Raynaud's phenomenon, digital ulcers and critical digital ischaemia

Complications of scleroderma
vasculopathy: Raynaud’s phenomenon,
digital ulcers and critical digital ischaemia
Ariane Herrick

Members and contributors to
working group
• Marina Anderson
• Eileen Baildam
• John Coppock
• Chris Denton
• Bridget Griffiths
• Frances Hall
• Jan Lamb
• Anne Mawdsley
• Lindsay Muir
• Pia Moinzadeh
• Tanaka Ngcozana
• Voon Ong
• Jay Pang
• Jasmin Sajna
• Hannah Thomason
• Yun Wah Wan
• Liz Wragg

Complications of SSc-vasculopathy
• Background:
Raynaud’s phenomenon
Digital ulceration
Critical digital ischaemia
• Consensus best practice pathways for:
Raynaud’s phenomenon
Digital ulceration
Critical digital ischaemia
• The challenges

Raynaud’s phenomenon
Pallor Cyanosis Rubor

Digital ulceration

The scale of the problem
• Of 2080 patients with SSc, 58% reported a
history of DUs
Steen et al. Rheumatology 2009
• Of 1168 patients with SSc, 17% developed
at least one DU over an 18 month period
Nihtyanova et al. Ann Rheum Dis 2008
• DU often occur early in the course of SSc
Hachulla et al. J Rheumatol 2007

Critical digital ischaemia
• A medical emergency

Complications of SSc-vasculopathy
• Background:
Raynaud’s phenomenon
Digital ulceration
Critical digital ischaemia
• Consensus best practice pathways for:
Raynaud’s phenomenon
Digital ulceration
Critical digital ischaemia
• The challenges

1.
Management of Raynaud’s Phenomenon
Establish diagnosis and identify any
underlying cause amenable to treatment
No underlying cause amenable to treatment
Treat underlying cause e.g. cryoglobulinaemia
2.
General/lifestyle measures:
Patient education
- Avoid cold, keep warm
- Stop smoking
(Complementary therapies)
Ineffective
3.
Drug therapy: first line
4.
CCB, ARB, SSRI, alpha blockers, ACE
inhibitors, topical nitrates
+
Antiplatelet and /or statin
therapy
Effective
Ineffective oral therapies/refractory disease
5.
Drug therapy: refractory
IV prostanoid
Effective
6.
Ineffective
PDE5 inhibitor
Effective
Progression to digital ulceration and/or critical ischaemia

Establish the diagnosis
• History
• Examination
• Investigations
(minimal set):
FBC, ESR or CRP
ANA
Capillaroscopy

1.
Management of Raynaud’s Phenomenon
Establish diagnosis and identify any
underlying cause amenable to treatment
No underlying cause amenable to treatment
Treat underlying cause e.g. cryoglobulinaemia
2.
General/lifestyle measures:
Patient education
- Avoid cold, keep warm
- Stop smoking
(Complementary therapies)
Ineffective
3.
Drug therapy: first line
4.
CCB, ARB, SSRI, alpha blockers, ACE
inhibitors, topical nitrates
+
Antiplatelet and /or statin
therapy
Effective
Ineffective oral therapies/refractory disease
5.
Drug therapy: refractory
IV prostanoid
Effective
6.
Ineffective
PDE5 inhibitor
Effective
Progression to digital ulceration and/or critical ischaemia

Complications of SSc-vasculopathy
• Background:
Raynaud’s phenomenon
Digital ulceration
Critical digital ischaemia
• Consensus best practice pathways for:
Raynaud’s phenomenon
Digital ulceration
Critical digital ischaemia
• The challenges

Management of Digital Ulceration
1.
Establish diagnosis early
2.
Treat any contributory cause e.g. infection,
large vessel disease
3.
Optimal wound care and analgesia
4. 5. 6.
Optimise oral vasodilators or
IV prostanoids
Consider surgical debridement in
patients with necrotic tissue or
underlying calcinosis
+ +
Antiplatelet and/or statin
therapy
Ineffective/recurrent ulceration
7.
Repeat IV prostanoids or PDE5 inhibitor or ERA
Ineffective
8.
Consider digital sympathectomy

Treat any contributory cause
• Infection (there may be underlying
osteomyelitis)
• Underlying calcinosis
• Large (proximal) vessel problems
• Vasculitis/coagulopathy
• Smoking
• Exacerbating therapies

Scenario - LcSSc with large vessel disease
• 49 year old
female with
lcSSc (11 years)
• Anticentromere
+ve
• Frequent IV
prostanoid
infusions
• ABPI 0.71
• Vascular
opinion and IV
iloprost
June 2008

Right popliteal angioplasty September 2008

Clinical course post-popliteal angioplasty September 2008
October 2008 and July 2009

Management of Digital Ulceration
1.
Establish diagnosis early
2.
Treat any contributory cause e.g. infection,
large vessel disease
3.
Optimal wound care and analgesia
4. 5. 6.
Optimise oral vasodilators or
IV prostanoids
Consider surgical debridement in
patients with necrotic tissue or
underlying calcinosis
+ +
Antiplatelet and/or statin
therapy
Ineffective/recurrent ulceration
7.
Repeat IV prostanoids or PDE5 inhibitor or ERA
Ineffective
8.
Consider digital sympathectomy

Complications of SSc-vasculopathy
• Background:
Raynaud’s phenomenon
Digital ulceration
Critical digital ischaemia
• Consensus best practice pathways for:
Raynaud’s phenomenon
Digital ulceration
Critical digital ischaemia
• The challenges

Management of Critical Digital Ischaemia
1.
Establish diagnosis and identify any treatable
contributory cause
No contributory cause
2.
Treat any contributory cause
- Large (proximal) vessel disease
-Vasculitis
-Coagulopathy
-Thromboembolism
- Smoking
3. 4. 5.
Admit for IV
Antiplatelet therapy Consider statin 6.
+ +
prostanoid and
+
analgesia
Antibiotic if any
possibility of infection
Ineffective
Effective
7.
Optimise oral vasodilator therapy
(consider PDE5 inhibitor)
8. Consider digital
9. Surgical debridement if necrotic
10.
sympathectomy + tissue
+
Short term anticoagulation

Complications of SSc-vasculopathy
• Background:
Raynaud’s phenomenon
Digital ulceration
Critical digital ischaemia
• Consensus best practice pathways for:
Raynaud’s phenomenon
Digital ulceration
Critical digital ischaemia
• The challenges

Key points + challenges
• Primary RP can be very symptomatic, but does not
progress to tissue injury
• Not all digital ischaemia in patients with SSc is due to
microangiopathy alone
• Digital ulcers may be a medical emergency, critical
ischaemia always is
• Optimal implementation of the recommendations
requires:
Rapid access clinics/’open door’ policy
Availability of drug treatment
Availability of MDT support
• Many questions still unanswered