WELL DONE AND HIGHLY QUALIFIED

1. Al-Quds
University
School of
Medicine

3. Venus Williams, is an American professional tennis
player who is a former World No. 1.She is 32-year-old,
African-American female. Was admitted to your clinic
suffering severe xerophthalmia (dry eyes), xerostomia
(dry mouth), xeroderma and parotid gland enlargement .
The erythrocyte sedimentation rate (ESR) was 105 mm/h
(N:0-10 mm/h).She complained from severe fatigue
episodes while training and playing. Some tests were
performed and the results were as shown below:
Schirmer's test : <4 mm wetting of the paper after 5
minutes . N: ≥15-10 mm after 5 minutes
Positive salivary gland biopsy findings.
Positive anti–SSA & anti–SSB antibodies results.
unstimulated salivary flow < 1.5mL in 15min
N: 0.3 - 0.4 ml/min (5.25 ml in 15 min)

4. Venus Williams is suffering from: Sjögren's Syndrome.
Overview
Sjögren's syndrome(S.S) is a systemic, chronic,
autoimmune, inflammatory disorder. Characterized by
lymphocytic infiltrates in exocrine organs. Most individuals
with Sjögren syndrome present with sicca symptoms.
In addition, numerous extraglandular features may
develop.
Primary Sjögren syndrome occurs in the absence of
another underlying rheumatic disorder, whereas Secondary
Sjögren syndrome is associated with another underlying
rheumatic disease, such as (SLE), rheumatoid arthritis (RA),

5. M.D HENRY Sjögren.

6. Etiology:
The etiology of Sjögren syndrome is not well understood.
Autoimmune disease
ongoing interaction between the innate and acquired immune systems.
There is presence of activated salivary gland epithelial cells expressing
major histocompatibility complex (MHC) class II molecules.
Association with the human leukocyte antigen(HLA)
Some evidence indicates that the true association of Sjögren syndrome
may be with HLA-DQA1.
According to Israeli research ,HLA-DR5 is associated with S.S in Israeli
persons.
Possible disease triggers
Viruses are viable candidates as environmental triggers, uncertain.
Epstein-Barr virus (EBV), HTLV-1, human herpesvirus 6 (HHV-6), HIV,
hepatitis C virus (HCV), and cytomegalovirus (CMV) may have a role.
Glandular pathology
New studies (Jan-2003) : role of apoptotic mechanisms in the pathogenesis
of primary Sjögren syndrome. A defect in Fas-mediated apoptosis, which is
necessary for down-regulation of the immune response, can result in a

7. Epidemiology
IN USA:
 Second most common rheumatologic disorder, behind
SLE.
 Sjögren syndrome affects 0.1-4% of the population.
Between different ethnic groups , the disease occurs
worldwide with similar prevalence.
The female-to-male ratio of Sjögren's syndrome is 9:1.
-The Journal of Clinical Endocrinology & Metabolism Vol. 94,
No. 6 2044-2051(2009)- Dehydroepiandrosterone (DHEA)
Role.
-2011 -Charles E. Schmidt College of Medicine, Florida-
Atlantic University.
Sjögren syndrome can affect individuals of any age but is

8. Prognosis
Sjögren syndrome carries a generally good
prognosis.
Morbidity associated with Sjögren syndrome is
mainly associated with the gradually decreased
function of exocrine organs.
Patients with primary Sjögren syndrome have a
normal life expectancy.
Children born to mothers with antibodies against
SSA/Ro are at an increased risk of neonatal lupus
and congenital heart block.

9. Presentation
The first symptoms in primary Sjögren syndrome can be
easily overlooked and overlapped.
Xerophthalmia (dry eyes) and xerostomia (dry mouth) are
the main clinical presentations in adults. Bilateral parotid
swelling is the most common sign of onset in children.
Extraglandular involvement in Sjögren syndrome falls into 2
general categories:
Periepithelial infiltrative processes include interstitial
nephritis, liver involvement, and bronchiolitis and generally
follow a benign course.
Extraepithelial extraglandular involvement in Sjögren
syndrome is related to B-cell hyperreactivity,
hypergammaglobulinemia, and immune complex formation
and includes palpable purpura, glomerulonephritis, and

10. Sicca symptoms (dry eyes and dry mouth and dry vagi
Fatigue.
Parotitis.
Cutaneous symptoms
Pulmonary symptoms
(xerotrachea),which can manifest as a dry cough.
Gastrointestinal symptoms.
Cardiac symptoms.
Renal disorders.
A history of recurrent miscarriages or stillbirths
Neurologic symptoms.(see neurology corner).

15. Diagnosis

16. Diagnosis of primary Sjögren syndrome requires 4
out of 6 of the below criteria:
Ocular symptoms - Dry eyes for more than 3
months, foreign-body sensation, use of tear
substitutes more than 3 times daily.
Oral symptoms - Feeling of dry mouth, recurrently
swollen salivary glands, frequent use of liquids to aid
swallowing.
Ocular signs – Schirmer’s test performed (< 5 mm
in 5 min).
Oral signs - Abnormal salivary scintigraphy
findings, abnormal parotid sialography findings,
abnormal sialometry findings (unstimulated salivary
flow < 1.5mL in 15min) .

