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Nursing Faculty Publications College of Nursing

5-2004

Sjogren’s Syndrome: Recognizing and Treating anAutoimmune DiseaseSusan M. DeNiscoSacred Heart University, deniscos@sacredheart.edu

Linda Ferro

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Recommended CitationDeNisco, S. & Ferro, L. (2004). Sjögren’s syndrome: Recognizing and treating an autoimmune disease. American Journal for NursePractitioners, 8(5), 9–21.

SJOGREN'S SYNDROME:Recognizing and Treatingan Autoimmune Disease

Susan DeNisco, APRN, MS, FNP

Linda Ferro, MSN, APRN, FNP

Sjogren's syndrome, one of the most common autoim-

mune diseases, is characterized hy cell-mediated lym-

phocytic infiltration of the exocrine glands, particularly

the salivary and lacrimal glands.' It receives little

attention in the literature, and frequently goes unrecog-

nized until progressive changes are apparent. This arti-

cle aids nurse practitioners in diagnosing the disorder

in its earliest stages and in initiating proper treatment.

Sjogren's syndrome was first identified in 1933 by aSwedish ophthalmologist named Henrik Sjögren, whoreported the association between dryness of the mouth

(xerostomia), dryness of the eyes (xerophthalmia), and chronicdeforming arthritis. It can develop as a primary disorder (clas-sic clinical features include parotid gland enlargement, xerosto-mia, and xerophthalmia), or it may be secondary to anextraglandular connective tissue disease, typically rheumatoidarthritis (RA), systemic lupus erythematosus (SLF), polymyosi-tis, systemic sclerosis, or biliary cirrhosis.'' Primary Sjogren's issometimes referred to as keratoconjunctivitis sicca (KCS), thesicca complex, or the sicca syndrome. In either its primary orsecondary form, Sjogren's syndrome may progress in severityand lead to end-organ damage.

IncidenceSjogren's syndrome affects 2 to 4 million persons in the UnitedStates, predominantly middle-aged women in the peri-menopausal or postmenopausal stage of life (it is 10 times morecommon in females than in males). The mean age at diagnosis is50 years.̂ Sjogren's syndrome occurs in persons of all races, butit is rarely seen in children. Thirty percent of patients withautoimmune rheumatic disease suffer from secondary Sjogren's.'

PathophysiologyThe pathophysiologic process appears to be autoimmune innature. Antibodies produce sensitized lymphocytes, whichselectively seek, infiltrate, and destroy exocrine glandular tis-sue. This phenomenon is characterized clinically by depressedproduction of tears and saliva.̂ Over time, the attack on thebody's immune system may lead to inflammation of internalorgans, causing irreversible damage. The specific cause ofSjogren's remains unknown, but it is likely that genetic, envi-ronmental, and infectious factors are involved.^ Retroviruses,Fpstein-Barr virus (FBV), and, most recently, hepatitis C \arus(HCV) have been implicated as causative pathogens.' Fvidencealso suggests that certain hormones, particularly estrogen, mayinfluence the autoimmune response in a manner simflar to thatnoted in diabetes or thyroiditis."

Clinical PresentationIn clinical practice, two presentations of Sjogren's syndrome areseen most frequently. One is the rapid development of severexerostomia and xerophthalmia, which is frequently accompa-nied by episodic parotid swelling in an otherwise healthy per-son (primary Sjogren's). Female patients are also likely toexperience vaginal dryness. The other presentation is the insid-ious and slowly progressive development of the sicca complex

'.8 No. 5 Moy2004 The Americon Journal for Nurse Proctitioners m 9

Immunology

in patients with RA or another connectivetissue disorder (secondary Sjogren's).'"Because the s)'mptoms of decreased sali-vary and lacrimal function are often sub-tle, the diagnosis is often delayed untilsevere symptoms are present. Other earlymanifestations of secondary Sjögrenssyndrome may include fatigue, arthral-gias, Raynaud's phenomenon (intermit-tent bilateral ischemia of the fingers, toes,and sometimes the ears and nose, withsevere pallor and often paresthesias andpain, usually brought on by cold andrelieved by heat), loss of appetite, andweight loss. It is not uncommon for 8 to10 years to elapse between symptomonset and the development of extraglan-dular complications.'

