Efficacy of therapeutic plasma exchange for treatment of stiff‐person syndrome

BackgroundThe efficacy of therapeutic plasma exchange (TPE) in stiff‐person syndrome (SPS) is unclear.Study Design and MethodsA retrospective analysis of patients diagnosed with SPS who underwent TPE and a systematic literature review were conducted.ResultsNine 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.ConclusionTPE may benefit patients with SPS who are not responsive to first‐line therapy, and it is well tolerated.