Chap 187 Sjogren Syndrome Flashcards
____ is a chronic, inflammatory, autoimmune disease characterized
by progressive lymphocytic and plasma cell infiltration of the exocrine glands,
especially salivary and lacrimal, with potential for systemic manifestations
Sjögren syndrome
classic
symptoms of ___ (2)
dry eyes (keratoconjunctivitis sicca ) and dry mouth (xerostomia
typically manifests at what age and what gender
typically manifests at 35-45 yr of age, with 90% of cases
among women
mean age at diagnosis in children is ___
gender?
9-10 yr; 75% are
girls.
most common manifestation in children? adult?
Recurrent parotid gland enlargement and parotitis are
the most common manifestations in children (>70%), whereas sicca syndrome
(dry mouth, painful mucosa, sensitivity to spicy foods, halitosis, widespread
dental caries) predominates in adults
current gold standard for diagnosis of SS
diagnosis is based on clinical features supported by
biopsy of salivary or parotid glands demonstrating foci of lymphocytic
infiltration, the
____
directed against an apoptotic cleavage product of α-fodrin, are a useful
diagnostic marker for juvenile Sjögren syndrome.
Anti–β-fodrin autoantibodies,
____ detects
abnormal tear production (≤5 mm of wetting of filter paper strip in 5 min)
Schirmer test
___ detects damaged ocular epithelial conjunctival and corneal cells.
Rose
bengal staining
____ characterized by intermittent unilateral parotid swelling
typically lasting only a few days. It is frequently associated with fever and may
undergo remission with puberty. Unlike in Sjögren syndrome, there is a male
predominance, juvenile recurrent parotitis is seen in the younger children (3-6
yr), and there is a lack of focal lymphocytic infiltrates on biopsy.
juvenile
recurrent parotitis ,
complication: increase risk for
mucosa assocaited lymphoid tissue lymphoma
maternal sjogren syndrome can be an antecendent to the
neonatal lupus
