Efficacy of therapeutic plasma exchange for treatment of stiff-person syndrome.

Abstract

BACKGROUND:

The efficacy of therapeutic plasma exchange (TPE) in stiff-person syndrome (SPS) is unclear.

STUDY DESIGN AND METHODS:

A retrospective analysis of patients diagnosed with SPS who underwent TPE and a systematic literature review were conducted.

RESULTS:

Nine patients with the presumptive diagnosis of SPS who underwent TPE were identified. The mean age was 55 years (range, 34-72 years) and 78% (n = 7) were female. Anti-GAD65 was present in 89% (n = 8) of the patients (range, 1.9-40,000 U/mL), and 33% (n = 3) had a history of diabetes. Forty-four percent (n = 4) of patients had previously received immunosuppressive medication and 67% (n = 6) received intravenous immune globulin. The main indication for TPE was worsening of symptoms despite treatment with first-line therapy. Seventy-eight percent of the patients (n = 7) had five TPE procedures. Seventy-eight percent (n = 7) of patients demonstrated at least minimal clinical improvement and 56% (n = 5) had a significant response. Most of the patients who demonstrated a significant response to treatment improved and their symptoms stabilized. Two patients (22%) developed adverse events, including catheter-associated infection and transient hypotension. Eighteen publications were found from the literature review, which resulted in a total of 26 patients diagnosed with SPS. Forty-two percent (n = 11) of patients had a significant symptomatic improvement after TPE treatment, and two patients (8%) developed adverse events.

CONCLUSION:

TPE may benefit patients with SPS who are not responsive to first-line therapy, and it is well tolerated.

• Authors: Monica B Pagano, Beth B Murinson, Aaron A R Tobian, Karen E King
• Journal: Transfusion (Transfusion) Vol. 54 Issue 7 Pg. 1851-6 (Jul 2014) ISSN: 1537-2995 [Electronic] United States
• PMID: 24527774 (Publication Type: Journal Article, Review, Systematic Review)
• Copyright: © 2014 AABB.

Topics

• Adult
• Aged
• Female
• Humans
• Male
• Middle Aged
• Plasma Exchange (adverse effects, statistics & numerical data)
• Prognosis
• Retrospective Studies
• Stiff-Person Syndrome (blood, diagnosis, epidemiology, therapy)
• Treatment Outcome