PMID- 33761724
OWN - NLM
STAT- MEDLINE
DCOM- 20210405
LR  - 20230103
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Print)
IS  - 0025-7974 (Linking)
VI  - 100
IP  - 12
DP  - 2021 Mar 26
TI  - Use of subcutaneous immunoglobulin in stiff person syndrome: Case series.
PG  - e25260
LID - 10.1097/MD.0000000000025260 [doi]
LID - e25260
AB  - INTRODUCTION: Intravenous immunoglobulin (IVIG) has been shown to be effective 
      for the treatment of stiff person syndrome (SPS). However, some patients might 
      not tolerate it. We report the tolerability profile of subcutaneous 
      immunoglobulin (SCIg) in patients with SPS who did not tolerate IVIG. To our 
      knowledge, the use of SCIg in SPS has not been reported before in a case series. 
      PATIENT CONCERNS: The five patients included in this case series presented with 
      various combinations of symptoms of spasms, axial and limb stiffness, and 
      exaggerated responses to outside stimuli. These symptoms often lead to gait and 
      functional impairment. DIAGNOSIS: Patients were diagnosed with classic SPS as 
      they met the clinical criteria, which require the presence of spasms, axial 
      rigidity, and hyperexcitability. INTERVENTIONS: Subcutaneous immunoglobulin 
      infusion. OUTCOMES: Five patients were identified that were treated with SCIg. 
      Three tested positive for serum anti-glutamic acid decarboxylase 65 antibodies 
      prior to any treatment. The mean age at SCIg initiation was 33 years (range: 
      22-47). The mean duration of SPS prior to SCIg initiation was 5.9 years (range: 
      2.5-7). All patients used IVIG for at least two months (up to 18 months) but 
      switched to SCIg due to IVIG side effects. Duration of SCIg use ranged from 
      4 months to 6 years (mean, 19.2 months). Upon switching to SCIg, the SPS symptoms 
      remained stable. SCIg was well-tolerated in most as only one patient discontinued 
      SCIg due to side effects. CONCLUSION: This case series highlights that SCIg could 
      be a treatment option for patients with SPS, especially when IVIG is not 
      feasible. Injection site reactions might be a limiting factor in some patients 
      treated with SCIg. Prospective controlled studies are needed to confirm SCIg 
      treatment durability and efficacy.
CI  - Copyright (c) 2021 the Author(s). Published by Wolters Kluwer Health, Inc.
FAU - Aljarallah, Salman
AU  - Aljarallah S
AD  - King Saud University, College of Medicine, Riyadh, Saudi Arabia.
FAU - Newsome, Scott D
AU  - Newsome SD
AUID- ORCID: 0000-0002-5284-4681
AD  - Johns Hopkins Hospital, Stiff Person Syndrome Center, Baltimore, MD, USA.
LA  - eng
PT  - Journal Article
PT  - Observational Study
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
RN  - 0 (Immunoglobulins)
RN  - 0 (Immunologic Factors)
SB  - IM
MH  - Adult
MH  - Diagnosis, Differential
MH  - Female
MH  - Humans
MH  - Immunoglobulins/*administration & dosage
MH  - Immunologic Factors/administration & dosage
MH  - Infusions, Subcutaneous/*methods
MH  - Male
MH  - Patient Selection
MH  - Retrospective Studies
MH  - *Stiff-Person Syndrome/diagnosis/immunology/physiopathology/therapy
MH  - Treatment Outcome
PMC - PMC9281948
COIS- The authors have no conflicts of interest to disclose.
EDAT- 2021/03/26 06:00
MHDA- 2021/04/07 06:00
PMCR- 2021/03/26
CRDT- 2021/03/25 01:02
PHST- 2020/12/08 00:00 [received]
PHST- 2021/03/04 00:00 [accepted]
PHST- 2021/03/25 01:02 [entrez]
PHST- 2021/03/26 06:00 [pubmed]
PHST- 2021/04/07 06:00 [medline]
PHST- 2021/03/26 00:00 [pmc-release]
AID - 00005792-202103260-00100 [pii]
AID - MD-D-20-12175 [pii]
AID - 10.1097/MD.0000000000025260 [doi]
PST - ppublish
SO  - Medicine (Baltimore). 2021 Mar 26;100(12):e25260. doi: 
      10.1097/MD.0000000000025260.