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Sjögren’s (show-grin) syndrome - a syndrome describing xerophthalmia (dry eyes) and xerostomia (dry mouth)- (Sicca complex)-due to immune-mediated destruction of exocrine glands, predominately of ...

Sjögren’s (show-grin) syndrome - a syndrome describing xerophthalmia (dry eyes) and xerostomia (dry mouth)- (Sicca complex)-due to immune-mediated destruction of exocrine glands, predominately of lacrimal and salivary.

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S. sjogrens S. sjogrens Presentation Transcript

  • Sjögren’s Syndrome 2009
  • OutlineHistory and DefinitionIncidence and EtiologyClinical findingsDiagnosis and PathologyPrognosis and Treatment
  • HistoryMikulicz - first description of Sjögren’s syndromeMikulicz’s syndrome-1892 described parotid and lacrimalgland enlargement associated with a small round cellinfiltrate .However, the term Mikuliczs syndrome could encompassso many different entities including tuberculosis, otherinfections, sarcoidosis, and lymphoma. Therefore notgiven credit for discovery.The term is still occasionally used to describe thehistological appearance of focal lymphocytic infiltrates onsalivary gland biopsies. View slide
  • HistoryHenrick Sjögren 1899-1987 Sweden1930-As ophthalmology resident discover women withrheumatism and corneal abrasions who could not producetears when crying and could not dissolve a lump of sugar intheir mouths.1933-Published his thesis paper on KeratoconjuctivitisSicca, describing 15 women with lacrimal gland dysfunctionleading to ulcerative lesions of the eyes. Was not wellreceived, did not acquire Docenti (Academic PhD).1951-Sjögren published a series of papers describing 80patients with the syndrome, in which the majority had arthritis.Sjögren’s syndrome was then recognized in literature. View slide
  • DefinitionSjögren’s (show-grin) syndrome - asyndrome describing xerophthalmia (dryeyes) and xerostomia (dry mouth)- (Siccacomplex)-due to immune-mediateddestruction of exocrineglands, predominately of lacrimal andsalivary.
  • DefinitionPrimary Sjögren’s syndrome - characterized bySicca complex and extra-glandular symptomswithout any additional connective tissue disorder.Secondary Sjögren’s syndrome occurs inassociation with another autoimmune disordersuch as SLE, RA, or scleroderma
  • IncidenceAs many as 1-2 million people in the US are affected,Prevalence of 1-3% of the population It is in the top three of rheumatic diseases behind systemic lupus erythematosis and rheumatoid arthritisApproximately 30% of RA pt have SS.
  • Primary Sjögren’sSyndrome has a ratio 9:1 of women to men Age range from 40-60, with mean of 52.7 years.However, case reports have been seen in children.
  • EtiologyPathogenesis of Sjögren’s syndrome is believedto be multifactorial.Known to be autoimmune, but studies suggestthat the disease process hasgenetic, environmental and neuroendocrinecomponents
  • EtiologyThere is a genetic predominance of HLA-DRgenotype in SS patients. HLA-DR is a majorhistocompatibility complex, MHC class II cell surfacereceptor. It is found on antigen presenting cells.These genotypes may also produce dysfunctionalglands that secrete abnormal amounts of immune-stimulatory chemokines. This is not seen in patientswithout Sjögren’s disease.
  • EtiologyAutoimmunity involved in Sjögren’s disease is still poorly understood. It isbelieved that genetically defective glandular tissue combined withimmunologic, environmental or neuroendocrine factors leads to loss of selfantigen awareness.Environmental factors may have link to viruses (Epstein–Barr virus, hepatitis Cvirus, human T-cell leukemia virus-1)After the initial trigger, this glandular tissue autoimmune complex then becomesinfiltrated with lymphocytes, predominately CD4 T cell (attracted by MHC class II)A cascade of events then occur with the CD4 T cell release of cytokine IL-1, TNF, and interferon-gamma, which have destructive effects on the tissue andinterfere with acetylcholine release, causing dysfunctional glands.
  • Clinical ManifestationsGlandular Sinus/nasalOcular Hepatobiliary and gastrointestinalOral GenitourinaryOtological MusculoskeletalLaryngeal VascularThyroid NeurologicPulmonary Hematologic/lymphatic
  • Glandular manifestationsDecreased lacrimal flowCorneal and conjunctiva epithelia damageLeads to dry eyes, foreign bodysensation, irritation, photosensitivity, thicksecretions at inner canthus, and visualimpairment.
