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Efficacy of therapeutic plasma exchange for treatment of stiff‐person syndrome
Author(s) -
Pagano Monica B.,
Murinson Beth B.,
Tobian Aaron A.R.,
King Karen E.
Publication year - 2014
Publication title -
transfusion
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.045
H-Index - 132
eISSN - 1537-2995
pISSN - 0041-1132
DOI - 10.1111/trf.12573
Subject(s) - medicine , adverse effect , retrospective cohort study , diabetes mellitus , therapeutic plasma exchange , surgery , endocrinology
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‐ GAD 65 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.