17. Workup
No single test is sufficiently sensitive or specific in
the diagnosis of Sjögren syndrome.
Laboratory test results may indicate the following:
•Elevated erythrocyte sedimentation rate (ESR)
•Anemia
•Hypergammaglobulinemia
•Presence of antinuclear antibodies, especially anti-Ro
and anti-La
•Presence of anti–alpha-fodrin antibody (reliable
diagnostic marker of juvenile Sjögren syndrome)
•Creatinine clearance may be diminished in up to 50% of
patients
Schirmer test.
Serum electrophoresis.
Sialography and Scintigraph.

19. Differential diagnosis: ^ ^
Parkinson disease.
Sarcoidosis
Bell’s palsy.
Graft versus host disease
Amyloidosis
HIV infection
Sarcoidosis
HTLV-1 infection
Lymphoma
HCV infection
Viral infection (eg, mumps,
Keratoconjunctivitis sicca
Epstein-Barr virus,
Anxiety and depression
cytomegalovirus, coxsackievirus
Viral infections (eg, mumps)
A, influenza)
Complications from contact lenses
DILS associated with HIV
Dehydration
disease
Hypervitaminosis A
Granulomatous diseases
Neurotropic keratitis
(sarcoidosis, tuberculosis,
Mucous membrane pemphigoid
leprosy)
Environmental irritants
Hyperlipoproteinemia
Mouth breathing
Hepatic cirrhosis
Chronic blepharitis
Hepatitis C
Chronic conjunctivitis
Bulimia
Rosacea
Recurrent parotiditis of
Therapeutic radiation or surgery to the head and neck
childhood
Age
Chronic pancreatitis
Alzheimer disease

20. Treatment:
No curative agents for Sjögren syndrome exist.
 The treatment of the disorder is essentially symptomatic.
 In secondary Sjögren syndrome, treatment is based on
the accompanying disease and its clinical features.
Skin and vaginal dryness
Patients should use skin creams, such as Eucerin, or skin
lotions, such as Lubriderm.
Arthralgias and arthritis
Acetaminophen or nonsteroidal anti-inflammatory drugs
(NSAIDs).

21. Dry Eyes:
•Artificial tears should be applied liberally.
•Topical steroids.
•Cyclosporine.
•Nutritional supplements.
Dry mouth:
•Patients with dry mouth can liberally drink sips of
water and take bottled water with them on trips.
Additional treatment considerations:
•In patients with major organ involvement, such as
lymphocytic interstitial lung disease, consider
therapy with steroids and immunosuppressive
agents, such as cyclophosphamide.

22. Surgical Therapy
Occlusion of the lacrimal puncta can
be corrected surgically. Electrocautery
and other techniques can be used for
permanent punctal occlusion.

24. S.S Could be associated Brain and spinal cord:
with Pachymeningit
Central & peripheral
manifestations : is and
hypophysitis
Myelitis.
DEVIC’S
Peripheral syndrome.
manifestations:
Autonomic
neuropathy
Trigeminal
neuralgia and
glossopharyng
eal neuralgia
Mononeuritis
multiplex.
Designed by :

25. Pachymeningitis /pachy·men·in·gi·tis/ (-men″in-ji´tis) inflammation
of the dura mater.
Pachymeningitis Normal .
Case.

26. Hypophysitis refers to an inflammation of the pituitary gland.
Hypophysitis is rare and not fully understood.

27. Myelitis involves the infection or the inflammation of white matter or
gray matter of spinal cord.

29. Devic's syndrome, is an autoimmune, inflammatory disorder
in which a person's own immune system attacks the optic
nerves and spinal cord.

30. S.S Could be associated Brain and spinal cord:
with Pachymeningit
Central & peripheral
manifestations : is and
hypophysitis
Myelitis.
DEVIC’S
Peripheral syndrome
manifestations:
Autonomic
neuropathy.
Suicide
disease.
Glossopharyng
eal neuralgia.
Mononeuritis
multiplex.
Designed by :

34. Mononeuritis multiplex is a painful, asymmetrical, asynchronous sensory
and motor peripheral neuropathy involving isolated damage to at least 2
separate nerve areas.

37. 41. A 57-year-old woman with a history
of hypertension,arthritis, Xerophthalmia
, and xerostomia. was referred to a
rheumatologist for evaluation.A mini-
chem panel shows no electrolyte
abnormalities. Her erythrocyte
sedimentation rate (ESR) is elevated,
and an antinuclear antibody test (ANA)
is positive. Further antibody studies are
performed, and the results are shown
below.
Anti-histones high titer.
Anti-SSA detected
Anti-SSB detected
Anti-Smith not detected
Anti-centromere not detected
Anti-RNP detected.
Which of the following diseases is
suggested by these results?
(A) CREST syndrome
(B) Diffuse form of scleroderma
(C) Drug-induced lupus
(D) Sjögren syndrome