Progressive systemic sclerosis: intrathecal pain management

At present, there is no reliable method for long-term treatment of severe pain from progressive systemic sclerosis (PSS) associated with Raynaud's phenomena leading to ischemia and ulcerations of the extremities. Long-term continuous intrathecal (IT) buprenorphine/bupivacaine analgesia was used in such a case.

The patient was a 71-year-old woman in whom conservative treatment, including opioids, dorsal column stimulation, and epidural bupivacaine, had failed to provide satisfactory, long-term relief for her severe lower extremity pain. An 18-gauge Portex intrathecal catheter was inserted via the L4-L5 interspace. An infusion of 4.75 mg/mL bupivacaine and 0.015 mg/mL buprenorphine was started from a portable Pharmacia-Deltec (St. Paul, MN) pump at a rate of 0.1 mL/h with optional bolus doses of 0.1 mL, 2-4 times/h by patient controlled intrathecal analgesia. The rate was adjusted to give the patient satisfactory (80-100%) pain relief.

The IT treatment was continued with 0.1 mL (approximately 0.5 mg/h bupivacaine) for most of the time (mean dose = 18.6 mg/d). This treatment gave the patient 90-100% pain relief, which subsequently improved the quality of her life. Nocturnal sleep duration increased from 2 hours before to 7-8 hours during the IT treatment. The treatment was complicated by transient post-dural puncture headache and further by meningitis, successfully treated with parenteral and intrathecal antibiotics. Every attempt to increase the IT bupivacaine to >20 mg/d led to transient urinary retention, paresthesia, and reduced motor strength in the lower extremities accompanied by gait disturbances. The IT treatment lasted for 861 days (of which 580 days were spent at home), until the patient's death, not related to the treatment.

Intrathecal infusion of buprenorphine/bupivacaine provided satisfactory long-term pain relief in a patient with PSS-associated Raynaud's phenomena, skin ulcerations, and intractable ischemic pain.