Mikulicz syndrome (Sclerosing sialo-dacryoadenite)
Contents
Definition
Mikulicz syndrome is a chronic benign disease in which both saliva glands located in front of the ears (glandula parotis) and the tear glands above the eyelids (glandula lacrimalis) swell. Most cases are flushed IgG4 related disease. One detects when elevated IgG4 is found in a tissue sample and sometimes in the blood. Similar symptoms can be seen in unusual cases of sarcoidosis (Rao D, 2017). The cause of the disease's localization is unknown, but an autoimmune process is likely. The result is that large amounts of hard, scar-like tissue can displace healthy tissue and reduce salivary and lacrimal gland function. Women are attacked more frequently than men.
Symptoms
Swollen glands and dry mouth. Swollen salivary glands in front of the ears (parotid glands) and under the lower jaws (submandibularis glands). Some suddenly notice extreme dryness in the mouth and swallowing problems because saliva production is reduced.
Symmetrical swelling above the eye (lacrimal glands) and dry eyes
Küttner's tumor caused by sclerosing sialo-adenitis, most often in a submandibular gland (benign, IgG4 related condition)-
Examinations
Medical history covers current symptoms such as swelling in the glands and dryness in the mouth and eyes. At the same time, symptoms from other organs that can be attacked in IgG4-related disease, such as lymph nodes, are sought.
Clinical noticeably hard but well-defined swellings are felt in the affected glands. Lymph nodes on the neck, under the arms and in the groin are also assessed. A general examination of the lungs, heart and stomach area is also done.
Laboratory tests may be inconspicuous, but for investigation one can consider measuring hemoglobin, white blood cells (leukocytes with differential counts), thrombocytes, CRP, kidney-liver-bile-pancreas and metabolism tests, immunoglobulins with sub-classes of IgG (including IgG4). Hormone tests (TSH, growth hormone/somatotropin, anti-diuretic hormone/ADH) for possible pituitary manifestations can also be considered. Antinuclear factors (ANA) are usually normal. Urine sting.
Imaging with ultrasound, alternatively an MRI examination can show the size of the swellings. PET/CT can be useful if you want to assess disease spread in the case of IgG4-related disease in arteries, salivary glands, lymph nodes and other organs. The method can thus be used for stage classification and monitoring (reference: Ebbo M, 2014).
Tissue sample (biopsy) is important to ensure the diagnosis (Deshpande V, 2012).
Similar diseases / differential diagnoses
- Alcoholism (swollen parotid glands)
- Bulimia / Chronic malnutrition (swollen parotid glands)
- Lymphoma
- Biopsy shows a characteristic findings
- Sarcoidosis
- Heerfordt's syndrome (Swollen glands and palsy in cranial nerves)
- Sjögren's syndrome Se Differential diagnoses of Sjögren's syndrome and the list below
- Salivary gland tumors (benign and malignant) with blockage in the drain between the parotid gland and the mouth (inside the cheek)
Mikulicz syndrome: Difference from Sjögren's syndrome:
- The incidence is the same for men and women (Sjøgren's is much more common among women (>10:1)
- Not noticeable fatigue
- Significantly enlarged tear and salivary glands but not chronic dry mouth (xerostomy) or dry eyes (xerophthalmia)
- Persistent gland enlargement
- At the same time other IgG4 manifestations such as autoimmune pancreatitis
- Increased level of IgG4 in the blood and IgG4+ plasma cell infiltration in tissues
- No occurrence of SSA / B antibody in blood sample
- Better response to corticosteroids treatment (prednisolone) compared to Sjögren's syndrome
- Tissue sample (biopsy) shows differences: IgG4-positive plasma cell infiltration is not typical for Sjøgren's syndrome.
Treatment
- Prednisone 5-7,5 mg/day can keep the condition in check. A higher dose initially (references: Yamamoto M, 2005; Nambiar S, 2021).
- rituximab (MabThera / Rixathon) usually also has a good effect and can reduce the need for Prednisolone (reference: Yamamoto M, 2015; Khosroshahi A, 2012).
Literature
- Takahashi H, 2016
- Yamamoto M, 2006 (Mikulicz syndrome and IgG4 disease)
- Nambiar S, 2021 (IgG4 related disease and treatment)
- Geyer JT, 2011
- Grans Compendium in Rheumatology
