What is the treatment for Raynaud's (Raynaud's disease) with gangrene?

From the Guidelines

Raynaud's phenomenon with gangrene requires immediate medical attention, and treatment should prioritize vasodilator medications, such as calcium channel blockers (nifedipine 30-60mg daily), as the first-line treatment, as supported by the most recent evidence 1.

Key Considerations

• The condition represents a severe complication where tissue death occurs due to prolonged lack of blood flow, necessitating prompt intervention to prevent further tissue loss and manage the underlying condition.
• Surgical intervention, including debridement of necrotic tissue or, in severe cases, amputation of the affected digit, may be necessary for established gangrene.
• Pain management with appropriate analgesics is essential, and patients should immediately stop smoking, keep affected areas warm, and avoid cold exposure and vasoconstrictive medications.
• Underlying conditions causing secondary Raynaud's, such as scleroderma or lupus, must be treated concurrently.

Management Approach

• The use of endovascular procedures to establish in-line blood flow to the foot is recommended for patients with nonhealing wounds or gangrene, as per the 2016 AHA/ACC guideline on the management of patients with lower extremity peripheral artery disease 1.
• Angiosome-directed endovascular therapy may be reasonable for patients with CLI and nonhealing wounds or gangrene, although the quality of evidence is low, and randomized data are needed to confirm its efficacy.
• Early and extensive initial surgical debridement in cases of gangrene, such as Fournier’s gangrene, improves survival, and consideration for fecal diversion may be necessary in cases with fecal contamination 1.

Lifestyle Modifications

• Patients should avoid known Raynaud phenomenon triggers, such as cold, trauma, stress, smoking, vibration injury, or certain drugs (e.g., bleomycin, clonidine, and ergot alkaloids), as suggested by expert opinion 1.
• Wearing proper (warm) clothing in cold conditions, such as a coat, mittens, hat, dry insulated footwear, and hand and/or foot warmers, is recommended to prevent the onset of symptoms.

From the FDA Drug Label

Adverse events reported with the use of intravenous iloprost in patients with frostbite from the published literature include headache, flushing, palpitations/tachycardia, nausea, vomiting, dizziness, and hypotension Pre-marketing safety data on AURLUMYN were obtained from 116 patients with Systemic Sclerosis receiving iloprost in 2 multicenter, double-blind, randomized, placebo-controlled studies in patients with Systemic Sclerosis experiencing symptomatic digital ischemic episodes (Raynaud's Phenomenon)

The FDA drug label does mention Raynaud's Phenomenon as an indication for the use of iloprost (IV) in patients with Systemic Sclerosis, but it does not directly address Raynaud's with gangrene. However, it does mention that iloprost is used in patients with symptomatic digital ischemic episodes, which may be related to gangrene.

• The label reports adverse events in patients with frostbite and Systemic Sclerosis, but does not provide information on the specific use of iloprost in patients with Raynaud's and gangrene.
• The safety profile of iloprost in patients with Systemic Sclerosis was similar to that observed with IV iloprost 2.
• No conclusion can be drawn about the use of iloprost in patients with Raynaud's and gangrene based on the available information.

From the Research

Definition and Classification of Raynaud's Phenomenon

• Raynaud's phenomenon (RP) is a vasospastic disorder characterized by episodic color changes of blanching, cyanosis, and hyperemia in response to cold and/or emotional stress 3.
• RP can be primary or secondary, with primary RP being more common in women and having a prevalence estimate ranging from 4% to 30% 3.
• Secondary RP is characterized by an age of onset of more than 30 years, painful and asymmetric attacks, ischemic skin lesions, positive autoantibodies, capillaroscopic abnormalities, and/or clinical features suggestive of connective tissue diseases (CTDs) 3.

Association with Gangrene

• Secondary RP can develop persistent digital ischemia, including ulcers and gangrene, especially in patients with systemic sclerosis 4.
• Gangrene is a severe complication of RP, and its management requires a comprehensive clinical assessment and investigation, including the detection of autoantibodies and nailfold capillaroscopic abnormalities 4.

Treatment Options

• Calcium-channel blockers, such as nifedipine and diltiazem, are effective in treating RP, especially in patients without underlying vascular disease 5.
• Other treatment options include sympatholytic agents, angiotensin II-receptor type I antagonists, and selective serotonin-reuptake inhibitors 3.
• Non-pharmacological management, such as abstinence from smoking and offending drugs, and avoidance of abrupt changes in temperature, is also important 3.

Management of Gangrene

• The management of gangrene in RP patients requires a multidisciplinary approach, including surgical intervention and pharmacological treatment 4.
• The use of prostaglandins has been established in severe cases, but standard therapeutic protocols are not yet available 3.
• Further research is needed to understand the complex pathogenesis of RP and to develop optimized approaches to management, especially in patients with gangrene 4.