Pain
Lucy Dong, MD
PGY2
Icahn School of Medicine at Mount Sinai
New York, New York, United States
Lucy Dong, MD
PGY2
Icahn School of Medicine at Mount Sinai
New York City, New York, United States
Amie Kim, MD
Associate Professor Emergency Medicine
Icahn School of Medicine at Mount Sinai
New York City, New York, United States
Lucy Dong, MD
Icahn School of Medicine at Mount Sinai
New York, New York, United States
Diffuse systemic sclerosis complicated by chronic ischemic joint pain and digital auto-amputations.
Case Description:
This case follows a 35-year-old transgender female with SLE and a 18 year history of diffuse systemic sclerosis complicated by auto-amputation of 10 digits. She presented to our clinic with debilitating chronic ischemic pain in multiple joints, significantly impairing her function and quality of life.
Over several years, she trialed numerous pain management strategies including long-acting opioids, multiple regional nerve block trials, adjuvant neuropathic agents including gabapentin and pregabalin, and even outpatient IV ketamine infusions. These interventions provided only transient or partial relief. Her opioid dose reached upwards of 1200mg of oxycodone a day. Given her persistent refractory pain, she was ultimately admitted to the intensive care unit for a PCA pump and IV ketamine infusions.
The patient reported significant improvement in pain levels during the hospitalization. She was subsequently weaned off her PCA pump and was discharged on a regimen of oral ketamine, extended-release opioids, and other adjunctive medications.
Discussions:
Chronic ischemic pain in systemic sclerosis is a multifactorial disease stemming from vascular compromise, neuropathic pain, and fibrosis. Digital auto-amputations are an uncommon but severe complication, often signaling end-stage vascular disease. Pain management is challenging, frequently requiring multimodal and interventional approaches.
Ketamine, an NMDA receptor antagonist, is increasingly recognized for its utility in refractory pain conditions. This case illustrates its potential role in systemic sclerosis-related ischemic pain when conventional therapies fail.
Conclusions:
This case highlights the profound pain burden in systemic sclerosis and explores the use of inpatient ketamine infusion as a rescue therapy in select patients with chronic ischemic pain. Early palliative care involvement and multidisciplinary care remain critical for optimizing outcomes and function.
