Table 1.

Features of cases with IgG4-RD administerd dupilumab

Author	1	2	3	4	5	6	7	8
	Simpson et al11	Ebbo et al8	Otani et al10	Nakajima et al9	Our case 1	Our case 2	Our case 3	Our case 4
Year	2020	2020	2021	2021
Age	67	51	57	51	53	56	67	50
Gender	Male	Male	Male	Female	Female	Female	Male	Female
2019 ACR/EULAR classification score	n/a	29	n/a	n/a	32	47	38	28
Organ involvement at diagnosis	Skin parotid glands paranasal sinus retropritoneal lung	Lacrimal glands parotid glands sublingual glands submandibular glands	Lacrimal glands parotid glands lung	Lacrimal glands paranasal sinus	Submandibular glands paranasal sinus	Lacrimal glands submandibular glands paranasal sinus lung	Pancreas submandibular glands paranasal sinus	Lacrimal glands
Biopsy proven (lesion of biopsy)	Yes (prostate)	Yes (lymph node)	Yes (parotid gland)	Yes (lacrimal gland)	Yes (submandibular gland)	Yes (submandibular gland)	Yes (submandibular gland)	Yes (lacrimal gland)
Serum IgG4 levels at diagnosis*	20.60 g/L	17.7 g/L	270 mg/dL	768 mg/dL	360 mg/dL	477 mg/dL	716 mg/dL	163 mg/dL
Previous immune therapy	None	GCs rituximab	GCs azathioprine	None	None	None	GCs mepolizumab	GCs
Indication of dupilumab	Atopic dermatitis asthma	Chronic rhinosinusitis with nasal polyps asthma	Asthma	Eosinophilic sinusitis	Eosinophilic sinusitis	Eosinophilic sinusitis	Asthma	Asthma
Dose of dupilumab	600 mg for the first dose 300 mg every 2 weeks	600 mg for the first dose 300 mg every 2 weeks	600 mg for the first dose 300 mg every 2 weeks	600 mg for the first dose 300 mg every 2 weeks	300 mg every 2 weeks	300 mg every 2 weeks	300 mg every 4 weeks	300 mg every 4 weeks
Glucocorticoids Immunosupressants	None	None	10 mg predonisolone 25 mg azathioprine	None	None	None	10 mg predonisolone	6 mg predonisolone
IgG4-RD responder index at dupilumab administration	n/a	n/a	n/a	n/a	9	15	3	3
Organ involvement at dupilumab administration	Skin parotid glands paranasal sinus retropritoneal lung	Salivary glands paranasal sinus lympho nodes	Lacrimal glands parotid glands lung	Lacrimal glands paranasal sinus	Submandibular glands paranasal sinus	Lacrimal glands submandibular glands paranasal sinus lung	Lung	Lung
Organ involvements not responded to dupilumab	n/a for parotid glands paranasal sinus	Salivary glands lympho nodes	None	None	None	None	None	None
Outcome	Effective to organ involvement	Ineffective to organ involvement	Effective to GC sparing	Effective to organ involvement	Effective to organ involvement	Effective to organ involvement	Effective to GC sparing	Effective to GC sparing

Summary table of the cases with IgG4-RD administered dupilumab.

*The originally reported units for serum IgG4 levels were written in the table because the methods of the measurement were estimated to be different.

GC, glucocorticoid; IgG4-RD, IgG4-related disease; n/a, not available.