SJOGREN'S SYNDROME
37
Al-Quds University School of Medicine -NEUROSCIENCE- -S.S-presentation- Prepared by : LAYTH HUSSEIN ng semester-2013
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Transcript of SJOGREN'S SYNDROME
Al-Quds UniversitySchool of Medicine-NEUROSCIENCE-
-S.S-presentation-
Prepared by : LAYTH
HUSSEIN
Spring semester-2013
In this
presentation:
The Case.
What is S.S?
I. Overview.
II. Presentation.
III.Diagnosis.
IV.Workup.
V. Treatment.
NEUROLOGY
CORNER.
USMLE
Venus Williams, is an American professional tennis player who is a former World No. 1.She is 32-year-old, African-American female. Was admitted to your clinic suffering severe xerophthalmia (dry eyes), xerostomia (dry mouth), xeroderma and parotid gland enlargement . The erythrocyte sedimentation rate (ESR) was 105 mm/h(N:0-10 mm/h).She complained from severe fatigue episodes while training and playing. Some tests were performed and the results were as shown below:
Schirmer's test : <4 mm wetting of the paper after 5 minutes . N: ≥15-10 mm after 5 minutesPositive salivary gland biopsy findings.Positive anti–SSA & anti–SSB antibodies results.unstimulated salivary flow < 1.5mL in 15minN: 0.3 - 0.4 ml/min (5.25 ml in 15 min)
Venus Williams is suffering from: Sjögren's Syndrome.
Overview
Sjögren's syndrome(S.S) is a systemic, chronic, autoimmune, inflammatory disorder. Characterized by lymphocytic infiltrates in exocrine organs. Most individuals with Sjögren syndrome present with sicca symptoms.
In addition, numerous extraglandular features may develop.
Primary Sjögren syndrome occurs in the absence of another underlying rheumatic disorder, whereas Secondary Sjögren syndrome is associated with another underlying rheumatic disease, such as (SLE), rheumatoid arthritis (RA), or scleroderma. primary and secondary types occur with similar frequency.
M.D HENRY Sjögren.
Etiology:
The etiology of Sjögren syndrome is not well understood.
Autoimmune diseaseongoing interaction between the innate and acquired immune systems.There is presence of activated salivary gland epithelial cells expressing major histocompatibility complex (MHC) class II molecules.
Association with the human leukocyte antigen(HLA)Some evidence indicates that the true association of Sjögren syndrome may be with HLA-DQA1.According to Israeli research ,HLA-DR5 is associated with S.S in Israeli persons.
Possible disease triggersViruses are viable candidates as environmental triggers, uncertain.Epstein-Barr virus (EBV), HTLV-1, human herpesvirus 6 (HHV-6), HIV, hepatitis C virus (HCV), and cytomegalovirus (CMV) may have a role. Glandular pathologyNew studies (Jan-2003) : role of apoptotic mechanisms in the pathogenesis of primary Sjögren syndrome. A defect in Fas-mediated apoptosis, which is necessary for down-regulation of the immune response, can result in a chronic inflammatory destruction of the salivary gland, resembling Sjögren syndrome .
Epidemiology
IN USA: Second most common rheumatologic disorder, behind SLE. Sjögren syndrome affects 0.1-4% of the population.
Between different ethnic groups , the disease occurs worldwide with similar prevalence.
The female-to-male ratio of Sjögren's syndrome is 9:1. -The Journal of Clinical Endocrinology & Metabolism Vol. 94, No. 6 2044-2051(2009)- Dehydroepiandrosterone (DHEA) Role.-2011 -Charles E. Schmidt College of Medicine, Florida- Atlantic University.
Sjögren syndrome can affect individuals of any age but is most common in elderly people. Onset typically occurs in the fourth to fifth decade of life.
Prognosis
Sjögren syndrome carries a generally good prognosis.
Morbidity associated with Sjögren syndrome is mainly associated with the gradually decreased function of exocrine organs.
Patients with primary Sjögren syndrome have a normal life expectancy.
Children born to mothers with antibodies against SSA/Ro are at an increased risk of neonatal lupus and congenital heart block.
Presentation
The first symptoms in primary Sjögren syndrome can be easily overlooked and overlapped.Xerophthalmia (dry eyes) and xerostomia (dry mouth) are the main clinical presentations in adults. Bilateral parotid swelling is the most common sign of onset in children.
Extraglandular involvement in Sjögren syndrome falls into 2 general categories:
Periepithelial infiltrative processes include interstitial nephritis, liver involvement, and bronchiolitis and generally follow a benign course. Extraepithelial extraglandular involvement in Sjögren syndrome is related to B-cell hyperreactivity, hypergammaglobulinemia, and immune complex formation and includes palpable purpura, glomerulonephritis, and peripheral neuropathy .
Sicca symptoms (dry eyes and dry mouth and dry vagina).Fatigue.Parotitis.Cutaneous symptoms Pulmonary symptoms (xerotrachea),which can manifest as a dry cough.Gastrointestinal symptoms.Cardiac symptoms.Renal disorders.A history of recurrent miscarriages or stillbirthsNeurologic symptoms.(see neurology corner).
Diagnosis
Diagnosis of primary Sjögren syndrome requires 4 out of 6 of the below criteria:Ocular symptoms - Dry eyes for more than 3 months, foreign-body sensation, use of tear substitutes more than 3 times daily.
