Sjogrens syndrome

Question 1

Sjogrens typical patient

Answer 1

Women, 9:1 ratio,
manifestion at peri-menopausal age.

Or, in conjunction with:
RA
Scleroderma
SLE
Polymositis

Question 2

Sjogrens specific symptoms

Answer 2

Autoimmune exocrinopathy and epithelitis

Specific symptoms

• Dry eyes and mouth
• Also almost always has systemic symptoms

Question 3

Autoantibodies associated with Sjogrens

Answer 3

Anti-Nuclear Antibodies in 95% (very sensitive, very non-specific)
RF in 75%

Specific ones:
anti-SSA/Ro, and anti-SSB/La
Anti-Sjogrens syn. antigens A and B, nuclear riboproteins.

Question 4

Official diagnosis of Sjogrens

Answer 4

score of 4 or higher on the EULAR/ACR classification

• minor salivary gland biopsy with lymphocytic infiltrate -=3
• SSA or SSB autoantibodies =3
• Ocular staining score 5 or higher on either eye =1
• Schirmer test less than 5mm/5min on either eye =1
• Unstimulated salivary flow <0.1ml/min = 1

Question 5

What is the test with filter paper in the eyes?

Answer 5

Schirmer test

Question 6

What agents are used for ocular staining

Answer 6

Fluoroscein to stain the cornea, revealing areas of damage or foreign bodies. undamaged corneas have no stain. Punctate stain shows significant damage

Lissamine-green to stain the conjunctiva. Vital stain excluded by healthy cells. Staining dead or degenerating cells, more eye damage indication

Question 7

Primary vs Secondary sjogrens

Answer 7

Primary: Dry eyes and mouth, with lymphocytic infiltration of salivary glands, no other autoimmune disease

Secondary: along with connective tissue disease, RA, Scleroderma, SLE, polymyositis

Question 8

Systemic symptoms of Sjogrens

Answer 8

• Fatigue
• Non-erosive polyarthritis
• Interstitial nephritis, and renal tubular acidosis
• Vasculitis
• Focal myositis
• Pneumonitis
• Generalized lymphadenopathy

-Drastically elevated risk of non-hodgkins lymphoma!!!
diffuse large B-cell lymphoma, marginal zone B cell lymphoma

Question 9

Sjogrens treatment

Answer 9

Pilocarpine or Cimevelin for dry eyes
Artificial tear drops

Artificial saliva or Nacetyl cysteine solutions to dilute oral mucus

NSAIDs or Steroids for arthralgia
Cyclosporin A for arthralgia