Successful rituximab treatment for severe rapidly progressive interstitial lung disease with anti-MDA5 antibody-positive juvenile dermatomyositis: a case report and literature review

Nishi, Kentaro; Ogura, Masao; Tamai, Naotaka; Gima, Masafumi; Ide, Kentaro; Koinuma, Goro; Kamei, Koichi; Ito, Shuichi

doi:10.1186/s12969-022-00723-5

Pediatric Rheumatology

Table 1 Literature review of 18 cases of RP-ILD with JDM

From: Successful rituximab treatment for severe rapidly progressive interstitial lung disease with anti-MDA5 antibody-positive juvenile dermatomyositis: a case report and literature review

Reference	Age/sex/race	Clinical symptom of JDM				Interval between onset of JDM and diagnosis of ILD	Interval between diagnosis of JDM and diagnosis of ILD	Clinical presentation of RP-ILD	Blood test
Reference	Age/sex/race	Fever	Rash	Arthralgia	Muscle weakness	Interval between onset of JDM and diagnosis of ILD	Interval between diagnosis of JDM and diagnosis of ILD	Clinical presentation of RP-ILD	AST/ALT/LDH/CK/ALD/KL-6	Antibody (level or index)
Tosun et al. [9]	10/M/Turkish	+	+	+	+	5 weeks	2 weeks	dyspnea	240/318/3545/437/NA/NA	ND
Zulian et al. [10]	3/F/Italy	+	+	+	+	8 months	7 months	cough, dyspnea	334/NA/3,640/403/NA/NA	ND
Nonaka et al. [3, 11]	1/F/Japanese	+	+	+	+	5 months	2 months	−	178/69/1658/116/18.4/2850	Anti-Jo-1 (12.8)
Nagai et al. [12]	5/F/Japanese	+	+	−	+	1 month	2 day	cough, dyspnea	256/169/649/40/32.9/3,173	ND
Sakurai et al. [13]	9/M/Japanese	+	+	−	+	6 months	2 months	ND	207/101/564/315/16.7/5138	Anti-MDA5 (NA)
Kobayashi et al. [3, 8, 13, 15, 16]	14/F/Japanese	+	+	ND	+	ND	1 month	mild dyspnea	244/ND/ND/1435/20.5/1695	Anti-MDA5^a
	7/M/Japanese	+	+	−	−	ND	2 months	cough, dyspnea	71/38/647/1250/9.6/2460	Anti-MDA5^a
	7/M/Japanese	−	+	ND	+	ND	1 month	−	228/ND/ND/348/20.0/2376	Anti-MDA5^a
Ishikawa et al. [3, 17]	10/M/Japanese	+	−	+	+	2 months	1 month	cough or dyspnea	302/275/463/405/18.5/1012	ND
Sato et al. [18]	7/M/Japanese	+	ND	ND	ND	ND	ND	ND	ND/ND/ND/ND/ND/ND
	2/F/Japanese	+	ND	ND	ND	ND	ND	ND	ND/ND/ND/ND/ND/ND	ND^b
	2/F/Japanese	+	ND	ND	ND	ND	ND	ND	ND/ND/ND/ND/ND/ND
Kobayashi et al. [3]	4/F/Japanese	+	+	ND	−	3 months	ND	cough or dyspnea	ND/ND/ND/ND/ND/ND	Anti-MDA5 (ND)
	6/F/Japanese	+	+	ND	+	5 months	ND	cough or dyspnea	ND/ND/ND/ND/ND/ND	Anti-MDA5 (ND)
	4/F/Japanese	+	+	ND	−	6 months	ND	cough or dyspnea	ND/ND/ND/ND/ND/ND	Anti-MDA5 (ND)
Hou et al. [19]	10/F/Chinese	+	ND	ND	+	2 months	ND	−	173/ND/419/588/ND/ND	Anti-MDA5 (ND)
Yeung et al. [6]	16/F/Chinese	+	+	+	−	2 months	1 month	cough	ND/64/407/858/ND/ND	Anti-MDA5 (ND)
Present case	2/F/Japanese	+	+	−	+	3 months	0 day	cough, dyspnea	69/49/576/22/13.5/3420	Anti-MDA5 (> 7500)

