Diagnosis and Management of Sjogren’s Syndrome

Posted by e-Medical PPT Saturday, December 31, 2011
What is Sjogren’s SyndromeSystemic autoimmune disease characterized by dry eyes and dry mouth.
Other organ systems often affected (extra-glandular manifestations)
May be primary - solitary process
Secondary disease accompanies another autoimmune disease - most often rheumatoid arthritis or SLE

Sjogren Syndrome is the third most common autoimmune disease

Presentation
In a prospective cohort study of 400 patients, 98% presented with dry mouth and 93% presented with dry eyes.2
Associated dry mouth symptoms:  difficulty speaking and eating and swallowing, and frequents sips of water.5
Associated dry eye symptoms:  grittiness, dryness, pruritis, foreign body sensation.
In one study of 195 Dutch patients, 85% reported fatigue12

Extraglandular Manifestations
Arthralgia/non-erosive arthritis characterized by tenderness, swelling, or effusion of peripheral joints
Gastrointestinal symptoms
Autoimmune thyroiditis
Pulmonary disease (chronic cough, recurrent bronchitis with diffuse interstitial infiltrates on radiography, abnormal spirometry, pulmonary alveolitis or fibrosis on computed tomography)
Raynaud’s phenomenon
Cutaneous vasculitis
Peripheral neuropathy
Lymphadenopathy (cervical, axillary, or inguinal)
Renal involvement (proteinuria, renal tubular acidosis, interstitial nephritis, glomerulonephritis, abnormal urinalysis)
Fever not associated with infectious process

Diagnosis of primary Sjogren Syndrome is strongly suggested in a patient with:
Signs and symptoms of oral ocular dryness
Positive antibodies for anti-SS-A and anti-SS-B antigen OR positive salivary gland biopsy
Sjogren syndrome often has an insidious onset, a variable course, and a wide spectrum of clinical manifestations, making diagnosis difficult and delayed.

Revised International Classification Criteria for Sjogren Syndrome
Ocular symptoms (at least one of the following):  daily, persistent, troublesome dry eyes for more than 3 months, recurrent sensation of sand or gravel in eyes, use of tear substitutes more than three time per day
Oral symptoms (at least one of the following symptoms):  daily feeling of dry mouth for more than three months, recurrent or persistently swollen salivary glands as a adult, need to drink liquids frequently to aid in swallowing dry food.
Ocular signs (at least one positive):  Schirmer test, Rose Bengal test or other ocular dye test
Histopathology (positive biopsy of a salivary gland)
Salivary gland involvement (positive results from at least one of the following tests):  unstimulated whole salivary flow collection (less than 1.5ml in 15 minutes); parotid sialography showing the presence of diffuse sialectasia; Salivary scintigraphy showing delayed uptake, reduced concentration and delayed excretion of tracer
Presence of Anti-SSA and Anti-SSB

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