Vasculitis Flashcards
Classify vasculitis: large vessel (2), medium vessel disease (2).
Large: Giant cell arteritis, Takayasu arteritis (affects aorta + its branches)
Medium: PAN (remember bowel ischaemia), Kawasaki (paediatric)
Classify small vessel vasculitis into 2 types and list 3 conditions each.
ANCA +ve: MPA, GPA (Wegener’s), EGPA (Churg-Strauss)
ANCA -ve: HSP, Good Pastures, Cryoglobulinaemic vasculitis
Vasculitis - P in PRICMCP
Constitutional symptoms: fever, malaise, myalgia, arthralgia, fatigue.
Skin-Eyes-ENT-Cardiac-Pulmonary-GI-renal-Neuro.
Skin: purpuric rash (blood spots/purple-colored spots)
Eyes: visual changes (diplopia/visual loss) - think GCA
ENT: nasal congestion, rhinorrhoea, bloody nasal discharge, nasal bridge destruction (broken nose with no trauma)
Cardiac: chest pain
Pulmonary: cough, haemoptysis, lung nodules
Abdo: pain (especially postprandial)
Renal: haematuria, HTN, damaged kidney
Neuro: mononeuritis multiplex (e.g. foot drop, numbness, tingling, lack of sensation, difficulty to move part of the body, painful)
Vasculitis - R in PRICMCP? (3)
Causes of secondary vasculitis.
- Drugs: furosemide, thiazides, hydralazine
- Connective tissue diseases
- Viral infections
Vasculitis - I in PRICMCP? (3)
Essentially establish how the diagnosis was made. Biopsy of the affected tissue is the most reliable way.
Temporal Artery Biopsy (TAB)
Renal biopsy (think small vessel vasculitis, especially ANCA +ve ones)
Skin biopsy of rash: ?Leukocytoclastic vasculitis (LCV)
Vasculitis - C in PRICMCP? (2)
Ischaemia from stenosis from inflammation: IHD, PVD, Stroke
Aneurysm
Anything serious from P(RICMCP)
The general approach to pharmacological tx of Vasculitis?
- Induce remission - usually high-dose Steroids +/- immunosuppressive agent in some disease (e.g. Cyclophosphamide)
- Remission maintenance - steroids are tapered gradually as tolerated (often long, up to 2y in GPA). Both steroid + other immSx agents are continued for a period of time
- Monitoring - for both disease activity as steroids tapers off/ceased + for drug toxicity
Giant Cell Arteritis: Clinical Features, Investigations, Treatment.
Headache, Visual changes, Jaw claudication, Scalp tenderness, Tongue claudication, PMR.
Investigate with ESR, TAB.
Tx with High-dose steroids, often needs >2yrs.
Arm claudication, HTN, the difference in BP each arm. Thoughts?
Consider large vessel vasculitis - Takayasu’s, where vasculitis affecting aorta and its branches + pulmonary artery.
Diagnose with CTA or Aorta.
Polyarteritis nodosa - clinical features - which 3 systems are frequently involved (3)? Inv (2)? Tx (2)
Think this diagnosis: renal (AKI) + bowel ischaemia (mesenteric angina) + mononeuritis. Also coronary arteries.
Otherwise - rash and systemic symptoms (non-specific).
Ask for Mesenteric Angiogram looking for Aneurysms (bead-like), ANCA -ve. Nerve/muscle biopsies.
Tx with High-dose steroids, relapse with Cyclophosphamide.
GPA/Wegener’s - most commonly affected systems? (3), Tx (3)
ENT (destruction of nasal bridge), Lung (haemoptysis, cough, granuloma, nodules), renal (small vessel GN)
Remember WeCner = C disease (nasal, lung, kidney), cANCA +ve (+PR3 positive)
Tx: Induce with pulse steroids + cyclophosphamide → maintenance AZA or MTX
Look for collapsed nasal septum.
Churg-Strauss (EGPA)
Most common organs involved (2) and the most prominent clinical feature (3)
Inv (3)
Treatment (2)
Lung and Renal (sometimes neuro)
pANCA +ve (40%).
Ivx: look for intense eosinophilia in the tissue biopsy, e.g. Sural nerve if PNS affected.