  • Sjögren’s syndrome dry eye
  • Glandular manifestationsInvolvement of unilateral or bilateral major and minor salivaryglandsDecreased salivary secretions-loss of lubrication, bufferingand antimicrobial capacities of saliva.Most common complication is increase in dentalcaries, especial root and incisor caries.Frequent fungal infectionsTongue fissuresPersistent salivary enlargement.
  • Tooth decay due to xerostomia
  • Acute pseudomembranous candidiasis
  • Parotid Enlargement occurs in 1/3 of SS patients
  • Clinical ManifestationsSS patients typically demonstrate mild to moderatesensorineural hearing loss in high frequencies.An association with anti-cardiolipin antibodies hasbeen demonstrated, but no cochlear or vestibularpathology has been found.May be caused by immune complexes in the striavascularis of the basal turn of the cochlea.
  • Clinical ManifestationsGranulomatous and non-granulomatouslaryngeal nodes have been described.Bamboo nodes - whitish or yellowish transversesubmucosal lesions localized to middle third ofvocal fold.However, hoarseness is rare first symptom
  • Bamboo nodes
  • Clinical Manifestationsthyroid abnormalities such as Hashimotos’sthyroiditis are associated with SSAntibodies to thyroglobulin can be found in up to1/2 of SS patients10-15% of Primary SS are clinically hypothyroidTherefore, all patients with SS should undergoroutine evaluation of thyroid function
  • Clinical ManifestationsEpistaxis and nasal crusting are common findings inSS, with 50% having nasal mucosa hypertrophy andup to 13% have septal perforationsXerotrachea (dry cough) and dyspnea are commonpulmonary symptoms.Esophageal spasm and dysmotility are also seen, butmay be secondary to absence of salivaAtrophic gastritis, celiac disease
  • Clinical ManifestationsLymphoma is a serious complication ofSS, occurring late in the disease.Risk of lymphoma is 44 times greater than thegeneral population.Lymphomas are classified as marginal-zone B-cell lymphomas, caused by chronic stimulationof auto-reactive B cells.
  • Diffuse large B-cell lymphoma in a 30 year-old woman with Sjögren’s syndrome.
  • Clinical Manifestations20-30% of patients have vasculitis, which includespalpable purpura, petechiae, or subcutaneous nodulesRenal involvement in 10% of patients-interstitialnephritis.Renal tubular acidosis also seen which is secondary tohyper-gammaglobulinemiaMyalgias, arthralgias, fatigue, and malaise
  • A) Palpable purpura in the lower extremities.B) Multiple necrotic cutaneous ulcers of the lower extremities in a patient with primary Sjögrens syndromeC) Annular urticarial lesions of the trunk
  • Criteria for Sjögren’s syndrome2002 US and European diagnosis criteria differed so much that there were ten times as many diagnosis in Europe than in US. Caused problems with clinical trials and publication reports 2002 American-European Consensus Group developed criteria for Sjögren syndrome.
  • Criteria for Sjögren’s syndrome2002 Ocular symptoms Oral symptoms Objective evidence of dry eyes Objective evidence of salivary-gland involvement Laboratory abnormality
  • Criteria for Sjögren’ssyndrome1. Ocular Symptoms (at least one must be present)Persistent dry eyes every day for more than 3 monthsRecurrent sensation of sand or gravel in eyesUse of tear substitute more than three time a day2. Oral symptoms (at least one present)Feeling of dry mouth every day for 3 monthsRecurrent swollen salivary glands as an adultNeed to drink liquids to aid in swallowing of dry foods.
  • xerostomia induced glossitis
  • Criteria for Sjögren’ssyndrome3. Objective evidence of dry eyes (one must bepresent)Schirmer TestRose-Bengal4. Lacrimal-gland biopsyFocal lymphocytic sialoadenitis in minor salivary glands-containing more than 50 lymphocytes per 4 mm of glandulartissue.
  • Schirmer’s testThis test consists of placing a small strip of filter paper insidethe lower eyelid (conjunctiva sac). The eyes are closed for 5minutes. The paper is then removed and the amount of moistureis measured. <5 mm in 5 minutes is positive for SS
  • Rose Bengal dyeRose Bengal (4,5,6,7-tetrachloro-2,4,5,7-tetraiodofluorescein) is astain. Its sodium salt is commonly used in eye drops to staindamaged conjunctiva and corneal cells and thereby identify damageto the eye.
