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From the American College of Rheumatology

Rheumatology for Primary Care
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IgG4-Related Disease Case Studies

Home » IgG4-Related Disease Case Studies
IgG4-Related Disease Case Studies
  • Case 1

  • Case 2

  • Case 1

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
  • Pt 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
  • 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.
  • Case 2

A 65 yo M with hx of HTN and pulmonary nodules presents with diplopia.

Questions to Ask?

  • Duration of symptoms?
  • Any other neurologic symptoms?
  • Any systemic symptoms?
  • Headache or jaw claudication?
  • History of anything similar?

Physical Exam

  • L proptosis with mildly restricted movement of L eye in all directions
  • Neurologic and general exams otherwise normal
  • No musculoskeletal symptoms
  • Cardiovascular, musculoskeletal, and lymph node exams are normal

Diagnostic Workup

  • Recommended labs: CBC w/ diff, CMP, TSH
  • Recommended imaging: CT head
Differential Diagnosis

Next Steps

  • Imaging is an important first step to confirm the presence of a mass and help characterize it.
  • Biopsy will likely be important.
  • Consider a broader imaging workup to determine the extent of disease and to potentially identify a more attractive biopsy target (perhaps pulmonary nodules, in this case).

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.

View Information About IgG4-Related Disease
Tami Bonnett-Admi2024-02-20T22:09:08+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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