Mikulicz syndrome with salivary and tear glands. Takahashi H, 2018. CC BY SA 3.0

Mikulicz syndrome (Sclerosing sialo-dacryoadenite)

Definition

Mikulicz syndrome is a chronic disease where both saliva glands located in front of The ears (glandula parotid) and over the eyelids (glandula lacrimalis) swell. The disease is now classified as one IgG4 related disease. Elevated IgG4 is found in tissue samples and sometimes in the blood. The cause of the disease is unknown, but an autoimmune process is likely. Women are attacked more frequently than men.

Symptoms

• Sudden extreme dry mouth and swallowing problems
• Symmetrical swelling of the eye (lakrimalis glandler), in front of the ears (parotid glands) and sometimes under the jaws (submandibular glandler)
• Dry eyes
• Swelling of the glands below the jaws
  • Küttner's tumor caused by sclerosing sialoadenite, most commonly in a submandibularis gland (benign, IgG4 related)

Diagnosis

Tissue sample / biopsy: Histologically massive infiltration of mononuclear cells

Incorrect diagnosis? (Similar diseases / differential diagnoses)

• Lymphoma
  • Biopsy shows a characteristic findings
• Sarcoidosis
  • Heerfordt's syndrome (Swollen glands and palsy in cranial nerves)
• Bulimia / Chronic malnutrition (swollen parotid glands)
• Chronic alcoholism (swollen parotid glands)
• Salivary glands (benign and malignant)
• Obstruction in the drain between the parotid gland and the mouth (inside of the cheek)

• Sjögren's syndrome
  • Se Differential diagnoses of Sjögren's syndrome and the list below

PET / CT, MRI and biopsy. Marginal zone B cell lymphoma that clinically mimics Mikulicz syndrome Affiliation: Section of Oral and Maxillofacial Oncology, Kyushu University Japan. CC BY 4.0

Mikulicz syndrome: Difference from Sjögren's syndrome:

1. Prevalence similar for men and women (Sjögren's syndrome is much more common among women (> 10: 1)
2. Not abnormal fatigue
3. Significantly enlarged tear and salivary glands but not chronic dry mouth (xerostomy) or dry eyes (xerophthalmia)
4. Persistent gland enlargement
5. More often other IgG4 manifestations such as autoimmune pancreatitis
6. Increased level of IgG4 and IgG4 + plasma cell infiltration
7. No occurrence of SSA / B antibody in blood sample
8. Better response to corticosteroids treatment (Prednisone) compared to Sjögren's syndrome
9. Histologic differences: IgG4-positive plasma cell infiltration is not typical of SS

Treatment

• Prednisone 5-7,5 mg / day can keep the condition at bay. A higher dose initially (reference: Yamamoto M, 2005)
• rituximab (MabThera / Rixathon) usually also has a good effect and can reduce the need for Prednisolone (reference: Yamamoto M, 2015)

Literature

• Takahashi H, 2016
• Yamamoto M, 2006 (Mikulicz syndrome and IgG4 disease)
• Geyer JT, 2011
• Grans Compendium in Rheumatology

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