What are the clinical hallmarks of Sjögren's syndrome?
keratoconjunctivitis sicca (dry eyes), xerostomia (dry mouth), and parotid gland swelling.
What are some of the extraglandular features of primary Sjögren's syndrome?
include fatigue, Primary Raynaud's phenomenon, polyarthralgia/arthritis, interstitial lung disease, neuropathy, and purpura.
What is the characteristic histopathologic finding of Sjogrens?
autoimmune disorder causing chronic mononuclear cell infiltration of the lacrimal and salivary glands
What is the patient population of Sjogrens?
1.Affects all races and all ages, but onset is greatest in MIDDLE age (40-60)- lupus is younger
2.90% of patients are female
3.Increased incidence of autoimmune disease in family members, especially SLE
Renal disease is infrequently of clinical significance in primary SS. How?
tubular interstitial nephritis,
type I renal tubular acidosis (RIA),
nephrogenic diabetic insipidus.
Not that severe
What are some potential GI symptoms associated with SS?
1/3 have varying degrees of esophageal dysfunction; some have chronic atrophic gastritis; liver involvement can be due to primary biliary cirrhosis, nonspecific hepatitis or autoimmune hepatitis.
What malignancies are particularly common in SS?
NON-HODGKIN’S LYMPHOMA (NHL) has a prevalence of 4.3% in primary SS with a median time to development from diagnosis being 7.5 yrs.;
mucosa-associated lymphoid tissue (MALT) lymphoma occurs mostly in chronic autoimmune disease such as SS
What Lab findings are consistent with SS?
1.Most patients with primary SS test positive for serum ANAs (85%).
2.Approximately 1/3 to ½ have anti-Ro/SS-A and anti-La/SS-B antibodies.
3.5% to 10% of patients with primary SS have low blood levels of C3 and C4.
4.5% to 10% of patients with primary SS have type II or III cryoglobulinemia or a monoclonal gammopathy.
5.5% to 15% have leukopenia or thrombocytopenia.
6. 75 to 95% have positive rheumatoid factor
What is this?
Schirmer’s test using filter paper is used to document decreased tear flow in SS
What causes this in SS?
keratoconjunctivitis sicca- keratitis caused by decreased lacrimal secretions
•Symptoms: dryness of the eyes, foreign body sensation, burning, photosensitivity
How is keratoconjunctivitis sicca best detected in SS?
Best detected by examination of the eyes after instilling rose Bengal dye to highlight epithelial lesions.
Oral manifestations of SS?
•Severe mouth dryness (xerostomia)
•Multiple dental caries
•Fissuring and ulceration of the lips, tongue, and buccal membranes
•Difficulty chewing and swallowing
•Parotid and/or submandibular salivary gland enlargement occurs in 50% of patients and is most often unilateral and episodic
What underlying pathologies place individuals at increased risk of Sjogren's Syndrome?
these would be called secondary SS
What is happening in this salivary gland in SS?
Mononuclear inflammatory infiltrates, interstitial fibrosis, and acinar atrophy of a minor salivary gland in a biopsy of lip is typical for long-standing Sjögren syndrome
How does the salivary gland appear grossly in SS?
the salivary gland is enlarged, white and sometimes admixed with cysts.
What are some other things on the DDx?
-antimuscarinics can cause xerostomia
-diabetes can cause parotid swelling
need all three
Other symptoms of SS?
skin and vaginal dryness
CNS involvement can be involved (anti-aquaporin Abs present with these)
pulmonary involvment also possible
DDx histo for SS
In some cases the lymphocytic infiltrate is polytypic, in keeping with a reactive process (SS). Howebeer, this population can undergo small clonal proliferations and develop into full blown lymphomes (shown here)