What causes lacrimal gland enlargement?

Causes of Lacrimal Gland Enlargement

Lacrimal gland enlargement is most commonly caused by inflammatory conditions, particularly sarcoidosis, Sjögren's syndrome, and IgG4-related disease, followed by infiltrative processes such as lymphoma and metastatic disease. These conditions require prompt evaluation to prevent morbidity and potential mortality.

Common Causes of Lacrimal Gland Enlargement

Inflammatory Conditions

• Sarcoidosis: A multi-system granulomatous disorder that commonly affects the lacrimal glands 1

  • Presents with bilateral lacrimal gland enlargement
  • Often accompanied by hilar lymphadenopathy on chest imaging
  • May show non-caseating granulomas on biopsy

• Sjögren's syndrome: Autoimmune exocrinopathy affecting lacrimal and salivary glands 1

  • Presents with aqueous tear deficiency and dry eye symptoms
  • Associated with increased risk of lymphoma (5% of patients) 1
  • Requires serological workup due to systemic implications

• IgG4-related disease: Increasingly recognized cause of dacryoadenitis 2, 3

  • Often presents with bilateral involvement
  • Frequently involves other head and neck structures (paranasal sinuses, salivary glands)
  • Characteristic histopathological findings

• Non-specific dacryoadenitis: Inflammatory condition without specific etiology 3

  • Generally presents with smaller lacrimal gland enlargement compared to other causes
  • Less likely to show the "wedge sign" on imaging (54.8% vs. 84.8% for IgG4-RD)

Neoplastic Conditions

• Lymphoma: Important cause of lacrimal gland enlargement, especially in older patients 3, 4

  • More common in patients with average age of 60 years
  • More frequently unilateral (62.5%) compared to inflammatory causes
  • Larger gland size (average 3.3 cm) compared to non-specific dacryoadenitis (2.3 cm)

• Epithelial tumors: Both benign and malignant 4

  • Benign: Pleomorphic adenoma
  • Malignant: Adenoid cystic carcinoma (pain is a cardinal symptom)
  • Imaging typically shows well-demarcated mass for benign lesions
  • Malignant lesions may show calcifications and bone destruction

Infectious Causes

• Viral infections: Associated with lacrimal gland enlargement 1

  • Epstein-Barr virus
  • HIV/AIDS (21% have dry eye)
  • Hepatitis C

• Bacterial dacryoadenitis: Acute inflammatory condition 5

  • Can progress to abscess formation if untreated
  • May require intravenous antibiotics

Other Causes

• Infiltrative conditions:

  • Hemochromatosis
  • Amyloidosis
  • Graft-versus-host disease (GVHD)

• Lacrimal gland prolapse: Presents as upper eyelid mass 6

  • Often shows mild chronic inflammation on histopathology
  • May be bilateral in about 27% of cases

Clinical Features and Diagnostic Approach

Key Clinical Features

• Palpable mass in the superotemporal orbit
• Displacement of the eyeball
• Decreased ocular motility
• Diplopia and ptosis
• Pain (particularly concerning for malignancy)

Diagnostic Workup

1. Imaging:

   • CT and MRI to evaluate:
     • Size and extent of enlargement
     • Unilaterality vs. bilaterality
     • Presence of "wedge sign"
     • Angle with orbital wall
     • Extra-lacrimal and extra-orbital involvement

2. Laboratory testing:

   • Serum ACE levels (for sarcoidosis)
   • Sjögren's antibodies (SS-A/Ro, SS-B/La)
   • IgG4 levels
   • Complete blood count
   • Inflammatory markers

3. Biopsy:

   • Often necessary for definitive diagnosis
   • Critical to differentiate inflammatory from neoplastic causes
   • Histopathology guides treatment approach

Management Considerations

• Inflammatory conditions: Often responsive to corticosteroids
• IgG4-related disease: Steroid therapy, may require immunosuppressants
• Lymphoma: Chemotherapy, often including monoclonal antibodies
• Epithelial tumors: Surgical excision, with adjuvant therapy for malignant lesions
• Infectious causes: Appropriate antimicrobial therapy

Clinical Pearls and Pitfalls

• Age is an important factor - lymphoma is more common in older patients (mean age 60 years)
• Bilateral involvement is more common in inflammatory conditions, particularly IgG4-RD and sarcoidosis
• Pain is a concerning symptom that may indicate malignancy, particularly adenoid cystic carcinoma
• Lacrimal gland enlargement in younger patients or males should raise suspicion for systemic conditions
• Extra-orbital head and neck involvement strongly suggests IgG4-RD

The diagnostic approach to lacrimal gland enlargement requires careful consideration of clinical presentation, imaging findings, and often histopathological confirmation to guide appropriate treatment and improve outcomes related to morbidity, mortality, and quality of life.