Raynaud's phenomenon, digital ulcers and critical digital ischaemia
Raynaud's phenomenon, digital ulcers and critical digital ischaemia
Raynaud's phenomenon, digital ulcers and critical digital ischaemia
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Complications of scleroderma<br />
vasculopathy: Raynaud’s <strong>phenomenon</strong>,<br />
<strong>digital</strong> <strong>ulcers</strong> <strong>and</strong> <strong>critical</strong> <strong>digital</strong> <strong>ischaemia</strong><br />
Ariane Herrick
Members <strong>and</strong> contributors to<br />
working group<br />
• Marina Anderson<br />
• Eileen Baildam<br />
• John Coppock<br />
• Chris Denton<br />
• Bridget Griffiths<br />
• Frances Hall<br />
• Jan Lamb<br />
• Anne Mawdsley<br />
• Lindsay Muir<br />
• Pia Moinzadeh<br />
• Tanaka Ngcozana<br />
• Voon Ong<br />
• Jay Pang<br />
• Jasmin Sajna<br />
• Hannah Thomason<br />
• Yun Wah Wan<br />
• Liz Wragg
Complications of SSc-vasculopathy<br />
• Background:<br />
Raynaud’s <strong>phenomenon</strong><br />
Digital ulceration<br />
Critical <strong>digital</strong> <strong>ischaemia</strong><br />
• Consensus best practice pathways for:<br />
Raynaud’s <strong>phenomenon</strong><br />
Digital ulceration<br />
Critical <strong>digital</strong> <strong>ischaemia</strong><br />
• The challenges
Raynaud’s <strong>phenomenon</strong><br />
Pallor Cyanosis Rubor
Digital ulceration
The scale of the problem<br />
• Of 2080 patients with SSc, 58% reported a<br />
history of DUs<br />
Steen et al. Rheumatology 2009<br />
• Of 1168 patients with SSc, 17% developed<br />
at least one DU over an 18 month period<br />
Nihtyanova et al. Ann Rheum Dis 2008<br />
• DU often occur early in the course of SSc<br />
Hachulla et al. J Rheumatol 2007
Critical <strong>digital</strong> <strong>ischaemia</strong><br />
• A medical emergency
Complications of SSc-vasculopathy<br />
• Background:<br />
Raynaud’s <strong>phenomenon</strong><br />
Digital ulceration<br />
Critical <strong>digital</strong> <strong>ischaemia</strong><br />
• Consensus best practice pathways for:<br />
Raynaud’s <strong>phenomenon</strong><br />
Digital ulceration<br />
Critical <strong>digital</strong> <strong>ischaemia</strong><br />
• The challenges
1.<br />
Management of Raynaud’s Phenomenon<br />
Establish diagnosis <strong>and</strong> identify any<br />
underlying cause amenable to treatment<br />
No underlying cause amenable to treatment<br />
Treat underlying cause e.g. cryoglobulinaemia<br />
2.<br />
General/lifestyle measures:<br />
Patient education<br />
- Avoid cold, keep warm<br />
- Stop smoking<br />
(Complementary therapies)<br />
Ineffective<br />
3.<br />
Drug therapy: first line<br />
4.<br />
CCB, ARB, SSRI, alpha blockers, ACE<br />
inhibitors, topical nitrates<br />
+<br />
Antiplatelet <strong>and</strong> /or statin<br />
therapy<br />
Effective<br />
Ineffective oral therapies/refractory disease<br />
5.<br />
Drug therapy: refractory<br />
IV prostanoid<br />
Effective<br />
6.<br />
Ineffective<br />
PDE5 inhibitor<br />
Effective<br />
Progression to <strong>digital</strong> ulceration <strong>and</strong>/or <strong>critical</strong> <strong>ischaemia</strong>
Establish the diagnosis<br />
• History<br />
• Examination<br />
• Investigations<br />
(minimal set):<br />
FBC, ESR or CRP<br />
ANA<br />
Capillaroscopy
1.<br />
Management of Raynaud’s Phenomenon<br />
Establish diagnosis <strong>and</strong> identify any<br />
underlying cause amenable to treatment<br />
No underlying cause amenable to treatment<br />
Treat underlying cause e.g. cryoglobulinaemia<br />
2.<br />
General/lifestyle measures:<br />
Patient education<br />
- Avoid cold, keep warm<br />
- Stop smoking<br />
(Complementary therapies)<br />
Ineffective<br />
3.<br />
Drug therapy: first line<br />
4.<br />
CCB, ARB, SSRI, alpha blockers, ACE<br />
inhibitors, topical nitrates<br />
+<br />
Antiplatelet <strong>and</strong> /or statin<br />
therapy<br />
Effective<br />
Ineffective oral therapies/refractory disease<br />
5.<br />
Drug therapy: refractory<br />
IV prostanoid<br />
Effective<br />
6.<br />
Ineffective<br />
PDE5 inhibitor<br />
Effective<br />
Progression to <strong>digital</strong> ulceration <strong>and</strong>/or <strong>critical</strong> <strong>ischaemia</strong>
Complications of SSc-vasculopathy<br />
• Background:<br />
Raynaud’s <strong>phenomenon</strong><br />
Digital ulceration<br />