DiagnosisAs an autoimmune disease, Sjögren'ssyndrome can pose a challenge to pri-mary care practitioners in terms of diag-nosis, particularly in the early stages.An accurate diagnosis of Sjogren's isobtained through a comprehensive his-tory, a focused physical examination,and selective diagnostic testing. Table 1lists signs and symptoms that are char-acteristic of the syndrome. These clinicalfeatures should be considered in tbereview of symptoms when Sjögren's ispart of the differential diagnosis.^ Table2 lists the San Diego diagnostic criteriafor Sjögrens syndrome.' These criteriaare stringent, requiring evidence for anautoimmune process associated withdestruction of salivary and lacrimalgland ttssues. At the other extreme, sev-eral groups (including the Copenhagenand EEC Study group) have based theirdiagnostic criteria for Sjögren's syn-drome on clinical findings of dry eyesand mouth, with no absolute require-ment for gland biopsy or presence ofautoantibodies.' The EEC study groupbelieves that the San Diego criteria iden-tify only the tip of the iceberg—namely,those patients with full-blown disease—and ignore those patients with milderforms of Sjögren's syndrome.'

Differential Diagnosis—Sjögren'ssyndrome is not the only disease processthat can cause lacrimal and salivarygland dysfunction. A comprehensive dif-ferential diagnosis must be considered

TABLE 1 SIGNS AND SYMPTOMS OF SJÖGREN'S SYNDROME^

ORAL/SALIVARY MANIFESTATIONSDry moufhFissures and ulcers of fhe tongue,

buccal mucosa, and lipsPefechial lesions on fhe hard palafeLichen planus-like appearance of

buccal mucosaCobblesfone appearance of fongueInflamed oral mucous membranesParofid gland hardening and/ar

enlargementDental cariesAdherence of food fo mucosal

surfacesDysphagia (especially dry foods)Difficulty chewingReduced salivary flow ratesChanges in or loss of senses of taste

and odorFrequency chronic candidiasis

infectionsInability to speak continuously

OCULAR MANIFESTATIONSForeign body sensationDecreased tear productionInability fo fearLight intoleranceFilamenfs of mucus on corneal

surfaceInflammation, erythemaEye fatigueCorneal ulcérationsIncreased blinkingVisual changes

SYSTEMIC OR EHRAGLANDULARMANIFESTATIONS

Shortness of breathScarring or fibrosisLymphoid infiltrates

GastrointestinalDysphagiaAbnormal esophageal motilityHepatomegalyBiliary cirrhosisDecreased appetiteWeight loss

GenitourinaryVaginal drynessDyspareunia

Hematologic/CirculatoryVasculifisPurpura

LymphoproliferativeGeneralized lymphomasPseudolymphomasMacroglobinemia

RenalRenal fubular acidosisNephrogenic diabefes insipidus

IntegumentaryDry skinChronic inflammafion of sweaf

glandsSkin ulcération, rashesHyperpigmenfafion

NeurologicPolymyasitisPeripheral neuropathyCranial neuropathy (frigeminal)Cerebral vasculifisInability to concenfrafeFafigue/sleep disordersDepression

MusculoskeletalArfhralgiasNonerosive polyarfhritisExtra-articular features of rheumafoid

arthrifis in secondary Sjögren'ssyndrome

Myalgias

1 0 • The Arriericon Joumol for Nurse Procfitioners May 2004 Vol. 8 No. 5

TABLE 2 SAN DIEGO CRITERIA FOR DIAGNOSIS OF SJOGREN'S SYNDROME

The diagnosis of prirriary Sjogren's syndrome is made when two of the followingthree cardinal features are present:'

1. Symptoms and objective signs of ocular dryness (Schirmer's test: <8-mmwetting per 5 minutes, and positive rose bengai staining of cornea todemonstrate keratoconjunctivitis sicca)

2. Symptoms and objective signs of dry mouth

-Decreased parotid flow rate using Lashley cups or other methods; and-Abnormal findings from biopsy of minor salivary gland (based onaverage of four évaluable lobules)

3. Serologie evidence of systemic autoimmunity

-Elevated rheumatoid factor >1:32O; or

-Elevated antinuclear antibody >1:320; or

-Presence of anti-SS-A(RO) or anti SS-B(LA) antibodies

The diagnosis of secondary Sjogren's syndrome is made when characteristicsigns and symptoms of Sjogren's syndrome (as described above) are presentalong with clinical features sufficient to allow for a diagnosis of rheumatoid arthri-tis, systemic lupus erythematosus, polymyositis, scleroderma, or biliary cirrhosis.

for primary care patients presentingwith dry eyes or mouth and parotidgland enlargement. Nurse practitioners(NPs) need to differentiate Sjogren'sfrom other infiltrative processes, auto-immune diseases, and toxic insults(Table 3). Lack of an absolute laboratorymarker of Sjogren's necessitates a diag-nosis based on a combination of factors:the presence of auto-antibodies, otherassessments, and a cUnical correlation.*

Diagnostic Testing—Selective diag-nostic studies are necessary to aid in theaccurate diagnosis of Sjogren's syn-drome. According to the San Diego cri-teria, immunologie tests are notdiagnostic by themselves, but they arevery helpful when interpreted in con-junction with findings from the historyand clinical exam. Once immunologietests are performed during the initialevaluation, they are generally notrepeated often. Primary care providersshould use the following list of laborato-ry studies to differentiate Sjogren's fromother immune disorders:

Minor salivary gland biopsy remainsthe gold standard for definitive diagnosisof Sjogren's syndrome. A probable diag-nosis of the syndrome can be based ondocumented decreased salivary function.'

