Mikulicz disease

Mikulicz Disease: A Rare Condition Characterized by Enlarged Glands

Mikulicz disease, also known as IgG4-related dacryoadenitis and sialoadenitis, is a rare condition characterized by the enlargement of the lacrimal and salivary glands. This condition is often associated with an elevated level of immunoglobulin G4 (IgG4) in the serum.

Key Features:

• Bilateral Enlargement: Mikulicz disease typically involves the bilateral enlargement of the lacrimal and salivary glands.
• Painless Swelling: The condition is often characterized by painless swelling of the affected glands.
• Symmetrical Involvement: The involvement of the glands is usually symmetrical, meaning that both sides are equally affected.

Causes and Associations:

• IgG4-Related Disease: Mikulicz disease is considered to be an IgG4-related systemic disorder, which means that it is associated with elevated levels of IgG4 in the serum.
• Immunological Involvement: The condition involves immunological mechanisms, including infiltration of lymphocytes and other immune cells into the affected glands.

References:

• [1] Mikulicz syndrome is defined as diffuse and bilateral enlargement of the salivary and lacrimal glands. (Source: 1)
• [2] Mikulicz's disease is a unique condition involving the enlargement of the lacrimal and salivary glands, similar to that observed in Sjogren's syndrome. (Source: 2)
• [3] Mikulicz syndrome is a chronic disease in which the glandular tissue of the head and neck are excessively enlarged, usually bilaterally. (Source: 3)
• [4] Mikulicz's disease (MD) refers to idiopathic, bilateral, painless, and symmetrical swelling of the lacrimal, parotid, and submandibular glands. (Source: 4)
• [5] Mikulicz disease is characterized by symmetrical, persistently enlarged lacrimal, salivary, and ... (Source: 7)

Mikulicz Disease Signs and Symptoms

Mikulicz disease, also known as IgG4-related dacryoadenitis and sialoadenitis, is a chronic condition characterized by the inflammation of the lacrimal glands (which produce tears), parotid glands, and submandibular glands (two of the major salivary glands). The symptoms of Mikulicz disease can vary from person to person, but here are some common signs and symptoms:

• Bilateral Enlargement of Glands: One of the hallmark symptoms of Mikulicz disease is the bilateral enlargement of the lacrimal, parotid, and submandibular glands. This can cause discomfort and pain in the affected areas.
• Dry Eyes (Xerophthalmia): Many people with Mikulicz disease experience dry eyes due to reduced tear production. This can lead to eye irritation, redness, and blurred vision.
• Dry Mouth (Xerostomia): Mikulicz disease can also cause a significant reduction in saliva production, leading to dry mouth, difficulty swallowing, and an increased risk of tooth decay.
• Recurring Fevers: Some people with Mikulicz syndrome may experience recurring fevers, which can be accompanied by generalised weakness, malaise, and fatigue.
• Swollen Salivary Glands: The submandibular and sublingual glands are often affected in Mikulicz disease, leading to swollen salivary glands that can cause discomfort and pain.
• Oral Dryness: Many people with Mikulicz disease experience severe oral dryness, which can lead to difficulty swallowing and speaking.

References

• [8] Mikulicz's disease (MD) refers to idiopathic, bilateral, painless, and symmetrical swelling of the lacrimal, parotid, and submandibular glands.
• [9] Some people with Mikulicz syndrome may experience recurring fevers. The fever may be accompanied by dry eyes, diminished tear production (xerophthalmia), and difficulty swallowing.
• [10] Signs & Symptoms. Mikulicz syndrome is characterized by the sudden onset of extreme dryness in the mouth (xerostomia) that may lead to difficulty swallowing and tooth decay.
• [13] If Mikulicz syndrome is diagnosed, that is, an increase in the salivary and lacrimal glands due to chronic lymphoproliferative diseases (lymphomas, lymphogranulomatosis, etc.), symptoms characteristic of the underlying disease will be observed in the clinical picture.

Diagnostic Tests for Mikulicz Disease

Mikulicz disease, also known as Mikulicz syndrome, can be challenging to diagnose due to its nonspecific symptoms and overlapping features with other conditions. However, several diagnostic tests can help confirm the diagnosis.

• Biopsy of the swollen gland: A biopsy of one of the swollen glands is key to the diagnosis of Mikulicz syndrome (1). This test helps rule out other causes of gland swelling.
• Ultrasound examination: An ultrasound examination of the area may also be performed to rule out other reasons for gland swelling (2).
• Schirmer/fluorescent dye test: The Schirmer/fluorescent dye test is a positive indicator of Mikulicz disease, but blood samples taken are negative for both SS-A and SS-B antibodies (1).
• Immunological and immunochemical study: An immunological and immunochemical study is carried out to diagnose Mikulicz disease, followed by an examination by an allergist-immunologist, as well as an ophthalmologist's consultation with the Schirmer test and a sample with fluorescein (11).
• Computed tomography (CT) scan: A CT scan from the head to the neck in MD patients revealed severe enlargement of symmetrical lacrimal and submandibular or parotid glands (12).

Diagnostic Criteria

The diagnosis of Mikulicz disease is based on the following criteria:

1. Symmetric and persistent swelling of the lacrimal glands and either or both of the major salivary glands (parotid and submandibular) (13, 14).
2. Exclusion of other diseases that may mimic this presentation, such as sarcoidosis, viral infection, or lymphoproliferative disorders (13).

Laboratory Tests

Results of laboratory tests showed high levels of serum IgG4 (1240 mg/dL) and IgE (401 μg/L) in some cases (14). However, these findings are not specific to Mikulicz disease.