Critical <strong>digital</strong> <strong>ischaemia</strong><br />
• Consensus best practice pathways for:<br />
Raynaud’s <strong>phenomenon</strong><br />
Digital ulceration<br />
Critical <strong>digital</strong> <strong>ischaemia</strong><br />
• The challenges
Management of Digital Ulceration<br />
1.<br />
Establish diagnosis early<br />
2.<br />
Treat any contributory cause e.g. infection,<br />
large vessel disease<br />
3.<br />
Optimal wound care <strong>and</strong> analgesia<br />
4. 5. 6.<br />
Optimise oral vasodilators or<br />
IV prostanoids<br />
Consider surgical debridement in<br />
patients with necrotic tissue or<br />
underlying calcinosis<br />
+ +<br />
Antiplatelet <strong>and</strong>/or statin<br />
therapy<br />
Ineffective/recurrent ulceration<br />
7.<br />
Repeat IV prostanoids or PDE5 inhibitor or ERA<br />
Ineffective<br />
8.<br />
Consider <strong>digital</strong> sympathectomy
Treat any contributory cause<br />
• Infection (there may be underlying<br />
osteomyelitis)<br />
• Underlying calcinosis<br />
• Large (proximal) vessel problems<br />
• Vasculitis/coagulopathy<br />
• Smoking<br />
• Exacerbating therapies
Scenario - LcSSc with large vessel disease<br />
• 49 year old<br />
female with<br />
lcSSc (11 years)<br />
• Anticentromere<br />
+ve<br />
• Frequent IV<br />
prostanoid<br />
infusions<br />
• ABPI 0.71<br />
• Vascular<br />
opinion <strong>and</strong> IV<br />
iloprost<br />
June 2008
Right popliteal angioplasty September 2008
Clinical course post-popliteal angioplasty September 2008<br />
October 2008 <strong>and</strong> July 2009
Management of Digital Ulceration<br />
1.<br />
Establish diagnosis early<br />
2.<br />
Treat any contributory cause e.g. infection,<br />
large vessel disease<br />
3.<br />
Optimal wound care <strong>and</strong> analgesia<br />
4. 5. 6.<br />
Optimise oral vasodilators or<br />
IV prostanoids<br />
Consider surgical debridement in<br />
patients with necrotic tissue or<br />
underlying calcinosis<br />
+ +<br />
Antiplatelet <strong>and</strong>/or statin<br />
therapy<br />
Ineffective/recurrent ulceration<br />
7.<br />
Repeat IV prostanoids or PDE5 inhibitor or ERA<br />
Ineffective<br />
8.<br />
Consider <strong>digital</strong> sympathectomy
Complications of SSc-vasculopathy<br />
• Background:<br />
Raynaud’s <strong>phenomenon</strong><br />
Digital ulceration<br />
Critical <strong>digital</strong> <strong>ischaemia</strong><br />
• Consensus best practice pathways for:<br />
Raynaud’s <strong>phenomenon</strong><br />
Digital ulceration<br />
Critical <strong>digital</strong> <strong>ischaemia</strong><br />
• The challenges
Management of Critical Digital Ischaemia<br />
1.<br />
Establish diagnosis <strong>and</strong> identify any treatable<br />
contributory cause<br />
No contributory cause<br />
2.<br />
Treat any contributory cause<br />
- Large (proximal) vessel disease<br />
-Vasculitis<br />
-Coagulopathy<br />
-Thromboembolism<br />
- Smoking<br />
3. 4. 5.<br />
Admit for IV<br />
Antiplatelet therapy Consider statin 6.<br />
+ +<br />
prostanoid <strong>and</strong><br />
+<br />
analgesia<br />
Antibiotic if any<br />
possibility of infection<br />
Ineffective<br />
Effective<br />
7.<br />
Optimise oral vasodilator therapy<br />
(consider PDE5 inhibitor)<br />
8. Consider <strong>digital</strong><br />
9. Surgical debridement if necrotic<br />
10.<br />
sympathectomy + tissue<br />
+<br />
Short term anticoagulation
Complications of SSc-vasculopathy<br />
• Background:<br />
Raynaud’s <strong>phenomenon</strong><br />
Digital ulceration<br />
Critical <strong>digital</strong> <strong>ischaemia</strong><br />
• Consensus best practice pathways for:<br />
Raynaud’s <strong>phenomenon</strong><br />
Digital ulceration<br />
Critical <strong>digital</strong> <strong>ischaemia</strong><br />
• The challenges
Key points + challenges<br />
• Primary RP can be very symptomatic, but does not<br />
progress to tissue injury<br />
• Not all <strong>digital</strong> <strong>ischaemia</strong> in patients with SSc is due to<br />
microangiopathy alone<br />
• Digital <strong>ulcers</strong> may be a medical emergency, <strong>critical</strong><br />
<strong>ischaemia</strong> always is<br />
• Optimal implementation of the recommendations<br />
requires:<br />
Rapid access clinics/’open door’ policy<br />
Availability of drug treatment<br />
Availability of MDT support<br />
• Many questions still unanswered