Sjogren's antibodies are frequentlyfound in patients with Sjogren's syn-drome. In 80% of patients with primarySjogren's, anti-SSA (formerly SS-A/Ro)and anti-SSB (formerly SS-B/La) anti-bodies are present. However, these anti-bodies are not specific to Sjogren'ssyndrome, as they are found in 20% to60% of patients with SLE and in 3% to10% of those with RA.'°

Rheumatoid factor (RF) is com-monly found in patients with RA, but itmay also be found in patients with otherconnective tissues diseases, includingSjogren's syndrome. A positive RF in apatient with Sjogren's syndrome doesnot necessarily indicate that he or shehas RA as well. An elevated RF (>l:320)and the presence of anti-SSA or anti-SSBantibodies provides evidence of sys-temic autoimmune disease.'

Antinuclear antibody (ANA) ismeasured to aid in diagnosing SLE. Anelevated ANA value (>l:320) signals thatpractitioners should continue theirinvestigation for autoimmune involve-ment. The ANA titer may be positive inpatients with Sjogren's as well; 90% ofthose with primary Sjogren's have aspeckled ANA pattern that is typical forthis disorder." However, this test alone

cannot be used to make a definitive diag-nosis of SLE (or Sjogren's syndrome, forthat matter) without other accompany-ing signs and symptoms.'^ Clinical fea-tures and exam findings have a higherdegree of importance in assessing diseaseactivity than does an ANA titer alone.'

Erythrocyte sedimentation rate(ESR) is helpful in identifying patientswho might have a systemic inflammato-ry disorder or a connective tissue disor-ders. An elevated ESR is found inapproximately 70% of patients withSjogren's syndrome.'

Immunoglobulins or gamma glob-ulins are elevated in many patients withSjogren's syndrome, especially whenthere is parenchymal involvement.Immunoglobulin (Ig)G, IgA, and IgMantibodies are helpful in monitoring dis-ease activity, particularly in patientstreated with high-dose prednisone andcyclophosphamide.

Additional Diagnostic Testing—NPs should perform a urinalysis andmeasure blood urea nitrogen and crea-tinine to determine whether patientswith suspected or documentedSjogren's syndrome have renal in-volvement or inflammatory changes.Chest radiograms may help to assessfor subtle signs of inflammation suchas pneumonitis.

Salivary function tests demonstratethe amount of saliva production, and,indirectly, the extent of mucosal dry-ness. Methods of evaluating salivary sta-tus include sialometry, sialography,sialochemistry, and imaging (ultra-sonography, computed tomography,magnetic resonance imaging [MRI]).'"Ultrasonography or MRI of the parotidglands is useful for determining thepresence of glandular cysts, enlarge-ment, and chronic inflammation.Secretory sialography, an invasive proce-dure, is not typically used in primarycare management.^

Confirmation of destructive lympho-cytic infiltration can be obtained bybiopsy of the minor salivary glands inthe lower lip. Lesions appear as clustersof lymphocytes that replace and destroythe secretory acinar tissue. Presence ofthis pattern is characteristic of Sjogren'ssyndrome.

0I.8N0.5 May 2004 The American Journal for Nurse Practitianers » 1 5

Immunology

^ ^ 9 DIFFERENTIAL DIAGNOSIS

PAROTID GLAND ENLARGEMENT

Bilateral

Viral infections: mumps, mononucleo-sis, influenza, EBV, Coxsackie A,CMV, HIV, HCV

SarcoidosisAmyloidosisSjögren's syndromeMetabolic: DM, hyperlipidemia.

chronic pancreatitis, hepaticcirrhosis

TuberculosisAcromegalyAnorexia or bulimia

Unilateral

Salivary gland neoplasm

Bacterial infectionChronic sialadenitisObstructionLymphoma

EBV = Epstein-Barr virus; CMV =cytomegalovirus; HIV = human immuno-deficiency virus; HCV = hepatitis Cvirus; DM = diabetes mellitus.

