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.