyangiitis
non-specific sx: fever, malaise, arthralgias and weight loss
TRIAD: Upper resp tract, lower resp tract and glomerulonephritis
Upper resp:
- sinusitis, nasal crusting/discharge, epistaxis, subglottal stenosis - hoarseness or stridor, otitis media, hearing loss, ear pain
Lower resp:
- dyspnoea, cough, pleuritis, haemoptysis, pulmonary infiltrates or nodules
Glomerulonephritis
- microscopic haematuria, urinary sediment, can cause CKD
Scleritis/episcleritis, orbital mass
cutaneous - leukocytoclastic angiitis, petechiae, nodules and vesicles
peripheral sensorimotor polyneuropathy
Investigations for granulomatosis with polyangiitis
cANCA positive in >90%
pANCA positive in 25%
CXR: cavitating lesions, nodules +/- fluffy infiltrates of pulmonary haemorrhage
Urinalysis: proteinuria and haematuria
CT: diffuse alveolar haemorrhage
Induction of remission of granulomatosis with polyangiitis: life/organ-threatening disease
1st line = methylprednisolone IV for 3-5d (followed by oral prednisolone) and cyclophosphamide
- given for 3-6m
- therapy adjunct: plasmapheresis (severe organ involvement and non-responsive to initial induction therapy)
2nd line = methylprednisolone IV for 3-5d (followed by oral prednisolone) and rituximab IV
Induction of remission in granulomatosis with polyangiitis: non-life/organ threatening
1st line = methylprednisolone IV for 3-5d (followed by oral prednisolone) and MTX (with folic acid)
2nd line = oral prednisolone and cyclophosphamide
oral prednisolone and rituximab
Maintenance of remission in granulomatosis with polyangiitis
1st line = prednisolone and MTX (folic acid)
Prednisolone and azathioprine
duration of maintenance therapy is usually 2y following remission, can vary
+osteoporosis and pneumocystis jiroveci prophylaxis (co-trimoxazole)
Features of eosinophilic granulomatosis with polyangiitis
Triad: adult-onset asthma, eosinophilia and vasculitis (+/- vasospasm, MI, DVT)
sinusitis, allergic rhinitis
Affects lungs, heart, nerves and skin
Present with septic shock, systemic inflammatory response syndrome or glomerulonephritis
Investigations for eosinophilic granulomatosis with polyangiitis
Raised eosinophils
ANCA +ve
Management of eosinophilic granulomatosis
Steroids
Cyclophosphamide
Rituximab
Features of microscopic polyangiitis
fever, weight loss, asthenia
MSK sx: myalgia, arthralgia
Neuro sx: peripheral neuropathy, convulsions, cerebral haemorrhage or infarction
Cutaneous sx: palpable papura, splinter haemorrhage
night sweats
cardiac sx: arrhythmia, HF, pericarditis, MI
heachache
raynaud’s
GI sx
glomerulonephritis
Remission of microscopic polyangiitis
Severe: 1st line = IV cyclophosphamide with high dose steroids, 3-6m
Non-severe: high dose steroids with MTX or mycophenolate mofetil
Refractory: 1st = rituximab if already had cyclophosphamide, if mot IVIG
Maintenance mx of microscopic polyangiitis
1st line = azathioprine or MTX
if C/I: MMF or leflunomide
at least 2y
Features of Henoch-Schonlein purpura
Preceded by hx of URTI
common in males age 3-15
Tetrad: palpable purpura, arthritis/arthralgia, abdo pain, renal disease
Mx of Henoch-Schonelin purpura
Supportive, analgesia, rest and good hydration
Steroids - renal impairment
Repeated urine dipstick and BP
Polymyositis
rare
insidious onset of progressive and symmetrical proximal muscle weakness
autoimmune mediated striated muscle inflammation
can cause dysphagia, dysphonia or resp weakness, difficulty standing, climbing stairs
may be paraneoplastic phenomen
Dermatomyositis
polymyositis plus skin signs
macular rash (shawl sign if over back and shoulder)
lilac-purple (heliotrope) rash on eyelids often with oedema
nailfold erythema (dilated capillary loops)
Gottron’s papules - roughened red papules over knuckles, elbows and knees
Underlying malignancy
Extra-mural signs in myositis
present in both
fever
arthralgia
raynaud’s
interstitial lung fibrosis
myocardial involvement
Investigations for myositis
Certain muscle enzymes can be increased: CK (first line - thousands) ALT, AST, LDH, Aldolase
EMG: shows characteristic fibrillation potentials
Muscle biopsy: confirms diagnosis
MRI: muscle oedema in acute myositis
Anti-MI2 and Anti-Jo1
Mx of polymyositis
1st line = prednisolone
Severe disease = azathioprine or MTX
Refractory cases = IVIG and rituximab
