Adults
IgG4-related disease
Swelling of glands or organs (salivary, lacrimal, pancreas, retroperitoneum, aorta) that can mimic cancer.
What to look for
- Salivary or lacrimal gland enlargement, autoimmune pancreatitis or cholangitis
- Retroperitoneal fibrosis, aortitis, orbital disease, pachymeningitis
- Raised serum IgG4 in many patients. A normal level does not exclude it.
What to do
- Send serum IgG4 and IgG subclasses, CBC with eosinophils, metabolic panel, lipase if relevant, and C3/C4.
- Tissue biopsy is usually needed to exclude lymphoma or other cancers.
- Obstructive jaundice, hydronephrosis or aortic involvement is urgent.
- Inebilizumab became the first FDA-approved treatment in 2025. Glucocorticoids and rituximab are also used.
About this urgency level
Specialist evaluation helps, but these are usually less time-sensitive than new inflammatory arthritis or connective tissue disease.
Timeframes are typical targets. Local pathways, capacity and clinical judgment come first.