Pulmonary vasculitis Flashcards
What are the ANCA-assoc vasculitis?
1) Eosinophilic granulomatosis with polyangitis (Churg-Strauss Syndrome)
2) Granulomatosis with polyangitis (Wegener Syndrome)
3) Microscopic polyangitis
What is Granulomatosis with Polyangitis (Wegener Syndrome)?
Triad: renal + lower resp tract + upper resp tract
Common Sxs:
Chronic rhinitis/ sinusitis/ mastoiditis lower resp tract involvement (diffuse alveolar haemorrhage)
Fever, LOW
Extrapulm: renal, skin, skeletal muscle
Ix:
ANCA: Anti-proteinase 3 (cANCA). But does not rule out if negative
Bx: granulomatous inflame and necrotizing vasculitis
HRCT: cavitary or non-cavitary nodules, localized or diffused infiltrate
Poor prognosis: ≥2 organ involvement, renal failure/ lung progression
What is the Rx for pulmonary vasculitis?
Cyclophosphamide causes infertility & bladder toxicity –> rituximab preferred
Depends on severity:
Benign (non-organ threatening)
Dangerous (involving major organs)
Life-threatening: renal failure, pulm haemorrhage, cardiac
Phases of Rx:
1) Induction
- To induce remission
2) Maintenance
- To maintain remission
Induction phase
Benign: glucocorticoid + MTX or MMF
Dangerous: glucocorticoid + cyclophosphamide or rituximab
Life-threatening: glucocorticoid + cyclophosphamide or rituximab + HD or plasma exchange +/- ECMO (if cardiac involved)
Dosing:
Glucocorticoids:
Initial dose: T. pred 1mg/kg/day
In severe cases: iv 7.5-15mg/kg/day for 1-3d
Cyclophosphamide
15mg/kg every 2-3w
Causes infertility & bladder toxicity thus rituximab is preferred
Rituximab
- 375mg/m2 of body surface once/week for 4 infusions
Maintenance phase
Low dose steroid + second-line
Azathioprine 2mg/kg/day preferred in EGPA & GPA
MTX 20-25mg/week
Rituximab 500mg
MMF
If refractory:
Recheck Dx
Use optimal dose
TRO infection, comorbidities
IVIg
Mepolizumab (IL-5 Ab blocker) for refractory EGPA
Prophylactic Abx (e.g. with Bactrim for PCP prophylaxis in those taking cyclophos/rituximab
What is Eosinophilic granulomatosis with polyangitis (Churg-Strauss Syndrome)?
Need to get ≥4/6:
Asthma
Eosinophilia (>10%)
Neuropathy in vasculitic pattern (e.g. mononeuritis multiplex)
Transient pulm infiltrates
Sinus disease
Extravascular eosinophilia on Bx
Usual presentation:
Start with rhinitis with nasal poly –> late onset asthma –> eosinophilia –> systemic vasculitis
Other sites involvement:
Nervous system, skin, joint, kidneys, GIT, cardiac (endomyocardial infiltration, arrythmia, pericarditis, valvular defect)
Ix:
ANCA: Anti-MPO (pANCA). But if –ve, still cannot rule out
HRCT: patchy GGO (pulm haemorrhage) & pneumonia (granuloma formation). Rare: nodule cavitation
ECHO/ cardiac MRI if suspicious of cardiac involvement
Lung Bx: necrotizing angiitis, granulomata, eosinophilia
Poor prognosis:
≥2 organ involvemen
ANCA-negative: usu presents with cardiomyopathy. (ANCA-positive usu has vasculitic type: GN, mononeuritis multiplex)
Types and classification of pulmonary vasculitis
Primary:
Large vessel:
a) Giant-cell arteritis
b) Takayasu’s arteritis
Medium-sized vessel:
a) Polyarteritis nodosa
b) Kawasaki disease
Small vessel:
a) ANCA-assoc:
- Granulomatosis with polyangiitis,
- Eosinophilic granulomatosis with polyangiitis, and
- Microscopic polyangiitis
b) Immune complex:
- Anti-GBM (Goodpasture),
- IgA vasculitis (Henoch-SchÖnlein purpura),
- Cryoglobulinemic vasculitis,
- Anti-C1q
Secondary:
a) Rheumatological
b) Pulmonary-renal (e.g. Goodpasture’s syndrome)
c) Relapsing polychronditis
d) Behcet’s syndrome
e) Chronic infection
f) Lymphoma
g) Drugs
How to diagnose EGPA?
Need to fulfil ≥4/6 criteria:
1) Asthma (obstructive)
2) Eosinophilia
3) Neuropathy (e.g. mononeuritis multiplex)
4) Pulmonary infiltrates (can be transient)
5) Sinusitis
6) Extravascular eosinophilia on Bx (e.g. on skin Bx/ lung Bx)
What are the organs that can be involved in EGPA?
CNS
Skin
Joint
Lungs
GIT
Cardiac - pericarcditis/ valvular defect/ arrythmia)
What are the Ix to Dx EGPA?
1) FBC - eosinophilia
2) pANCA (aka Anti-MPO)
3) HRCT
4) Echo/ cardiac MRI - if cardiac involvement
5) Organ Bx showing eosinophilia e.g. skin/ lung/ kidneys
How to diagnose Granulomatosis with polyangitis (Wegener Syndrome)?
Triad of: renal + upper resp tract + lower resp tract
Common presentation:
rhinitis/ sinusitis/ mastoiditis + DAH/ cavitary nodule + GN
What are the Ix to diagnose GPA?
1) cANCA
2) Tissue Bx: granulomatosis inflammation & necrotizing vasculitis
3) HRCT: cavitary or non-cavitary nodule
What is Microscopic Polyangitis (MPA)?
May mimic GPA
Less common to involve pulmonary
Other sites: necrotizing GN, mononeuritis multiplex, skin lesion, UIP on HRCT (rare)
Histo: no granulomas
Need to differentiate from Polyarteritis nodosa - only affect arteries and arterioles (never small vessels like MPA)
Ix: ANCA-MPO Ab
What are the Immune complex small-vessel vasculitis
Immune complex small-vessel vasculitis:
a) Anti-glomerular basement membrane disease (Goodpasture disease)
b) Cryoglobulinemic vasculitis
c) IgA vasculitis (Henoch-Scholein purpura)
d) Anti-C1q vasculitis (Hypocomplementemic urticarial vasculitis)
What is good pasture syndrome
What is Cryoglobulinemic vasculitis
What is IgA vasculitis (Henoch-Scholein purpura)
What is Anti-C1q vasculitis (Hypocomplementemic urticarial vasculitis)
What are the Ix in pulm vasculitis
