Sjogren’s Syndrome

SJOGREN’S SYNDROME
RECURRENT NON-INFECTIOUS PAROTID SWELLING (SEE GEN SALIVARY)
AUTOIMMUNE (PSEUDOSIALECTASIS)
80% B
ANTI-RO
ANTI-SSA, SS-B
SJOGRENS,  BENIGN LYMPHOEPITHELIAL LESION OF GODWIN,  MIKULICZ SYNDROME, CHRONIC PUNCTATE PAROTITIS
ALL CAN BE USED TO DESCRIBE ANY IDIOPATHIC/AUTOIMMUNE RECURRENT PAROTID SWELLING
ALL FELT TO BE SECONDARY TO SOME TYPE OF INSULT—VIRAL INFXN OF THE SALIVARY GLANDS—INSTIGATES A CD-4 AUTOIMMUNE ATTACK ON THE GLANDS
CHRONIC PUNCTATE PAROTITIS
GARTH HEMENWAY, MD
MIKULICZ SYNDROME
VIENNA PHYSICIAN: JOHANN VON MIKULICZ-RADECKI
MOST NOTED FOR HIS OESOPHAGOSCOPE
ALSO DESCRIBED FOAMY HISTIOCYTES IN RHINOSCLEROMA
BENIGN LYMPHOEPITHELIAL LESION OF GODWIN—–JOHN T GODWIN, MD (PATHOLOGIST)
MORE OF A DISTINCT MASS—USUALLY IN THE TAIL OF THE PAROTID
PRIMARY SJOGRENS(SICCA COMPLEX) (HLA- DR3)
HENRICK SJOGRENS
AUTOIMMUNE Dz OF THE EXOCRINE GLANDS WHICH PROGRESSIVELY DESTROYS THEM
XEROPTHALMIA (GRITTY EYE) KERATOCONJUNCTIVITIS SICCA, XEROSTOMIA, PAROTIDITIS, NASAL CRUSTING
PAINLESS, UNILAT?
FEMALE 9:1
AGE 40-60
RARELY ASSOC EDEMA
5% DEV A LYMPHOPROLIFERATIVE NEOPLASM (40%)
HIGH ASSOC WITH NHL(44 X) AND HASHIMOTOS(5%)
SECONDARY SJOGRENS (HLA- DR4)
ASSOC WITH A CT Dz (PRIMARILY R.A.)
AUTOIMMUNE Dz
ANTI – RO        =SJOGRENS, RA, SLE
ANTI -SSA -SSB        =SJOGRENS, RA, SLE
FANA/ANA        =SLE
ANTI -SM        =SLE
ANTI – JO-1                 =POLYMYOSITIS
ANTI -CENTROMERE    =CREST VARIANT OF PSS (SCLERODERMA)
ANTI -SCL -70 (DNA TOPOISOMERASE)    =SCLERODERMA
Dx : LABIAL Bx MINOR SALIVARY GLAND 60% (PAROTID Bx = 100% SENSITIVE)—#INFLAMMATORY CELLS/AREA—-HALMARK = EPIMYOTHELIAL ISLANDS
SCHIRMER’S TEST, SLIT LAMP, 1% ROSE BENGAL STAINING OF CONJUNCTIVA, SALIVARY FLOW RATES
PUNCTATE SIALECTASIS “BUNCH OF GRAPES” ON SIALOGRAPHY
Rx: SYMPTOMATIC (ARTIFICIAL TEARS/SALIVA),?PILOCARPINE, SUGAR FREE SIALOGOGUSE,NASAL SALINE, DENTAL CARE
AVOID DECONGESTANTS, ANTIHISTAMINES, AND DIURETICS.
SOME ADVOCATE TOTAL NERVE SPARRING PAROTIDECTOMY!—-TAKE THE DEEP LOBE!
RECURRENT SIALENITIS–ANTECEDENT DEHYDRATION/ POOR IMMUNE DEFENSES
PRIMARILY STAPH AREUS
Rx WITH REHYDRATION, ANTI STAPH ABX (NAF), WARM COMPRESSES, SIALOGOGUES, ORAL IRRIGATIONS, MASSAGE, PAIN MEDS
SIALOSIS = ENLARGED GLAND
RECURRENT, BILAT, NONTENDER
USUALLY SECONDARY TO DRUGS, UNDERLYING Dz, OR BULEMIA
MULTINODULAR GLAND
SECONDARY TO UNDERLYING Dz—TB, SARCOID (UVEOPAROTID FEVER OF HEERFORDT)
MUMPS
A SINGLE SEROTYPE PARAMYXOVIRUS
QUITE POSSIBLY THE MOST COMMON CAUSE OF UNILAT SNHL(VIRAL SPIRAL GANGLIONITIS)
AFFECTS ALL AGES—MORE COMMON IN CHILDREN OVER THE AGE OF 1
14-18 D INCUBATION PERIOD—-SOMETIMES ASSOC WITH CONVULSIONS IN SMALL CHILDREN
PAROTITIS, SUBANDIBULAR GLANDS, ORCHITIS—–70% BILATERAL
LYMPHOCYTIC MENINGITIS IS RARE
PANCREATITIS, OOPHORITIS, ENCEPHALITIS, THYROIDITIS AND ARTHRITIS ARE RARE SEQUELAE

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