51. Mikulich Syndrome. Sjorgen Syndrome

Question 1

Primary Sjögren’s syndrome (pSS)

Answer 1

• Autoimmune disorder=>
• Immune system mistakenly attacking body’s moisture-producing glands=>
• Dry eyes and mouth

-Typically occurs around age 40.
-Sex: More common in women.
-Rheumatic Disease: Higher risk if the individual has rheumatoid arthritis or lupus.

Question 2

Distinguishes secondary Sjögren’s syndrome (sSS) from primary Sjögren’s syndrome (pSS)

Answer 2

• Secondary Sjögren’s syndrome (sSS) occurs w/ another rheumatic disease=>
• Systemic Lupus Erythematosus (SLE)
• Rheumatoid Arthritis
• Systemic Sclerosis

Question 3

Etiological factors of Sjögren’s syndrome

Answer 3

• Genetic Predisposition
• Triggering Mechanism=> An infection by virus or strain of bacteria

Question 4

How Sjögren’s syndrome diagnosed

Answer 4

• Blood Tests
• Eye Tests=> Schirmer tear test measures tear production
• Imaging=>sialogram: An X-ray with dye to measure saliva flow
  Biopsy=> tissue from salivary glands=>
• Detect inflammatory cells

Question 5

Treatment options for Sjögren’s syndrome

Answer 5

• Medications=» Prescription eyedrops like cyclosporine (Restasis)
• Saliva Production: Drugs such as pilocarpine (Salagen)=>
• Increase saliva and tears.
  * NSAIDs => arthritis
• Antifungal medications => yeast infections in the mouth
• Surgery=>Punctal Occlusion=>
• Seal tear ducts with collagen or silicone plugs to preserve tears

Question 6

Mikulicz Syndrome

Answer 6

• painless, symmetrical swelling of the major salivary glands (parotid, submandibular, and sublingual) and lacrimal glands.
• Part of => benign lymphoepithelial lesions (BLL).

-Benign lymphoepithelial lesions (BLL)=> focal collections of lymphocytes that gradually form follicles, resembling mucosa-associated lymphoid tissue (MALT). These lesions are typically found in the major salivary and lacrimal glands.

Question 7

Etiology of Mikulicz Syndrome

Answer 7

• Not well understood=>
• May be related to autoimmune processes =>
• Often overlaps w/ Sjögren’s syndrome and MALT lymphoma

Question 8

How Mikulicz Syndrome related to MALT lymphoma

Answer 8

• Associated w/ benign lymphoepithelial lesions=>similar in appearance to MALT lymphoma
• Mikulicz Syndrome could progress to MALT lymphoma

Question 9

How Mikulicz Syndrome differentiated from other conditions with similar symptoms

Answer 9

• Histopathological Analysis
• Autoimmune Testing=> rule out Sjögren’s syndrome
• Imaging=>exclude other causes of glandular enlargement=>
• Tumors or infectious processes

Question 9

Treatment options for Mikulicz Syndrome

Answer 9

• Symptomatic Management=> Artificial tears and saliva substitutes to manage dryness.
• Immunosuppressive Therapy=> medications like corticosteroids or other immunosuppressants
• Regular Monitoring=>detect progression to MALT lymphoma or complications
  Surgical Intervention

-Many individuals manage well with symptomatic treatment and regular monitoring.