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19 Cards in this Set

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What is Sjögren's syndrome?
a chronic immune-mediated, inflammatory disorder of exocrine gland dysfunction
What are the most common manifestations of Sjögren's syndrome?
• inflammation and destruction of the lacrimal and salivary glands (leading to dry eyes)
• dry mouth (xerostomia)
What is keratoconjunctivitis sicca or xerophthalmia?
aka dry eyes
What are systemic features of Sjögren's syndrome?
• interstitial lung disease
• lymphoma
• vasculitis
Differentiate between primary and secondary Sjögren's syndrome
• Primary: no underlying immunologic disorder is present
• Secondary: another immunologic disorder is present (ex. SLE, RA, Scleroderma, primary biliary cirrhosis)
Sjorgen's syndrome is more common in which gender?
females
What human leukocyte antigens (HLA) are associated with Sjorgen's syndrome?
• HLA-DR2
• HLA-DR3
What are the 2 categories of clinical features of Sjorgen's syndrome?
• exocrine gland dysfunction
• extraglandular manifestations
List examples exocrine gland dysfunction associated with Sjorgen's syndrome
• problems secondary to lacrimal gland dysfunction (ex. dry eyes, corneal abrasions, erythematous eyes)

• problems sceondary to salivary gland dysfunction

• problems secondary to other exocrine dysfunction (ex. dysparenunia, pancreatic malabsorption, pancreatitis)
List examples of problems that are secondary to salivary gland dysfunction
• dental caries
• dry mouth
• dysphagia
• gastroesophageal reflux
• lingual and labial fissures
• oral sores
• parotid and/or submandibular gland swelling
List examples of extraglandular features of Sjorgen's syndrome
• CNS (ex. MS, stroke, dementia, myelitis)
• endocrine (ex. hypothyroidism(
• liver (ex. hepatomegaly, primary biliary cirrhosis)
• musculoskeletal (ex. polymyositis, polyarthralgias, polyarthritis)
• peripheral nervous system (ex. peripheral sensorimotor neuropathy)
• pulmonary (ex. chronic bronchitis, interstitial pneuomonitis/fibrosis, COPD, etc.)
• renal (ex. tubulointersitial nephritis)
• reticuloendothelial system (ex. splenomegaly, lymphadenopathy)
• skin & mucous membranes (ex. xerosis, purpura, photosensitive lesions)
• vascular (ex. Raynaud's, small vessel vasculitis)
Which extraglandular manifestation is of most concern in Sjorgen's syndrome?
a marked increase in the prevalence of lymphoma
What are common criteria in diagnosing Sjorgen's syndrome?
• subjective & objective evience of dry eyes and dry mouth
• presence of at least one of the following autoantibodies (ANA, rheumatoid factor, anti-Ro, anti-La)
• exclusion of underlying disease that mimics Sjorgen's
What is a Schirmer's filter paper test?
• used to objectively document keratoconjunctivitis sicca by measuring decreased tear production
• < 5 mm of wetting in 5 minutes after placement in the lower eyelid
What test can be used to demonstrate reduce salivary production?
provacative sialometry
What are other differentials to consider in patients with Sjorgen's syndrome?
• hepatitis B
• HIV
• human T-cell leukemia virus
• infiltrative disease (ex. sarcoidosis, amyloidosis, hemochromatosis)
• multiple sclerosis
• mycobacteria infection
• syphilis
What are some examples of drug classes that have anticholinergic properties that can cause dry eyes and dry mouth (which can mimic Sjorgen's syndrome)?
• antidepressants
• antihypertensives
• decongestants
What are systemic treatment options for exocrine dysfunction?
pilocarpine or cevimeline
What are treatment options for the systemic manifestations of Sjorgen's syndrome?
• NSAIDs
• antimalarial drugs (ex. hydroxchloroquine, cloroquine)
• glucocorticoids
• immunosuppresive agents