Table 1 Open in new tab Meta-analysis...

Domain

Factor

RR/WMD (95% CI)

GRADE certainty rating^a

Heterogeneity

Egger’s test, P-value

P-value

I², %

Subtype^b

2.21 (1.78, 2.77)

Moderate

0.134

24.8

0.162

0.49 (0.37, 0.65)

Moderate

0.061

34.0

0.603

CADM

0.44 (0.20, 0.97)

Low

0.751

0.0

—

ASS

0.28 (0.00, 6554.79)

Low

0.017

82.4

—

Demographics

Age

11.19 (9.29, 13.08)

High

0.001

56.1

0.859

Male sex

1.53 (1.34, 1.75)

High

0.101

24.2

0.081

Clinical features

Dysphagia

2.09 (1.21, 3.60)

High

<0.0001

83.6

0.310

Cutaneous ulceration

2.73 (1.33, 5.59)

Moderate

0.389

3.1

—

Raynaud’s phenomenon

0.61 (0.39, 0.95)

Moderate

0.698

0.0

0.055

ILD

0.49 (0.32, 0.76)

High

0.011

52.3

0.381

Blood parameters

−1189.96 (−2132.74, −247.18)

Moderate

<0.0001

84.5

0.182

LDH

−336.52 (−514.40, −158.64)

Moderate

0.093

44.8

—

ALT

36.29 (−313.18, 385.77)

Low

0.001

85.5

—

ESR

4.4 (−6.2, 14.9)

Low

0.028

60.2

—

Autoantibodies

Anti-TIF1γ

4.68 (3.37, 6.48)

High

<0.0001

68.8

0.543

Anti-NXP2

1.16 (0.73, 1.87)

Moderate

0.278

17.4

0.271

Anti-c

1.59 (0.33, 7.74)

Low

0.217

34.5

—

Anti-HMGCR

0.55 (0.19, 1.61)

Low

0.277

21.6

—

Anti-MDA5

0.17 (0.02, 1.28)

Low

0.457

0.0

—

Anti-Mi2

1.05 (0.28, 3.92)

Low

0.381

5.6

—

Anti-SRP

0.40 (0.14, 1.21)

Low

0.790

0.0

—

Any ASS antibody

0.41 (0.26, 0.64)

High

0.746

0.0

<0.001

Anti-Jo1

0.45 (0.25, 0.84)

High

0.700

0.0

0.051

Anti-PL7

0.68 (0.15, 3.07)

Low

0.541

0.0

—

Anti-PL12

1.59 (0.89, 2.86)

Low

0.789

0.0

—

Anti-EJ

0.17 (0.07, 0.44)

Low

0.964

0.0

—

Anti-OJ

1.56 (0.68, 3.52)

Low

0.870

0.0

—

Anti-KS

1.23 (0.05, 30.12)

Very low

0.717

0.0

—

MSA negative

0.89 (0.50, 1.59)

Low

0.073

50.4

—

ANA positivity

0.91 (0.58, 1.41)

Low

<0.0001

75.0

0.113

Table 1

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Meta-analysis results, including calculated effect sizes, heterogeneity and publication bias for each factor

Domain	Factor	RR/WMD (95% CI)	GRADE certainty rating^a	Heterogeneity		Egger’s test, P-value
P-value	I², %
Subtype^b	DM	2.21 (1.78, 2.77)	Moderate	0.134	24.8	0.162
	PM	0.49 (0.37, 0.65)	Moderate	0.061	34.0	0.603
	CADM	0.44 (0.20, 0.97)	Low	0.751	0.0	—
	ASS	0.28 (0.00, 6554.79)	Low	0.017	82.4	—
Demographics	Age	11.19 (9.29, 13.08)	High	0.001	56.1	0.859
Demographics	Male sex	1.53 (1.34, 1.75)	High	0.101	24.2	0.081
Clinical features	Dysphagia	2.09 (1.21, 3.60)	High	<0.0001	83.6	0.310
	Cutaneous ulceration	2.73 (1.33, 5.59)	Moderate	0.389	3.1	—
	Raynaud’s phenomenon	0.61 (0.39, 0.95)	Moderate	0.698	0.0	0.055
	ILD	0.49 (0.32, 0.76)	High	0.011	52.3	0.381
Blood parameters	CK	−1189.96 (−2132.74, −247.18)	Moderate	<0.0001	84.5	0.182
	LDH	−336.52 (−514.40, −158.64)	Moderate	0.093	44.8	—
	ALT	36.29 (−313.18, 385.77)	Low	0.001	85.5	—
	ESR	4.4 (−6.2, 14.9)	Low	0.028	60.2	—
Autoantibodies	Anti-TIF1γ	4.68 (3.37, 6.48)	High	<0.0001	68.8	0.543
	Anti-NXP2	1.16 (0.73, 1.87)	Moderate	0.278	17.4	0.271
	Anti-c	1.59 (0.33, 7.74)	Low	0.217	34.5	—
	Anti-HMGCR	0.55 (0.19, 1.61)	Low	0.277	21.6	—
	Anti-MDA5	0.17 (0.02, 1.28)	Low	0.457	0.0	—
	Anti-Mi2	1.05 (0.28, 3.92)	Low	0.381	5.6	—
	Anti-SRP	0.40 (0.14, 1.21)	Low	0.790	0.0	—
	Any ASS antibody	0.41 (0.26, 0.64)	High	0.746	0.0	<0.001
	Anti-Jo1	0.45 (0.25, 0.84)	High	0.700	0.0	0.051
	Anti-PL7	0.68 (0.15, 3.07)	Low	0.541	0.0	—
	Anti-PL12	1.59 (0.89, 2.86)	Low	0.789	0.0	—
	Anti-EJ	0.17 (0.07, 0.44)	Low	0.964	0.0	—
	Anti-OJ	1.56 (0.68, 3.52)	Low	0.870	0.0	—
	Anti-KS	1.23 (0.05, 30.12)	Very low	0.717	0.0	—
	MSA negative	0.89 (0.50, 1.59)	Low	0.073	50.4	—
	ANA positivity	0.91 (0.58, 1.41)	Low	<0.0001	75.0	0.113