Reference	Chest CT findings	Myositis on femoral MRI	Respiratory support			Treatment	Outcome	Duration from of RP-ILD onset to death
Reference	Chest CT findings	Myositis on femoral MRI	O2	CMV	ECMO	Treatment	Outcome	Duration from of RP-ILD onset to death
Tosun et al. [9]	CABB	ND	ND	ND	ND	PSL, MPT, IVCY	Death	7 weeks
Zulian et al. [10]	CABB, GGO, SCS	ND	+	+	+	PSL, MPT, CPM	Alive	−
Nonaka et al. [3, 11]	CABB, SCS	+	+	−	−	MPT, PSL, CyA, IVIG	Alive	−
Nagai et al. [12]	CABB, GGO, TB, SCS	+	ND	ND	ND	PSL, MPT, CyA, IVCY, IVIG	Death	4 weeks
Sakurai et al. [13]	Pleural effusion	+	+	+	−	mPSL, MPT, CyA, IVCY, PE	Death	3 weeks
Kobayashi et al. [3, 8, 13, 15, 16]	GGO, SCS	ND	−	−	−	PSL, MPT, CyA, IVIG	Alive	−
	GGO	ND	+	ND	ND	PSL, MPT, AZP, CyA	Death	1 month
	GGO, SCS	ND	ND	ND	ND	PSL, MPT, CyA	Alive	−
Ishikawa et al. [3, 17]	CABB, GGO, SCS	+	+	+	−	PSL, MPT, CyA, IVIG	Death	7 weeks
Sato et al. [18]	GGO	ND	ND	ND	ND	PSL, MPT, CyA, IVIG	Death	< 4 months
	GGO	ND	ND	ND	ND	PSL, MPT, CyA, IVCY, IVIG	Death	< 4 months
	GGO	ND	ND	ND	ND	PSL, MPT, CyA, IVCY, IVIG	Death	< 4 months
Kobayashi et al. [3]	SCS	ND	ND	ND	ND	ND	Death	2 months
	SCS	ND	ND	ND	ND	ND	Death	2 months
	CABB, GGO, TB, SCS	ND	ND	ND	ND	ND	Death	1 month
Hou et al. [19]	CABB, GGO, SCS	ND	ND	ND	ND	mPSL, IVCY, IVIG	Death	2 months
Yeung et al. [6]	CABB, SCS	+	+	−	−	PSL, MPT, Tac, MMF, MTX, IVIG, IVCY, RTX	Alive	−
Present case	CABB, GGO, TB, SCS	+	+	+	+	PSL, MPT, CyA, Tac, MMF, IVCY, IVIG, PE, RTX	Alive	−

M Male, F Female, JDM Juvenile dermatomyositis, ILD Interstitial lung disease, RP-ILD Rapidly progressive interstitial lung disease, CT Computed tomography, CABB Consolidation around bronchovascular bundles, SCS Subpleural curvilinear shadow, GGO Ground-glass opacity, TB Traction bronchiectasis, MRI Magnetic resonance imaging, O2 Oxygen, CMV Continuous mandatory ventilation, ECMO Extracorporeal membrane oxygenation, PSL Prednisolone, MPT Methylprednisolone pulse therapy, IVCY Intravenous cyclophosphamide, CPM Cyclophosphamide, CyA Cyclosporine A, IVIG Intravenous immunoglobulin, MDA5 melanoma differentiation-association gene 5, PE Plasma exchange, AZP Azathioprine, Tac tacrolimus, MMF Mycophenolate mofetil, MTX Methotrexate, RTX Rituximab, ND No data
^aThe levels of anti-MDA5 antibody in each patient (range, 357–902 U). ^bTwo patients were positive for anti-MDA5 antibody (range, 78–172 U). One case was not tested for it

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ISSN: 1546-0096

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