Oral symptoms - Feeling of dry mouth, recurrently swollen salivary glands, frequent use of liquids to aid swallowing.
Ocular signs – Schirmer’s test performed (< 5 mm in 5 min).
Oral signs - Abnormal salivary scintigraphy findings, abnormal parotid sialography findings, abnormal sialometry findings (unstimulated salivary flow < 1.5mL in 15min) .
Positive minor salivary gland biopsy findingsPositive anti–SSA or anti–SSB antibody results
Workup
No single test is sufficiently sensitive or specific in the diagnosis of Sjögren syndrome.
Laboratory test results may indicate the following:•Elevated erythrocyte sedimentation rate (ESR)•Anemia•Hypergammaglobulinemia•Presence of antinuclear antibodies, especially anti-Ro and anti-La•Presence of anti–alpha-fodrin antibody (reliable diagnostic marker of juvenile Sjögren syndrome)•Creatinine clearance may be diminished in up to 50% of patientsSchirmer test.Serum electrophoresis.Sialography and Scintigraph.Biopsy.
Differential diagnosis: ^ ^
SarcoidosisGraft versus host diseaseHIV infectionHTLV-1 infectionHCV infectionKeratoconjunctivitis siccaAnxiety and depressionViral infections (eg, mumps)Complications from contact lensesDehydrationHypervitaminosis ANeurotropic keratitisMucous membrane pemphigoidEnvironmental irritantsMouth breathingChronic blepharitisChronic conjunctivitisRosaceaTherapeutic radiation or surgery to the head and neckAgeAlzheimer disease
Parkinson disease.Bell’s palsy.AmyloidosisSarcoidosisLymphomaViral infection (eg, mumps, Epstein-Barr virus, cytomegalovirus, coxsackievirus A, influenza)DILS associated with HIV diseaseGranulomatous diseases (sarcoidosis, tuberculosis, leprosy)HyperlipoproteinemiaHepatic cirrhosisHepatitis CBulimiaRecurrent parotiditis of childhoodChronic pancreatitisAcromegalyAmyloidosisGonadal hypofunctionDiabetes mellitus
Treatment:
No curative agents for Sjögren syndrome exist. The treatment of the disorder is essentially symptomatic. In secondary Sjögren syndrome, treatment is based on the accompanying disease and its clinical features.
Skin and vaginal drynessPatients should use skin creams, such as Eucerin, or skin lotions, such as Lubriderm.
Arthralgias and arthritisAcetaminophen or nonsteroidal anti-inflammatory drugs (NSAIDs).
Dry Eyes:
•Artificial tears should be applied liberally.
Dry mouth:
•Patients with dry mouth can liberally drink sips of water and take bottled water with them on trips.
Additional treatment considerations:
•In patients with major organ involvement, such as lymphocytic interstitial lung disease, consider therapy with steroids and immunosuppressive agents, such as cyclophosphamide.
•Topical steroids.•Cyclosporine.•Nutritional supplements.
Surgical TherapyOcclusion of the lacrimal puncta can be corrected surgically. Electrocautery and other techniques can be used for permanent punctal occlusion.
Neurology
Corner
S.S Could be associated with Central & peripheral manifestations :
Brain and spinal cord:Pachymeningitis and hypophysitisMyelitis.DEVIC’S syndrome.
Peripheral manifestations:Autonomic neuropathyTrigeminal neuralgia and glossopharyngeal neuralgiaMononeuritis multiplex.Designed by :
LAYTH
Pachymeningitis /pachy·men·in·gi·tis/ (-men″in-ji´tis) inflammation of the dura mater.
Pachymeningitis Case.
Normal .
Hypophysitis refers to an inflammation of the pituitary gland. Hypophysitis is rare and not fully understood.
Myelitis involves the infection or the inflammation of white matter or gray matter of spinal cord.
Devic's syndrome, is an autoimmune, inflammatory disorder in which a person's own immune system attacks the optic nerves and spinal cord.
S.S Could be associated with Central & peripheral manifestations :
Brain and spinal cord:Pachymeningitis and hypophysitisMyelitis.DEVIC’S syndrome
Peripheral manifestations:Autonomic neuropathy.Suicide disease.Glossopharyngeal neuralgia.Mononeuritis multiplex.
Designed by : LAYTH
Mononeuritis multiplex is a painful, asymmetrical, asynchronous sensory and motor peripheral neuropathy involving isolated damage to at least 2 separate nerve areas.
41. A 57-year-old woman with a history of hypertension,arthritis, Xerophthalmia , and xerostomia. was referred to a rheumatologist for evaluation.A mini-chem panel shows no electrolyte abnormalities. Her erythrocyte sedimentation rate (ESR) is elevated, and an antinuclear antibody test (ANA) is positive. Further antibody studies are performed, and the results are shown below. Anti-histones high titer.Anti-SSA detectedAnti-SSB detectedAnti-Smith not detectedAnti-centromere not detectedAnti-RNP detected.
Which of the following diseases is suggested by these results? (A) CREST syndrome(B) Diffuse form of scleroderma (C) Drug-induced lupus(D) Sjögren syndrome(E) Systemic lupus erythematosus (SLE)