  • Labial gland biopsyA gold standard of diagnosis
  • Histopathology of labial gland biopsy
  • Lymphocytic and plasma cells infiltrateTwo excretory ducts and 3 mucous salivary gland acini are seen
  • Criteria for Sjögren’ssyndrome5. Objective evidence of salivary glandinvolvement(one must be present)Salivary-gland scintigraphyParotid sialographyUnstimulated whole sialometry- < 1.5 ml in 15 min
  • ScintigraphyMust show delayed uptake of technetium-99m
  • Criteria for Sjögren’ssyndrome •6. Laboratory Abnormality (one must be present) •Anti-SS-A(Ro) or Anti SS-B(La)(more specific) •ANA •IgM rheumatoid factor
  • LaboratorySS patients of both primary and secondary Sjögren’ssyndrome have marked hypergammaglobulinemia(IgG>IgA>IgM), elevated total protein and sedimentationrate, persistent rheumatoid factors, and a decreased WBCcount.SS-A/Ro and SS-B/La (anti-RNA antibodies). Antibodiesoccur in approximately 60% of patients with Sjögrenssyndrome and are associated with early disease onset, longerdisease duration, parotid gland enlargement, a higherfrequency of extra-glandular manifestations, and more intenselymphocytic infiltrationThese test are nonspecific, also seen in many autoimmuneinflammatory conditions.
  • Indirect immunofluorescein anti-SSA/Ro autoantibodies
  • Criteria for Sjögren’ssyndrome Primary SS is defined as the presence of 4 of the 6 diagnostic criteria present. Secondary SS is defined as presence of connective tissue disease with a positive category 1 or 2 and a positive result in 2 of the remaining 4 criteria
  • Exclusion criteriaPast head and neckradiation treatmentHep C infectionAIDSPre-existing lymphomaor sarcoidosisGraft vs host diseaseAnticholinergic drugs
  • Prognosis and TherapiesAs with all autoimmune disease, there is nocure, and so therapy is aimed at symptomaticrelief.Sjögren’s syndrome is indolent in its course, butone must be aware of the risk of lymphomas inthis patient population.
  • Table 1 Management of glandular manifestations of primary Sjögrens syndrome Mavragani CP et al. (2006) The management of Sjögrens syndrome Nat Clin Pract Rheumatol 2: 252–261 doi:10.1038/ncprheum0165
  • MedicationsOral anti-muscarinic therapy proven useful forxerostomia.Pilocarpine-first muscarinic receptor antagonistapproved by FDA for use in Sjögren syndrome.Dose 5mg qid.Evoxac is next generation muscarinic receptorwith greater affinity, but less cardiac side effectsof anti-muscarinic therapy.
  • MedicationsExtra-glandular disease such as muscularpains, renal and pulmonary disease may benefitfrom use of immune modulator such as systemicsteroids and anti-malarials.However, these therapies have not been provento provide help with sicca symptoms.
  • ConclusionSjögren’s syndrome has early ENTmanifestations-that include dry mouth andparotid enlargement.Otolaryngologist, as part of a multidisciplinaryteam, may prevent prolong delays in the diseaseby having a high degree of suspicion.Otolaryngologist play key role in diagnosticevaluation, biopsy and treatment.
  • Sorry for the drylecture, but this is Sjögren’s syndrome. Questions?
  • ReferencesRehman, Habab-ur Sjögren’s syndrome Yonsei medical journal vol. 44, No. 6, pp 947-954, 2003Fox PC. Autoimmune diseases and Sjogren’s syndrome: an autoimmune exocrinopathy. Ann N Y Acad Sci2007;1098:15–21Mavragani CP, Moutsopoulos NM, Moutsopoulos HM. The management of Sjögrens syndrome. Nat ClinPract Rheumatol. May 2006;2(5):252-61Manoussakis MN, Moutsopoulos HM. Sjögrens syndrome. Otolaryngol Clin North Am 1999;32:843-60Gutta R. McLain L. McGuff SH. Sjögren syndrome: a review for the maxillofacial surgeon. Oral MaxillofacSurg Clin North Am. 2008 Nov;20(4):567-75Clinical Manifestations and Early Diagnosis of Sjögren Syndrome Stuart S. Kassan and Haralampos M.Moutsopoulos Arch Intern Med. 2004;164(12):1275-1284Sjögrens disease. Elizabeth J Mahoney, Jeffrey H Spiegel Otolaryngol Clin North AmAug 2003 (Vol. 36, Issue 4, Pages 733-45)Mavragani CP, Moutsopoulos HMConventional Therapy of Sjogrens Syndrome. [Journal Article]Clin Rev Allergy Immunol 2007 Jun; 32(3):284-91Fox RI. Sjögrens syndrome. Lancet. Jul 23-29 2005;366(9482):321-31Srinivasan S, Slomovic AR Sjogren’s disease Compr Ophthalmol Update. 2007 Jul-Aug;8(4):205-12