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IgG4-Related Disease Case Study

Home ยป IgG4-Related Disease Case Study
IgG4-Related Disease Case Study
  • Case

  • Case

A 50 yo M with hx of pancreatitis and asthma presents with chronic facial swelling.

Questions to Ask?

  • Symmetry?
  • Swelling in other body areas?
  • Any new medications or other exposures?
  • Sicca symptoms?

Clinical Presentation & History

  • Exam reveals bilateral lacrimal, parotid, and submandibular gland swelling
  • Palpable cervical and axillary lymphadenopathy
  • No fever, night sweats, weight loss, or other systemic symptoms
  • Patient notes two episodes of idiopathic pancreatitis in the past 2 years, but has no symptoms at present, and the abdominal exam is unremarkable.

Diagnostic Workup

  • Recommended labs: CBC w/ diff, CMP, ESR, CRP, HIV, IGRA, SPEP/SFLC, ANA, RF, SSA/SSB, IgG subsets, IgG4, IgE, C3, C4
  • Recommended imaging: CT head and chest/abdomen/pelvis
Differential Diagnosis

Next Steps

  • Broad imaging evaluation helps with disease characterization and identification of a biopsy target (likely head/neck gland in this case).
  • Imaging and biopsy results, as well as any laboratory evidence of organ system compromise, would guide next steps.

Differential Diagnosis

The combination of bilateral salivary and lacrimal gland enlargement with lymphadenopathy and a history of idiopathic pancreatitis suggests the possibility of IgG4-related disease (IgG4-RD).

Cancer, infection, or other autoimmune disease are still on the differential.

Differential Diagnosis

Given unilateral proptosis, there is a high pretest probability of a mass lesion.

Orbital masses are not uncommon in IgG4-related disease (IgG4-RD), and a number of ocular/periocular structures (particularly lacrimal glands) may be involved.

Related Content

IgG4-Related Disease Overview

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Primary Care2024-05-21T13:44:10+00:00

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This product was funded by a medical grant from Pfizer. The content was written by a physician work group. See Authors

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