SCF ENCYCLOPEDIA ENTRY — IgG4-RELATED DISEASE (IgG4-RD)

SCF-AIMD-SYS-0009 | SCF PATHOGENESIS / VIRAGENESIS / THERAPEUTIC RECONSTRUCTION DOSSIER

Disease Classification:

Systemic fibroinflammatory immunometabolic disorder characterized by IgG4-positive plasma cell infiltration, Th2/Treg immune skewing, storiform fibrosis, obliterative phlebitis, and multi-organ tumefactive lesions.

I. SCOPE & POSITIONING

Clinical Definition:

IgG4-related disease (IgG4-RD) is a chronic, multi-organ immune-mediated condition defined by lymphoplasmacytic infiltration enriched in IgG4-positive plasma cells, progressive fibrosis, and mass-forming inflammatory lesions affecting pancreas, salivary glands, orbit, kidneys, lungs, retroperitoneum, and vasculature.

Core Clinical Domains:

• Tumefactive organ enlargement (pancreas, salivary, lacrimal)
• Autoimmune pancreatitis
• Sialadenitis / dacryoadenitis
• Retroperitoneal fibrosis
• Orbital pseudotumor
• Tubulointerstitial nephritis
• Pulmonary nodules / fibrosis
• Aortitis / periarteritis

Key Clinical Risks:

• Progressive organ fibrosis
• Misdiagnosis as malignancy