Domain	Factor	RR/WMD (95% CI)	GRADE certainty rating^a	Heterogeneity		Egger’s test, P-value
P-value	I², %
Subtype^b	DM	2.21 (1.78, 2.77)	Moderate	0.134	24.8	0.162
	PM	0.49 (0.37, 0.65)	Moderate	0.061	34.0	0.603
	CADM	0.44 (0.20, 0.97)	Low	0.751	0.0	—
	ASS	0.28 (0.00, 6554.79)	Low	0.017	82.4	—
Demographics	Age	11.19 (9.29, 13.08)	High	0.001	56.1	0.859
Demographics	Male sex	1.53 (1.34, 1.75)	High	0.101	24.2	0.081
Clinical features	Dysphagia	2.09 (1.21, 3.60)	High	<0.0001	83.6	0.310
	Cutaneous ulceration	2.73 (1.33, 5.59)	Moderate	0.389	3.1	—
	Raynaud’s phenomenon	0.61 (0.39, 0.95)	Moderate	0.698	0.0	0.055
	ILD	0.49 (0.32, 0.76)	High	0.011	52.3	0.381
Blood parameters	CK	−1189.96 (−2132.74, −247.18)	Moderate	<0.0001	84.5	0.182
	LDH	−336.52 (−514.40, −158.64)	Moderate	0.093	44.8	—
	ALT	36.29 (−313.18, 385.77)	Low	0.001	85.5	—
	ESR	4.4 (−6.2, 14.9)	Low	0.028	60.2	—
Autoantibodies	Anti-TIF1γ	4.68 (3.37, 6.48)	High	<0.0001	68.8	0.543
	Anti-NXP2	1.16 (0.73, 1.87)	Moderate	0.278	17.4	0.271
	Anti-c	1.59 (0.33, 7.74)	Low	0.217	34.5	—
	Anti-HMGCR	0.55 (0.19, 1.61)	Low	0.277	21.6	—
	Anti-MDA5	0.17 (0.02, 1.28)	Low	0.457	0.0	—
	Anti-Mi2	1.05 (0.28, 3.92)	Low	0.381	5.6	—
	Anti-SRP	0.40 (0.14, 1.21)	Low	0.790	0.0	—
	Any ASS antibody	0.41 (0.26, 0.64)	High	0.746	0.0	<0.001
	Anti-Jo1	0.45 (0.25, 0.84)	High	0.700	0.0	0.051
	Anti-PL7	0.68 (0.15, 3.07)	Low	0.541	0.0	—
	Anti-PL12	1.59 (0.89, 2.86)	Low	0.789	0.0	—
	Anti-EJ	0.17 (0.07, 0.44)	Low	0.964	0.0	—
	Anti-OJ	1.56 (0.68, 3.52)	Low	0.870	0.0	—
	Anti-KS	1.23 (0.05, 30.12)	Very low	0.717	0.0	—
	MSA negative	0.89 (0.50, 1.59)	Low	0.073	50.4	—
	ANA positivity	0.91 (0.58, 1.41)	Low	<0.0001	75.0	0.113

Grading of Recommendations, Assessment, Development and Evaluations certainty rating: very low (the true effect is probably markedly different from the estimated effect), low (the true effect might be markedly different from the estimated effect), moderate (the authors believe that the true effect is probably close to the estimated effect), and high (the authors have a lot of confidence that the true effect is similar to the estimated effect). ^bThe risk of cancer for each IIM subtype is estimated against each study’s wider IIM population, not the general population. ALT: alanine transaminase; ASS: anti-synthetase syndrome; CADM: clinically amyopathic dermatomyositis; CI: confidence interval; CK: creatine kinase; DM: dermatomyositis; HMGCR: 3-hydroxy 3-methylutaryl coenzyme A reductase; ILD: interstitial lung disease; LDH: lactate dehydrogenase; MDA5: melanoma differentiation-associated gene 5; MSA: myositis specific autoantibody; NXP2: nuclear matrix protein 2; RR: risk ratio; SAE1: small ubiquitin-like modifier-1 activating enzyme; SRP: signal recognition particle; TIF1γ: transcriptional intermediary factor-1 gamma; WMD: weighted mean difference.

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