XEROPHTHALMIA (DRY EYE)

inflammation

Stevens-Johnson syndrome (erythemamultiforme)

PemphigoidConjunctivitisBlepharitisSjögren's syndrome

Neurologic

Impaired lacrimal or eyelid function(cranial nerve impairment)

Blepharospasm (uncontrolled blinkreflex)

TfiYlff UA ft

BurnsDrugs: decongestants, antihistamines.

antihypertensives, acne treatment.muscle relaxants, tranquilizers.oral contraceptives

Other

TraumaHypovitaminosis ALid scarringAnesthetic corneaEpithelial irregularityEnvironmental factors (eg, low

humidity, cigarette smoke expo-sure, high wind environments)

Schirmer's test, which uses filterpaper to evaluate tear formation by thelacrimal gland, is easy for NPs to per-form in practice. A strip of WhatmanNo. 41 filter paper is placed under theconjunctival sac for 5 minutes, afterwhich the moistened portion of thepaper is measured. Wetting of less than5 mm is a strong indicator of diminishedtear secretion (normal tear production is>10 mm). More reliable diagnostic mea-sures include ocular staining with rosebengal dye and biomicroscopy (slit lampevaluation). Staining of either the con-junctiva or cornea, best seen váth thebiomicroscope, indicates that smallsuperficial erosions are present—a signof primary Sjögren's syndrome.

TreatmentOnce NPs make the diagnosis ofSjögren's syndrome, they should use acollaborative approach in terms of

managing the disease in order toenhance patient comfort and preventcomplications. An ophthalmologist anda dentist and/or otorhinolaryngologistshould be consulted for managementissues specific to the patient's sympto-matology. A rheumatologist or immu-nologist may be consulted in patientswith an exacerbation of symptoms orfor re-evaluation. Although Sjögren's isa chronic, incurable illness, nearly allpatients can lead productive lives withproper management.

At the same time, NPs should con-sider the economic burden of the rec-ommended treatment plan. Most of theitems discussed below are available overthe counter (OTC), and are not coveredby insurance. According to the Sjögren'sSyndrome Foundation, a typical patientmay need to spend $625 to $1500 peryear on these products.

Symptom Control—The current

XEROSTOMIA (DRY MOUTH)

Vital infection

latrogenic: Irradiation tohead and neck

Organic Disease

Autoimmune diseaseDMHypertensionDehydrationSarcoidosisHIV infectionSjögren's syndromePsychogenic: depression, anorexia,

bulimia, anxiety

Toxins

Medication: antihistamines, antihyper-tensives; alpha and beta blockers;parasympatholytic agents; antide-pressants, especially tricyclic anti-depressants; muscle relaxants;antispasmodics

treatment approach for patients withSjögren's syndrome is conservative, andis aimed at symptom control. Treatmentcan be considered in three phases:(1) external moisture replacement orcapture (especially important to treat theoral cavity, eyes, nose, skin, and genitaltract); (2) stimulation of endogenoussecretions, (especially important for theoral cavity); and (3) treatment of sys-temic manifestations (ie, pulmonar}' dis-ease, vasculitis, pseudolymphoma)."

Xerophthalmia. The cornerstone oftreatment for xerophthalmia is regularuse of artificial tears, which are used pri-marily to increase patient comfort. Avariety of artificial aqueous or mucoustear preparations, as well as an ocularointment, can be used to ease the dis-comfort of ocular dryness (Table 4).^Artificial tears contain a moisture com-ponent and in some products, a preser-vative. Preservative-free solutions are

1 6 • The Americon Journal for Nurse Proctitioners Moy2004 Vol. 8 No. 5

^ ^ Q TREATMENTS FOR XEROPHTHALMIA'

PRESERVED ARTIFICIAL TEARSAbsorbotears

Duratears Naturale

Hypotears

Liquifilm

Murocel

Tears Plus

NON-PRESERVED TEARSAquaSite

BioTears

Cellufresh

HypoTeors PF

Ocular Ointment

Lacri-Lube

theoretically more prone to bacterialinfection, although many products arepackaged in single-dose vials to reducethis risk. Preserved artificial tears maycause burning or irritation, so alterna-tive products must be considered inthese cases. Artificial tears can be usedas frequently as needed, and they areavailable ÜTC. Some products (eg,lubricating ointments) are more viscousand have longer-lasting effects, but theyalso leave a residue and are less pre-ferred by patients than are the aqueousproducts." They are generally reservedfor nocturnal use because they can causesignificant blurring of vision.

