PMID- 35006287
OWN - NLM
STAT- MEDLINE
DCOM- 20220228
LR  - 20220429
IS  - 1437-160X (Electronic)
IS  - 0172-8172 (Print)
IS  - 0172-8172 (Linking)
VI  - 42
IP  - 2
DP  - 2022 Feb
TI  - Rituximab and intravenous immunoglobulin treatment in PM/Scl antibody-associated 
      disease: case-based review.
PG  - 359-364
LID - 10.1007/s00296-021-05075-z [doi]
AB  - Autoantibodies to the 75-kDa and 100-kDa subunits of the PM/Scl nucleolar protein 
      complex are associated with an overlap syndrome, manifesting with clinical 
      features of systemic sclerosis and idiopathic inflammatory myopathy. We describe 
      the diverse clinical features in a series of 4 cases with anti-PM/Scl-75 and/or 
      anti-PM/Scl-100 antibodies, including severe proximal muscle weakness, 
      oesophageal dysfunction, respiratory weakness requiring mechanical ventilation, 
      Raynaud's, calcinosis cutis, sclerodactyly and critical digital ischaemia. 
      Despite the severity of striated and oesophageal muscle weakness, all patients 
      responded very well to immune suppression, and calcinosis cutis in one case 
      regressed substantially. We highlight the efficacy of Rituximab and intravenous 
      immunoglobulin therapy (IVIg) in these cases, enabling return to normal muscle 
      function within six months. Rituximab was preferentially chosen for cases with 
      hyper-gammaglobulinemia and multiple autoantibodies in addition to anti-PM/Scl, 
      and IVIg was utilised for cases where a rapid onset of effect was required, such 
      as severe ventilator-dependent respiratory muscle weakness and oesophageal 
      dysfunction.
CI  - (c) 2022. The Author(s).
FAU - Srikantharajah, Denesh
AU  - Srikantharajah D
AD  - Department of Rheumatology, St George's University Hospitals NHS Foundation 
      Trust, London, SW17 0QT, UK.
FAU - Lloyd, Mark E
AU  - Lloyd ME
AD  - Department of Rheumatology, Frimley Healthcare NHS Foundation Trust, Frimley, UK.
FAU - Kiely, Patrick D W
AU  - Kiely PDW
AUID- ORCID: 0000-0002-8091-2717
AD  - Department of Rheumatology, St George's University Hospitals NHS Foundation 
      Trust, London, SW17 0QT, UK. patrick.kiely@nhs.net.
AD  - Institute of Medical and Biomedical Education, St George's, University of London, 
      London, UK. patrick.kiely@nhs.net.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Review
DEP - 20220110
PL  - Germany
TA  - Rheumatol Int
JT  - Rheumatology international
JID - 8206885
RN  - 0 (Antirheumatic Agents)
RN  - 0 (Autoantibodies)
RN  - 0 (Immunoglobulins, Intravenous)
RN  - 4F4X42SYQ6 (Rituximab)
SB  - IM
MH  - Adult
MH  - Antirheumatic Agents/*administration & dosage
MH  - Autoantibodies/immunology
MH  - Female
MH  - Humans
MH  - Immunoglobulins, Intravenous/*administration & dosage
MH  - Male
MH  - Middle Aged
MH  - Muscle Weakness/etiology
MH  - Myositis/complications/*drug therapy
MH  - Rituximab/*administration & dosage
MH  - Scleroderma, Systemic/complications/*drug therapy
MH  - Young Adult
PMC - PMC8800925
OTO - NOTNLM
OT  - Intravenous immunoglobulin
OT  - Myositis
OT  - PM/Scl autoantibody
OT  - Raynaud's
OT  - Rituximab
OT  - Systemic sclerosis
COIS- The authors declare that there is no conflict of interest.
EDAT- 2022/01/11 06:00
MHDA- 2022/03/01 06:00
PMCR- 2022/01/10
CRDT- 2022/01/10 12:26
PHST- 2021/10/28 00:00 [received]
PHST- 2021/12/18 00:00 [accepted]
PHST- 2022/01/11 06:00 [pubmed]
PHST- 2022/03/01 06:00 [medline]
PHST- 2022/01/10 12:26 [entrez]
PHST- 2022/01/10 00:00 [pmc-release]
AID - 10.1007/s00296-021-05075-z [pii]
AID - 5075 [pii]
AID - 10.1007/s00296-021-05075-z [doi]
PST - ppublish
SO  - Rheumatol Int. 2022 Feb;42(2):359-364. doi: 10.1007/s00296-021-05075-z. Epub 2022 
      Jan 10.