Anti-Jo1 Syndrome: Understanding a Rare Cause of Interstitial Lung Disease.
anti-Jo1 antibody
anti-synthetase syndrome
idiopathic inflammatory myositis
interstitial lung disease
Journal
Mediterranean journal of rheumatology
ISSN: 2529-198X
Titre abrégé: Mediterr J Rheumatol
Pays: Greece
ID NLM: 101730166
Informations de publication
Date de publication:
Dec 2022
Dec 2022
Historique:
received:
03
12
2021
revised:
14
06
2022
accepted:
30
06
2022
medline:
11
4
2023
entrez:
10
4
2023
pubmed:
11
4
2023
Statut:
epublish
Résumé
Anti-Jo1 syndrome is one of the most common amongst the various anti synthetase syndromes (ASS), which forms a subgroup of the idiopathic inflammatory myositis (IIM). It is characterised by myositis, interstitial lung disease (ILD), fever, Raynaud's phenomenon, and mechanic's hands; associated with the presence of anti-Jo1 antibodies in serum. Being an orphan disease, the clinical diagnosis is often delayed. In this retrospective study, all patients diagnosed as Anti-Jo1 syndrome, from two tertiary care hospitals in Western Maharashtra, between 01 January 2019 - 31 December 2020, were enrolled. The parameters studied included demographic data, clinical features at presentation, laboratory parameters, spirometry, and radiographic findings, along with treatment instituted. A total of 17 patients (8 males, 9 females) qualified for inclusion in the study. The mean age of diagnosis was 40 (±13) years with mean time to diagnosis being 2 years (± 0.6 years), from first clinical presentation. The most common presenting symptoms encountered were arthritis (n = 12, 70.5%), fever (n = 16, 70.5%), myositis (n=11, 64.7%) and breathlessness (n=10, 58.8%).10 patients had ILD at presentation on high resolution computerised tomography of chest (n=10, 58.8%) with restrictive lung defect on spirometry. Six patients required induction of immunosuppression using pulse methylprednisolone (n=6) and Rituximab (n=6), while 11 were managed with oral steroids. Mycophenolate mofetil (n=10) and Azathioprine (n=7) were used as maintenance immunosuppression. Anti-Jo1 syndrome is a myositis syndrome, presenting with a multitude of clinical features. Steroids and disease modifying anti rheumatic drugs form mainstay of therapy.
Sections du résumé
Background
UNASSIGNED
Anti-Jo1 syndrome is one of the most common amongst the various anti synthetase syndromes (ASS), which forms a subgroup of the idiopathic inflammatory myositis (IIM). It is characterised by myositis, interstitial lung disease (ILD), fever, Raynaud's phenomenon, and mechanic's hands; associated with the presence of anti-Jo1 antibodies in serum. Being an orphan disease, the clinical diagnosis is often delayed.
Materials and methods
UNASSIGNED
In this retrospective study, all patients diagnosed as Anti-Jo1 syndrome, from two tertiary care hospitals in Western Maharashtra, between 01 January 2019 - 31 December 2020, were enrolled. The parameters studied included demographic data, clinical features at presentation, laboratory parameters, spirometry, and radiographic findings, along with treatment instituted.
Result
UNASSIGNED
A total of 17 patients (8 males, 9 females) qualified for inclusion in the study. The mean age of diagnosis was 40 (±13) years with mean time to diagnosis being 2 years (± 0.6 years), from first clinical presentation. The most common presenting symptoms encountered were arthritis (n = 12, 70.5%), fever (n = 16, 70.5%), myositis (n=11, 64.7%) and breathlessness (n=10, 58.8%).10 patients had ILD at presentation on high resolution computerised tomography of chest (n=10, 58.8%) with restrictive lung defect on spirometry. Six patients required induction of immunosuppression using pulse methylprednisolone (n=6) and Rituximab (n=6), while 11 were managed with oral steroids. Mycophenolate mofetil (n=10) and Azathioprine (n=7) were used as maintenance immunosuppression.