In summary, the diagnostic tests for Mikulicz disease include biopsy of the swollen gland, ultrasound examination, Schirmer/fluorescent dye test, immunological and immunochemical study, and computed tomography scan. The diagnosis is based on symmetric and persistent swelling of the lacrimal glands and either or both of the major salivary glands, with exclusion of other diseases that may mimic this presentation.

References:

(1) Context reference 1 (2) Context reference 2 (11) Context reference 11 (12) Context reference 12 (13) Context reference 13 (14) Context reference 14

Treatment Options for Mikulicz Disease

Mikulicz disease, also known as IgG4-related plasmacytic syndrome, is a rare autoimmune disorder that affects the lacrimal and salivary glands. While there is no definitive curative treatment for this condition, various drug therapies have been shown to be effective in managing its symptoms.

Glucocorticoids

Glucocorticoids, such as prednisone, are often used as a first-line treatment for Mikulicz disease. These medications can help reduce inflammation and swelling in the affected glands, leading to rapid improvement in glandular function [12][13]. In some cases, glucocorticoids may be administered at high doses (30-40 mg/day) to achieve quick relief from symptoms [12].

Immunosuppressive Agents

In addition to glucocorticoids, immunosuppressive agents like mizoribine have been used to treat Mikulicz disease. Mizoribine has been shown to be effective in reducing inflammation and improving glandular function, particularly when used in combination with corticosteroids [3][6]. Other immunosuppressive agents, such as rituximab, may also be considered for treatment in some cases [9].

Other Treatment Options

While less common, other treatment options may include:

• Artificial tears and saliva to maintain moisture in the eyes and mouth
• A soft moist diet to alleviate oral symptoms
• Symptomatic therapy with NSAIDs (nonsteroidal anti-inflammatory drugs) for pain relief

It's essential to note that each patient's response to treatment may vary, and a personalized approach is often necessary. Consultation with a healthcare professional is recommended to determine the best course of treatment for individual cases.

References:

[1] Treatment of Mikulicz syndrome (no definitive curative treatment) [2] Most therapies are directed toward treating any underlying disease [3] A case of Mikulicz's disease, IgG4-related plasmacytic syndrome, successfully treated by corticosteroid and mizoribine [3] [6] MZR is an immunosuppressive agent used for renal transplants and patients with rheumatoid arthritis (RA), lupus nephritis, and nephrotic syndrome in Japan [9] Some studies have found that the combination of GCs with low-dose rituximab is more effective and safer in the treatment of IgG4-RD compared to GCs alone [9] [12] Treatment of Mikulicz's disease. Mikulicz's disease is mainly treated by the administration of steroids. [13] Mikulicz's disease is a unique condition involving the enlargement of the lacrimal and salivary glands, similar to that observed in Sjogren's syndrome; however, Mikulicz's disease is clinically characterized by infrequent autoimmune reactions and responsiveness to glucocorticoid treatment.

Differential Diagnosis of Mikulicz Disease

Mikulicz disease, also known as Mikulicz syndrome, is a non-specific inflammatory enlargement of at least two or more of the salivary and lacrimal glands with xerostomia (dry mouth). The differential diagnosis of Mikulicz disease involves distinguishing it from other conditions that may present with similar symptoms.

Conditions to Consider:

• Sjögren's Syndrome: A systemic autoimmune disorder characterized by exocrine gland inflammation, particularly affecting the salivary and lacrimal glands. [8]
• Tuberculosis: A bacterial infection that can cause enlargement of the salivary and lacrimal glands. [1]
• Leukemia: A type of cancer that affects the blood and bone marrow, which can also cause glandular enlargement. [4]
• IgG4-related Mikulicz's disease: A rare condition characterized by the presence of lesions in the head and neck, salivary and lacrimal gland enlargement, and elevated IgG4 levels. [3]

Diagnostic Features:

• Enlargement of at least two or more of the salivary and lacrimal glands
• Xerostomia (dry mouth)
• S-shaped contour to the upper eyelid, suggesting lacrimal gland lesions [6]
• Roentgenograms showing a pattern of variations indicating differential diagnosis from other conditions [5]

Key Takeaways:

• Mikulicz disease is a non-specific inflammatory condition that requires differential diagnosis from other systemic autoimmune disorders and infections.
• Conditions to consider in the differential diagnosis include Sjögren's Syndrome, Tuberculosis, Leukemia, and IgG4-related Mikulicz's disease.
• Diagnostic features include glandular enlargement, xerostomia, and specific radiographic findings.

References:

[1] Although the disorder is almost always described as benign, it always occurs in association with another underlying disorder such as tuberculosis, leukemia, ...

[3] by Y Otani · 2022 · Cited by 5 — IgG4-related Mikulicz's disease is suspected and diagnosed based on the presence of lesions of the head and neck, salivary and lacrimal gland enlargement, and ...

[4] by D Meyer · 1971 · Cited by 19 — The term Mikulicz's syn- drome was used to describe enlargement of the salivary gland with or without lacrimal gland involvement occurring in leukemia,.

[5] by P Rubin · 1957 · Cited by 18 — The resulting roentgenograms have shown a pattern of variations indicating that sialography can be used for the differential diagnosis of the two conditions.

[6] by N Aoki · 2024 — The S-shaped contour to the upper eyelid is a useful sign in the differential diagnosis of palpebral edema, suggesting lacrimal gland lesions, as in this case.

[8] by K Tsubota · 2000 · Cited by 131 — Mikulicz's disease (MD) is a well-known disorder characterized by enlarged lacrimal and parotid glands caused by infiltration with lymphocytes.