Asthma, migratory pulmonary infiltrates, eosinophilia. Allergic symptoms: allergic rhinitis, eczema.
High-dose steroids +/- Cyclophosphamide
Microscopic Polyangiitis (MPA)
Most common organs involved (2)
Treatment (2)
Renal and Lung.
High-dose steroids + Cyclophosphamide
Goodpasture’s (anti-GBM antibody) disease
Major systems involved (2)
Treatments (3)
Pulmonary (haemorrhage/haemoptysis) but most commonly rapidly progressive GN/renal failure (crescents on kidney biopsy)
Tx: prednisolone + Cyclophosphamide + plasmapheresis to remove anti-GBM antibodies.
Pred ~6 months, Cyclo 2-3 months as maintenance. Monitor anti-GBM levels monthly.
Cryoglobulinaemia: features (3)
Associated diseases (3 categories)
Investigation (2)
Treatment (3)
Non-specific: but suspect when palpable purpura, peripheral neuropathy, arthralgia.
Associated with Haematological (lymphoma, MM, Waldenstrom’s, MGUS), Viral (HIV, Hep B/C), CTD (SLE, Sjogren’s).
Raised Cryo + low C4. Ultimately need biopsy.
Tx: Treat underlying disease. Prednisolone + Rituximab (or Cyclophosphamide) +/- Plasmaphresis (if severe)
Vasculitis clinical exam?
Fever, Pallor, pallor conjunctiva
Rash - purpura, livedo reticularis (NET-like pattern erythematous rash)
ENT: Nasal septum - saddle-shaped nose (look from the side!)
Lung: describe signs, no haemoptysis by bedside?
Abdo: tenderness
Renal: fluid overload - would like to see urine for RBC casts, dysmorphic RBCs, proteinuria.
Neuro: Mononeuritis or full if GCA
What investigation results would you like to see in this patient with Vasculitis? (general approach)
Bloods: anaemia, neutrophilia, raised ESR (often >70), renal impairment, ANCA, anti-GBM, cryoglobulin if hx suggestive.
Urine: dysmorphic RBCs, casts, proteinuria
Imaging: CXR (bilateral diffuse infiltrate). mesenteric/coronary artery bead-like aneurysms in PAN…etc.
Biopsy results: e.g. crescentic GN in Anti-GBM syndrome, TAB, sural nerve biopsy in Churg Strauss.
What is your approach to managing this patient with Vasculitis? (main focus = non-pharm + monitoring)
Goals: confirm Dx, maintenance of remission, screen & Mx complications
Confirm Dx: review biopsy, blood, urine, imagings.
A: screen & treat underlying depression, consider alternative meds if on causative agents (e.g. lasix), consider investigating for underlying CTD, viral (HIV/Hepatitis) - i.e. causes of 2ndary vasculitis.
S: screen for complications
- Steroid related complications: OP, Diabetes, Dyslipidaemia, HTN, Infections, PUD
- ImmSx: cytopaenia, haemorrhagic cystitis
- Vascular complications: IHD, PVD, CVA - guided by symptoms. Start with ECG, Bloods/Urine for renal failure/GN, CXR/CT to look for evidence of pulmonary involvement.
T: Non-pharm
- Educate - complications of disease, meds, prognosis
- CV risk factor Mx: weight loss, smoking cessation, diet, exercise
- Infection prophylaxis/precautions/vaccines
- Steroid plan - written document
- Osteoporosis - DEXA, VitD, Calcium, Bisphosphonate/Denosumab.
- Fertility (Cyclophosphamide - counseling, sperm/ovum banking)
- Malignancy screening - annual full skin exam, age-appropriate malignancy screening + monitor for Haematuria (Cyclophosphamide - bladder Ca)
T: Pharm
- Induce remission with High-dose Prednisolone +/- ImmSx (usually Cyclophosphamide)
- Maintenance: slow reduction of steroids + steroid sparing immSx
- Mesna if Cyclophosphamide (uroprotection)
- Consider Bactrim prophylaxis
Involve family, GP and ensure continued support & motivation
Ensure f/u and screen for drug toxicity & complications of disease as aforementioned.
