IgG4-related systemic disease in a Native American man

Area of Science:

• Rheumatology and Immunology
• Pathology
• Endocrinology

Background:

• Immunoglobulin G4-related systemic disease (IgG4-RD) is an emerging clinical entity.
• It is characterized by IgG4-positive plasma cell infiltration across various organs.
• Accurate diagnosis can be challenging due to its diverse presentations.

Observation:

• A case of biopsy-confirmed IgG4-related systemic disease with extensive multi-organ involvement is presented.
• Affected organs included the pituitary gland (hypophysitis), lacrimal glands (dacryoadenitis), retroperitoneum, and kidneys (tubulointerstitial nephritis).
• The patient's presentation highlighted the systemic nature and diagnostic complexity of IgG4-RD.

Findings:

• The disease involves infiltration by polyclonal IgG4-positive plasmacytes.
• Manifestations are widespread, including dacryoadenitis, sialadenitis, thyroiditis, pneumonitis, retroperitoneal fibrosis, pancreatitis, sclerosing cholangitis, tubulointerstitial nephritis, prostatitis, and hypophysitis.
• The presented case exemplifies multi-organ involvement with hypophysitis, dacryoadenitis, retroperitoneal fibrosis, and tubulointerstitial nephritis.

Implications:

• Raising awareness of IgG4-related systemic disease within the medical community is essential for timely diagnosis.
• Prompt recognition and management, particularly with systemic corticosteroids, can lead to favorable patient outcomes.
• Further research into the pathogenesis and treatment of IgG4-RD is warranted.