PMID- 34643702
OWN - NLM
STAT- MEDLINE
DCOM- 20220225
LR  - 20220225
IS  - 1554-6578 (Electronic)
IS  - 0022-3069 (Linking)
VI  - 80
IP  - 11
DP  - 2021 Nov 19
TI  - Diagnosis and Clinical Development of Sporadic Inclusion Body Myositis and 
      Polymyositis With Mitochondrial Pathology: A Single-Center Retrospective 
      Analysis.
PG  - 1060-1067
LID - 10.1093/jnen/nlab101 [doi]
AB  - To review our diagnostic and treatment approaches concerning sporadic inclusion 
      body myositis (sIBM) and polymyositis with mitochondrial pathology (PM-Mito), we 
      conducted a retrospective analysis of clinical and histological data of 32 
      patients diagnosed as sIBM and 7 patients diagnosed as PM-Mito by muscle biopsy. 
      Of 32 patients identified histologically as sIBM, 19 fulfilled the 2011 European 
      Neuromuscular Center (ENMC) diagnostic criteria for "clinico-pathologically 
      defined sIBM" at the time of biopsy. Among these, 2 patients developed sIBM after 
      years of immunosuppressive treatment for organ transplantation. Of 11 patients 
      fulfilling the histological but not the clinical criteria, including 3 cases with 
      duration <12 months, 8 later fulfilled the criteria for clinico-pathologically 
      defined sIBM. Of 7 PM-Mito patients, 4 received immunosuppression with clinical 
      improvement in 3. One of these later developed clinico-pathologically defined 
      sIBM; 1 untreated patient progressed to clinically defined sIBM. Thus, muscle 
      histology remains important for this differential diagnosis to identify sIBM 
      patients not matching the ENMC criteria and the PM-Mito group. In the latter, we 
      report at least 50% positive, if occasionally transient, response to 
      immunosuppressive treatments and progression to sIBM in a minority. The 
      mitochondrial abnormalities defining PM-Mito do not seem to define the threshold 
      to immunosuppression unresponsiveness.
CI  - (c) 2021 American Association of Neuropathologists, Inc.
FAU - Winkler, Maren
AU  - Winkler M
AD  - From the Section of Neuromuscular Diseases, Department of Neurology, University 
      Hospital of Bonn, Bonn, Germany (MW, CvL, KK-H, CK, JR); Group Practice for 
      Neurology, Bonn, Germany (SN); Center for Rare Diseases, University Hospital of 
      Bonn, Bonn, Germany (CK).
FAU - von Landenberg, Christina
AU  - von Landenberg C
AD  - From the Section of Neuromuscular Diseases, Department of Neurology, University 
      Hospital of Bonn, Bonn, Germany (MW, CvL, KK-H, CK, JR); Group Practice for 
      Neurology, Bonn, Germany (SN); Center for Rare Diseases, University Hospital of 
      Bonn, Bonn, Germany (CK).
FAU - Kappes-Horn, Karin
AU  - Kappes-Horn K
AD  - From the Section of Neuromuscular Diseases, Department of Neurology, University 
      Hospital of Bonn, Bonn, Germany (MW, CvL, KK-H, CK, JR); Group Practice for 
      Neurology, Bonn, Germany (SN); Center for Rare Diseases, University Hospital of 
      Bonn, Bonn, Germany (CK).
FAU - Neudecker, Stephan
AU  - Neudecker S
AD  - From the Section of Neuromuscular Diseases, Department of Neurology, University 
      Hospital of Bonn, Bonn, Germany (MW, CvL, KK-H, CK, JR); Group Practice for 
      Neurology, Bonn, Germany (SN); Center for Rare Diseases, University Hospital of 
      Bonn, Bonn, Germany (CK).
FAU - Kornblum, Cornelia
AU  - Kornblum C
AUID- ORCID: 0000-0002-0111-7281
AD  - From the Section of Neuromuscular Diseases, Department of Neurology, University 
      Hospital of Bonn, Bonn, Germany (MW, CvL, KK-H, CK, JR); Group Practice for 
      Neurology, Bonn, Germany (SN); Center for Rare Diseases, University Hospital of 
      Bonn, Bonn, Germany (CK).
FAU - Reimann, Jens
AU  - Reimann J
AUID- ORCID: 0000-0003-3349-6877
AD  - From the Section of Neuromuscular Diseases, Department of Neurology, University 
      Hospital of Bonn, Bonn, Germany (MW, CvL, KK-H, CK, JR); Group Practice for 
      Neurology, Bonn, Germany (SN); Center for Rare Diseases, University Hospital of 
      Bonn, Bonn, Germany (CK).
LA  - eng
PT  - Journal Article
DEP - 20211013
PL  - England
TA  - J Neuropathol Exp Neurol
JT  - Journal of neuropathology and experimental neurology
JID - 2985192R
RN  - 0 (Immunosuppressive Agents)
SB  - IM
MH  - Aged
MH  - Biopsy
MH  - Diagnosis, Differential
MH  - Disease Progression
MH  - Female
MH  - Humans
MH  - Immunosuppressive Agents/therapeutic use
MH  - Male
MH  - Middle Aged
MH  - Mitochondria/*pathology
MH  - Muscle, Skeletal/pathology
MH  - Myositis, Inclusion Body/*diagnosis/drug therapy/*pathology
MH  - Organ Transplantation
MH  - Polymyositis/drug therapy/*pathology
MH  - Retrospective Studies
OTO - NOTNLM
OT  - Diagnosis
OT  - Differential
OT  - Immunosuppression
OT  - Inclusion body
OT  - Mitochondria
OT  - Myositis
OT  - Polymyositis
EDAT- 2021/10/14 06:00
MHDA- 2022/02/26 06:00
CRDT- 2021/10/13 12:22
PHST- 2021/10/13 12:22 [entrez]
PHST- 2021/10/14 06:00 [pubmed]
PHST- 2022/02/26 06:00 [medline]
AID - 6395280 [pii]
AID - 10.1093/jnen/nlab101 [doi]
PST - ppublish
SO  - J Neuropathol Exp Neurol. 2021 Nov 19;80(11):1060-1067. doi: 
      10.1093/jnen/nlab101. Epub 2021 Oct 13.