Conclusion
UNASSIGNED
Anti-Jo1 syndrome is a myositis syndrome, presenting with a multitude of clinical features. Steroids and disease modifying anti rheumatic drugs form mainstay of therapy.
Identifiants
pubmed: 37034360
doi: 10.31138/mjr.33.4.437
pii: MJR-33-4-437
pmc: PMC10075366
doi:
Types de publication
Journal Article
Langues
eng
Pagination
437-443Informations de copyright
© 2022 The Mediterranean Journal of Rheumatology (MJR).
Déclaration de conflit d'intérêts
The authors declare no conflict of interest.
Références
Immunol Res. 2013 Jul;56(2-3):362-70
pubmed: 23572427
Mod Pathol. 2010 Jun;23(6):874-80
pubmed: 20228783
Lung India. 2019 Apr;36(Supplement):S1-S35
pubmed: 31006703
J Clin Rheumatol. 2021 Jun 1;27(4):150-155
pubmed: 31895110
PLoS One. 2015 Nov 05;10(11):e0133702
pubmed: 26539981
Rheumatology (Oxford). 2014 Sep;53(9):1630-8
pubmed: 24706995
Am J Respir Crit Care Med. 2019 Oct 15;200(8):e70-e88
pubmed: 31613151
Chest. 2010 Dec;138(6):1464-74
pubmed: 21138882
Arthritis Rheum. 2002 Dec 15;47(6):614-22
pubmed: 12522835
PLoS One. 2016 May 12;11(5):e0155381
pubmed: 27171228
Rheumatology (Oxford). 2015 Aug;54(8):1420-8
pubmed: 25740830
Am J Ther. 2016 Mar-Apr;23(2):e639-45
pubmed: 25830868
J Rheumatol. 2016 Aug;43(8):1566-74
pubmed: 27252419
Clin Exp Rheumatol. 2016 Sep-Oct;34 Suppl 100(5):181-185
pubmed: 27749242
J Rheumatol. 2013 May;40(5):640-6
pubmed: 23457378
Respir Med. 2011 Aug;105(8):1238-47
pubmed: 21514811
Am J Respir Crit Care Med. 2001 Oct 1;164(7):1182-5
pubmed: 11673206
Ann Med Interne (Paris). 2003 Nov;154(7):483-8
pubmed: 14732841
Medicine (Baltimore). 2015 Aug;94(32):e1144
pubmed: 26266346
Arthritis Care Res (Hoboken). 2013 May;65(5):800-8
pubmed: 23203765
Rheumatology (Oxford). 2016 Jul;55(7):1318-24
pubmed: 27060110
Lancet. 2003 Sep 20;362(9388):971-82
pubmed: 14511932
Clin Pulm Med. 2016 Sep;23(5):218-226
pubmed: 27594777
Respiration. 1983;44(2):143-6
pubmed: 6836189
Lung India. 2016 Jan-Feb;33(1):20-6
pubmed: 26933302
J Scleroderma Relat Disord. 2020 Oct;5(3):178-191
pubmed: 35382516
Am J Respir Crit Care Med. 2002 Jan 15;165(2):277-304
pubmed: 11790668
Eur Respir Rev. 2015 Jun;24(136):216-38
pubmed: 26028634
Respir Med. 2009 Nov;103(11):1719-24
pubmed: 19497723
Clin Rev Allergy Immunol. 2017 Feb;52(1):1-19
pubmed: 26424665
Best Pract Res Clin Rheumatol. 2020 Aug;34(4):101503
pubmed: 32284267
Autoimmun Rev. 2014 Sep;13(9):883-91
pubmed: 24704867
Am J Respir Crit Care Med. 2013 Sep 15;188(6):733-48
pubmed: 24032382
Clin Rheumatol. 2000;19(5):371-7
pubmed: 11055826
Rheumatology (Oxford). 2009 Aug;48(8):968-71
pubmed: 19531628
Curr Rheumatol Rep. 2011 Jun;13(3):175-81
pubmed: 21455765