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Flashcards in Vasculitis Deck (18)
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1
Q

Classify vasculitis: large vessel (2), medium vessel disease (2).

A

Large: Giant cell arteritis, Takayasu arteritis (affects aorta + its branches)

Medium: PAN (remember bowel ischaemia), Kawasaki (paediatric)

2
Q

Classify small vessel vasculitis into 2 types and list 3 conditions each.

A

ANCA +ve: MPA, GPA (Wegener’s), EGPA (Churg-Strauss)

ANCA -ve: HSP, Good Pastures, Cryoglobulinaemic vasculitis

3
Q

Vasculitis - P in PRICMCP

A

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)

4
Q

Vasculitis - R in PRICMCP? (3)

A

Causes of secondary vasculitis.

  1. Drugs: furosemide, thiazides, hydralazine
  2. Connective tissue diseases
  3. Viral infections
5
Q

Vasculitis - I in PRICMCP? (3)

A

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)

6
Q

Vasculitis - C in PRICMCP? (2)

A

Ischaemia from stenosis from inflammation: IHD, PVD, Stroke

Aneurysm

Anything serious from P(RICMCP)

7
Q

The general approach to pharmacological tx of Vasculitis?

A
  1. Induce remission - usually high-dose Steroids +/- immunosuppressive agent in some disease (e.g. Cyclophosphamide)
  2. 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
  3. Monitoring - for both disease activity as steroids tapers off/ceased + for drug toxicity
8
Q

Giant Cell Arteritis: Clinical Features, Investigations, Treatment.

A

Headache, Visual changes, Jaw claudication, Scalp tenderness, Tongue claudication, PMR.

Investigate with ESR, TAB.

Tx with High-dose steroids, often needs >2yrs.

9
Q

Arm claudication, HTN, the difference in BP each arm. Thoughts?

A

Consider large vessel vasculitis - Takayasu’s, where vasculitis affecting aorta and its branches + pulmonary artery.

Diagnose with CTA or Aorta.

10
Q

Polyarteritis nodosa - clinical features - which 3 systems are frequently involved (3)? Inv (2)? Tx (2)

A

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.

11
Q

GPA/Wegener’s - most commonly affected systems? (3), Tx (3)

A

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.

12
Q

Churg-Strauss (EGPA)

Most common organs involved (2) and the most prominent clinical feature (3)

Inv (3)

Treatment (2)

A

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

13
Q

Microscopic Polyangiitis (MPA)

Most common organs involved (2)

Treatment (2)

A

Renal and Lung.

High-dose steroids + Cyclophosphamide

14
Q

Goodpasture’s (anti-GBM antibody) disease

Major systems involved (2)

Treatments (3)

A

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.

15
Q

Cryoglobulinaemia: features (3)

Associated diseases (3 categories)

Investigation (2)

Treatment (3)

A

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)

16
Q

Vasculitis clinical exam?

A

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

17
Q

What investigation results would you like to see in this patient with Vasculitis? (general approach)

A

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.

18
Q

What is your approach to managing this patient with Vasculitis? (main focus = non-pharm + monitoring)

A

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.