Fig. 23.1
Bedside examination of nailfold demonstrates enlarged capillary loops and areas of dropout of capillaries typical of scleroderma
In managing this patient it is recognized that she is at risk for vascular lesions including digital ischemic ulcers. She may also be at risk overtime of larger vessel disease in that patients with limited SSc may have an increased risk to develop ulnar artery occlusion. There is also a risk of digital artery disease with digital loss among patients who are anti-centromere positive. However, she has never had any lesions. Her treatment starts with the foundation of non-drug therapy including education to reduce fear and enhance self-care, clear instruction on cold avoidance, avoidance of any potential aggravating drugs (see Chap. 19), and ready access for communication of problems. I prefer to start a calcium channel blocker (CCB) alone in this case and would start with amlodipine at 5 mg. The patient is asked to have access to blood pressure monitoring at home. I would start additional antiplatelet therapy (CCBs do block platelets) with aspirin at 81 mg po daily. An antioxidant is not discouraged but I make no specific recommendation except a healthy diet. The patient is asked to report the degree of benefit or any problems in a 2-week period following starting amlodipine and if doing well with no complications the amlodipine is continued at the low daily dosing. If no benefit and no significant side effects, then the amlodipine is increased to 10 mg daily. Most patients will do well at 5–10 mg and do not benefit from higher dosing. I only move to higher doses (15–20 mg) or combination vasodilator therapy if ischemic lesions occur. The goal is not to eliminate every Raynaud’s event but to reduce the risk of ischemic ulcers or tissue loss and to improve quality of life. Once these goals are accomplished then it is appropriate to continue the same therapy year round and to make sure that the patient calls immediately with any worsening symptoms, signs of digital lesions, or new adverse side effects of the medication.
Case 2: Patient with Scleroderma Digital Ischemic Ulcer
Discussion points—Patient with SSc digital ischemic ulcers
Moving the dose of a sustained-release CCB to the maximum tolerated dose is usually the best option for several reasons: the class of dihydropyridine CCBs are the best studied and are potent peripheral vasodilators; the extended release are safer; there is no definitive data that combination therapy is better or safer.
The phosphodiesterase five inhibitors are popular because it makes biological sense that they would work by increasing nitric oxide in the vasculature. However, the studies show variable benefit and they may not be as potent as the CCB, although studies are required to compare CCBs and phosphodiesterase inhibitors.
Angiotensin-converting enzyme inhibitors are most important in a high renin state as seen in a scleroderma renal crisis; but they do not appear to have an impact in treating RP.
The enthusiasm for botulinum injection at the base of finger for severe RP is growing. However, it is largely based on uncontrolled case series. The biological effect of botulinum is complex and more studies are needed to define its use.
While the use of the endothelin-1 receptor antagonist (ERA) bosentan is approved, one needs to be selective on its use. It has the potential of liver toxicity and reduced new ulcer formation but did not eliminate the risk. In addition, the study of the second-generation ERA macitentan was stopped yet to be reported reasons.
It is often challenging to know if an ulcer is secondarily infected or not. We recommend to treat with systemic antibiotics if there is any indication of infection. We recommend erroring on the use of a statin because it makes biological sense, although it is recognized that more studies are needed to define their role.
A 50-year-old woman with SSc and interstitial lung disease presents with recurrent intense Raynaud’s complicated by digital ulcers despite the use of intense cold avoidance and the use of nifedipine XL at a 30 mg dosage. The examination discloses no evidence of larger vessel disease and a negative Allen’s test excluding significant ulnar artery disease. She has no comorbid risk factors and is a never smoker. The ulcers are distal painful typical of ischemic digital ulcers (Fig. 23.2) and have been reoccurring on the index and middle fingers bilaterally.
Fig. 23.2
Examination of the finger demonstrates a distal ischemic ulcer secondary to peripheral vascular disease of scleroderma
This patient is at risk for the complications of digital ulcers including pain with poor quality of life, loss of hand function, soft tissue infection, and possible loss of involved digits. The underlying disease process is the vascular disease related to SSc. The options for the acute situation for this patient include using combination oral vasodilators, moving to intravenous prostaglandin therapy, combination oral therapy, topical nitrates, local botox injections, or considering surgical options. Given the idea of a practical long-term treatment, I would recommend first moving the nifedipine XL (or amlodipine) to maximum tolerated dose (usually nifedipine XL 90 mg); if on maximum tolerated dose there is no benefit then I would add a PDE5 inhibitor (if available); I use sildenafil at 20 mg po tid; I usually titrate up slowly to the maximum dosing. Some experts use topical nitrates instead of a PDE5 inhibitor. I have not found the addition of an ACE inhibitor to be helpful but have used an ARB (losartan 50 mg) or SSRI (fluoxetine 20 mg) with some benefit (recognizing this strategy is not evidence based) when the PDE5 inhibitor is not available. If the patient does not tolerate (low blood pressure or edema) the enhanced vasodilator therapy or there is no benefit, then a trial of digital botulinum toxin (botox) injections can be tried. The usual tolerable dose of the CCB is continued. While success of botox injections in this setting is reported, it is not yet supported by insurance in the USA and I have been disappointed with long-term outcomes in similar cases.
In patients with recurrent digital ulcers who have not responded to enhanced vasodilator therapy, we move to intravenous prostacyclin. In the USA we use epoprostenol and in Europe iloprost is available. This is given via a peripheral vein at a low-dose infusion of 2 ng/kg/min over 5–6 h daily for 3–5 days, either in an outpatient infusion center or in hospital, depending on local arrangements. I usually stop the CCB or other vasodilators during days of the infusion treatment. This approach of acute administration of intravenous prostacyclin both provides immediate benefit by reducing acute vasospasm and, coupled with restarting and continued use of a daily oral vasodilator, may prevent new ischemic ulcers.
We do not move to surgical options unless the patient has signs of larger vessel disease or deep-tissue ischemia not responding to medical management. Local care of the ulcer is critical for improved outcome (see Chap. 21). We carefully debride ulcers with excess necrotic tissue or if there are signs of secondary sequestered infection. A hydrocolloid dressing with topical antibiotics (mupirocin) or silver-impregnated dressing is used. Any sign of deeper tissue infection is treated with systemic antibiotics that cover for resistant staphylococcal aureus (trimethoprim/sulfamethoxazole). I have a low threshold to use systemic antibiotics due to the complications that can occur in the avascular tissue of SSc. Thus any suggestion of infection and I start systemic antibiotics. Once the ulcer appears clear with good granulation tissue then I move to light dressing with Vaseline or as it becomes small and contracts I move to no dressing; allowing the small ulcer to crust over, dry, and heal with time. Ulcers are painful and adequate analgesia is required. Pain isolated to the bed of the ulcer is expected. However, pain beyond the ulcer, extending into the proximal finger, suggests continued ischemia and the threat of a deep injury or spreading infection.
I manage pain first with topical care. Often covering the ulcer with Vaseline will reduce pain if the ulcer itself is the sole source of pain. The use of a nonsteroidal anti-inflammatory drug (ibuprofen) alone or with acetaminophen is our first approach. Opiates at low dose (oxycodone 5 mg every 6 h) may be needed. Topical lidocaine or lidocaine/prilocaine use on the finger outside of the ulcer bed is helpful to some patients. Keeping the ulcer protected from trauma and the involved finger(s) warm is very important. We will have the patient stay home away from a work situation that may cause finger trauma (typing) or undue cold exposure, at least until the acute phase is over. One can tell if the acute phase is over when the patient has well-controlled pain, no signs of current ulcer worsening, and no new ulcers emerging.
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