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Updated: May 22, 2025

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
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Navigating the labyrinth: Phenotypes of IgG4-related disease

M Shah1, K Bajan2, R Samant3

  • 1Department of Internal Medicine, P. D. Hinduja Hospital and Medical Research Centre, Mumbai, Maharashtra, India.

Journal of Postgraduate Medicine
|March 14, 2025
PubMed

Insights

This case report details IgG4-related disease (IgG4-RD) in an elderly female presenting with neck swelling and cough. Diagnosis required advanced imaging and biopsy, revealing overlapping head, neck, and systemic IgG4-RD features.

Area of Science:

  • Immunology
  • Rheumatology
  • Pathology

Background:

  • Immunoglobulin G4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition.
  • Clinical presentation can be diverse, often mimicking other diseases, posing diagnostic challenges.

Purpose of the Study:

  • To report a unique case of IgG4-related disease (IgG4-RD) in an elderly female.
  • To highlight the diagnostic complexities and overlapping clinical phenotypes of IgG4-RD.

Main Methods:

  • Initial evaluation included ultrasound, thyroid biopsy, and high-resolution computed tomography (HRCT) of the lungs.
  • Infective causes were excluded through comprehensive microbiological assessment.
  • Positron emission tomography (PET)-guided lung biopsy with immunohistochemical staining confirmed the diagnosis.

Main Results:

  • The patient presented with submental neck swelling, dry cough, and weight loss.
  • Ultrasound revealed chronic sialadenitis and a lymphocytic thyroid nodule.
  • HRCT showed multifocal lung consolidations and lymphadenopathy.
  • Diagnosis of IgG4-related disease (IgG4-RD) was confirmed via PET-guided lung biopsy.

Conclusions:

  • IgG4-related disease (IgG4-RD) can present with varied symptoms and imaging findings.
  • A high index of suspicion and advanced diagnostic techniques are crucial for timely diagnosis.
  • This case underscores the importance of considering IgG4-RD in patients with unexplained systemic inflammation and organ involvement.

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