Comparison of clinical features between patients with anti-synthetase syndrome and dermatomyositis: results from the MYONET registry

Hum, RM; Lilleker, JB; Lamb, JA; Oldroyd, AGS; Wang, G; Wedderburn, LR; Diederichsen, LP; Schmidt, J; Danieli, MG; Oakley, P; Griger, Z; Nguyen Thi Phuong, T; Kodishala, C; Vazquez-Del Mercado, M; Andersson, H; De Paepe, B; De Bleecker, JL; Maurer, B; McCann, L; Pipitone, N; McHugh, N; New, RP; Ollier, WE; Krogh, NS; Vencovsky, J; Lundberg, IE; Chinoy, H; MYONET Registry

doi:10.1093/rheumatology/kead481

Inflammatory myopathies

Comparison of clinical features between patients with anti-synthetase syndrome and dermatomyositis: results from the MYONET registry

Rheumatology (Oxford)Published 12 Sep 2023Cross-sectional study

An erratum to this article was published (Rheumatology (Oxford), 1 Aug 2024 ↗)

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Authors' conclusion

DM-type rashes are frequent in patients with ASyS; however, distinct clinical manifestations differentiate these patients from classical DM.

Study context

Population: Adults with ASyS or DM from the MYONET registry; DM sine dermatitis or discordant dual autoantibody specificities excluded
Participants: 405 / 649 / 203

Exposure: ASyS vs DM
DMn = 405reference
ASySn = 649
ASyS-DMskinn = 203
Outcome: DM-type rashes, DM vs ASyS-DMskin

Methodological detailsregistry-based (MYONET)

Registry-based (MYONET)

Main finding

As reported in the abstract

Group	Value	Violaceous rash	V-sign	Shawl sign
DM	61%	n = 166, 41%	n = 124, 31%	n = 133, 33%
ASyS-DMskin	44%	n = 57, 9%	n = 28, 4%	n = 18, 3%

Value

DM61%

ASyS-DMskin44%

Violaceous rash

DMn = 166, 41%

ASyS-DMskinn = 57, 9%

V-sign

DMn = 124, 31%

ASyS-DMskinn = 28, 4%

Shawl sign

DMn = 133, 33%

ASyS-DMskinn = 18, 3%

DM vs ASyS-DMskin: all P < 0.005 — heliotrope rash (main values), violaceous rash, V-sign, shawl sign more frequent in DM

Higher frequency of extramuscular manifestations (Mechanic's hands, RP, arthritis, ILD, cardiac involvement) in ASyS-DMskin vs DM: all P < 0.001

Observational data: associations, not effects.

Further findings

Cancer-associated myositis — DM vs ASyS

both P < 0.001

DM17%

ASyS3%

ASyS-DMskin3%

Outcome	P value
Cancer-associated myositisDM vs ASyS-DMskin	both P < 0.001

Parts of this Brief are withheld: the automatic checks could not confirm them against the abstract.

Reference

Authors: Hum RM, Lilleker JB, Lamb JA, Oldroyd AGS, Wang G, Wedderburn LR, et al.
All 28 authors
Hum RM, Lilleker JB, Lamb JA, Oldroyd AGS, Wang G, Wedderburn LR, Diederichsen LP, Schmidt J, Danieli MG, Oakley P, Griger Z, Nguyen Thi Phuong T, Kodishala C, Vazquez-Del Mercado M, Andersson H, De Paepe B, De Bleecker JL, Maurer B, McCann L, Pipitone N, McHugh N, New RP, Ollier WE, Krogh NS, Vencovsky J, Lundberg IE, Chinoy H, MYONET Registry
Journal: Rheumatology (Oxford)
Published: 12 Sep 2023
DOI: 10.1093/rheumatology/kead481
PubMed: 37698987

Topic: Inflammatory myopathies

Exposure

ASyS (with and without DM-type skin involvement) vs DM

Exposure definitions

DM: DM patients with anti-Mi2/-TIF1γ/-NXP2/-SAE/-MDA5 autoantibodies
ASyS: ASyS patients with anti-tRNA synthetase autoantibodies (anti-Jo1/-PL7/-PL12/-OJ/-EJ/-Zo/-KS)

Original publication

Comparison of clinical features between patients with anti-synthetase syndrome and dermatomyositis: results from the MYONET registry. Rheumatology (Oxford). Published 12 September 2023. doi:10.1093/rheumatology/kead481

Read the original article ↗

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