Autoinflammatory & rare systemic diseases
Zanubrutinib monotherapy for IgG4-related head and neck disease
Authors' conclusion
Zanubrutinib monotherapy produced substantial imaging-defined and clinical improvements in active glandular IgG4-RD without glucocorticoid induction.
Study context
- Population
- Participants with active lacrimal and submandibular gland IgG4-RDevaluable n = 8
- Participants
- 10 participants
- Outcome
- Change in lacrimal and submandibular gland volume by blinded FDG PET/MRI
- Follow-up
- Week 24
Methodological detailsopen-label, single-arm
- Open-label
- Single-arm
Main finding
As reported in the abstract
Mean gland volume change
Lacrimal gland volume decrease (mean), P = .008
46.7%
Submandibular gland volume decrease (mean), P = .008
29.9%
Secondary outcomes
Metabolic imaging · week 24
- Total lesion glycolysis change, P = .05
- −91.6 g
- Total metabolic lesion volume change, P = .05
- −20.7 cm³
Clinical disease activity (IgG4-RD Responder Index) · week 24
- IgG4-RD Responder Index change, P = .01
- −6.0 points
Serum IgG4 · week 24
- Serum IgG4 change, P = .008
- −417 mg/dL
- Alongside reductions in circulating plasmablasts
Safety
- Serious events (COVID-19, occurred off treatment)
- 1
- Adverse events predominantly mild
Original publication
Zanubrutinib monotherapy for IgG4-related head and neck disease. Ann Rheum Dis. Published 14 July 2026. doi:10.
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