this presentation includes general idea about SS and it's effect on peripheral and central nervous system.

1. Sjögren’s Syndrome
(SS)

2. Case Hx:
• A 53 yrs old female, House wife, admitted to a
community hospital due to numbness and weakness in
her right extremities. Also she has a long history of
dryness in mouth (for 1 year before admission) and
difficulty in swallowing (esp. solid sort of food). In
addition, she suffered dryness in her eyes So she can’t
open her eyes in the morning. Her complaint also
involved severe episodes of fatigability.

3. One main difference ?
R
L
Exocrine glands Endocrine glands

4. SS overview
• It is a systemic, chronic, autoimmune inflammatory disease.
• It is characterized by lymphatic infiltration in exocrine glands.
• SS have two types these are : primary and secondary.
• primary Sjögren’s Syndrome (pSS) occurs in the absence of another
underlying rheumatic disorder (such as SLE, RA or scleroderma), at
the opposite of secondary Sjögren’s Syndrome (sSS).
• SS affects females more than males (ratio of 9:1).

5. What is really happening in there?
Pathophysiology of SS ?
Multifactorial?

6. Clinical features
Most patients with Sjögren’s Syndrome present with glandular
symptoms (Sicca symptoms) in addition, several extra-glandular
features may develop:
A- Glandular:
• Xerostomia (mouth dryness).
• Xerophthalmia (eye dryness).
• Xeroderma.
• Vaginal dryness.
• Glandular swelling.

7. Clinical features
B- Extra-grandular:

8. Neurological corner
1- Peripheral manifestations (PNS): commonest
• Autonomic neuropathy: C/F lightheadedness when standing with
severe fatigability, decreased or increased sweating, and feeling full
despite eating small meals.
• Trigeminal Neuralgia: C/F numbness or burning of the face.
• Glossopharyngeal Neuralgia: C/F pain in the back of the throat, which
may worsen while swallowing.
• Mono neuritis multiplex.

9. Neurological corner
2- Central manifestations (CNS): rare
• Myelitis: involves an infection or the inflammation of white or grey
matter of spinal cord.
• Pachymenigitis: inflammation of dura matter.
• Hypophysitis: pituitary gland inflammation.
• Optic neuritis.

10. Neurological corner

11. Diagnosis
• ESR.
• CRP.
• ANA.
• RF.
• Anti-SS-A (Anti-Ro).
• Anti SS-B (Anti-La).
• Labial Salivary gland biopsy.
• Vaginal swab.
• Specific tests: Schirmer test.

12. Treatment
• Education: maintain good hydration by drinking plenty of water and
chew gums.
• Artificial tears (eye drops).
• Mouth sprays.
• HCQ.
• MTX.
• Immunosuppressive drugs.
• NSAIDs.