The following guidelines may beuseful in helping patients to find theappropriate product(s) to meet theirneeds: If artificial tears are not lastinglong enough, use a more viscous tear,try a tear with a different osmolality usea tear with a different vehicle, or consid-er referral to an ophthalmologist forpunctal occlusion (see below). If tearsburn after instillation, choose a tear witha different preservative or use a non-pre-served tear. Patients who dwell in low-humidity environments should startusing artificial tears as early in thecourse of their disease as possible.^

If artificial tears do not provide ade-quate lubrication and comfort, ophthal-mologic surgery can be considered.Punctal occlusion can provide tempo-rary blockage with collagen plugs orpermanent occlusion with cattery orlaser treatment.^ The purpose of the pro-cedure is to block the punctum, or tearduct, which carries tears and debrisaway from the surface of the eye.Blocking these drainage ducts preventstears from draining away too quicklyand keeps the eyes lubricated.

Last, patients should consider envi-ronmental factors that contribute to dryeyes and cause irritation, such as low-humidity environments (eg, highly air-conditioned spaces, non-humidifiedheated areas, airplanes), cigarette smoke,and windy conditions. Increasing the useof artificial tears before symptom onsetwill help to alleviate discomfort and toprevent potential corneal abrasion.^ Useof humidifiers is helpful, as well as wear-ing wrap-around sunglasses to decreasetear evaporation when outdoors.

Xerostomia. Dry mouth is a majorcontributing factor to dental caries. It isimportant to teach patients withSjögren's syndrome the importance ofgood oral hygiene and the need to avoidexcess sugars. Topical fluorides are war-ranted to control tooth decay. Patientscan apply fluoride daily, and dentalhygienists can apply it at regular visits toprotect dental enamel. NPs should alsomake sure that patients use their tooth-brush correctly, and that they floss andmassage their gums to remove debrisbetween the teeth. Use of toothpastestargeted at preventing periodontal dis-ease (eg, Retardex, Biotene) can also behelpful* Some patients obtain relief byusing oral sprays containing mucins andglycoprotein (eg, Mouthkote, Salivart).

Some patients may need to makedietary modifications such as ingestingsoft or moist foods only. Others whoretain salivary function may find thatsalivary flow stimulation is helpful:Sugarless gum, hard candy, and mintscan alleviate symptoms and increasesaliva production. Pilocarpine tablets (5mg 4 times daily) can act as a pharmaco-logie stimulant to increase salivary flowfor up to 2 hours after ingestion. Themost common side effects of pilocarpine

are sweating and gastrointestinal intoler-ance, which are controllable by decreasingthe dose. Pilocarpine is contraindicated inpatients with a history of uncontrolledasthma, peptic ulcer disease, acute iritis,narrow angle glaucoma, or unstable car-diovascular disease. Response to pilo-carpine may take up to 6' weeks."Cevimeline, an oral muscarinic agonist,has been shovm to have a longer peakonset of action than does pilocarpine,and it is an effective adjunct to treat-ment. The recommended dosage is 30mg orally 3 times daily.

Excess water consumption canremove mucus from the lining of thebuccal mucosa and should be avoided;patients should be informed that fre-quent small sips of water are preferable

Classic clinical features

of primary Sjögren's

syndrome include

parotid gland

enlargement,

jcerostomîa, and

xerophthalmia.

for symptom control. NPs should alsoeducate patients about avoiding medica-tions (prescription and over-the-counter) that cause dry mouth.

Low-grade oral yeast infections arecommon in patients vidth Sjögren's syn-drome. Topical antifungal troches areuseful in treating this problem.* Toensure optimal results, patients whowear dentures should remove themwhile the antifungal medication is dissolv-ing in the mouth. Dentures must be treat-ed to remove candidal organisms from thesurface. This can be accomplished bysoaking the dentures overnight in a solu-tion of 1/700 dilution of benzalkoniumchloride and then cleaning them

d 8 No. 5 May 2004 The American Journal for Nurse Practitioners « 1 7

Immunology

carefully with a toothbrush. In severecases, oral therapy with a systemic anti-fungal may be necessary.̂

Dry skin. Frequent use of creams orlotions may be helpful for patients expe-riencing dry skin. Vaginal dryness is alsoa problem for women, and can lead todyspareuma. Many different vaginallubricants, including topical estrogen,are available to treat this problem.^

Systemic Manifestations—Whereasmany patients with Sjogren's syndromerequire only local moisturizing therapyfor xerostomia or KCS, others may needsystemic medication for extraglandularinvolvement. The armamentarium fortreating systemic manifestations includesnonsteroidal anti-inflammatory drugs(NSAIDs), corticosteroids, and immuno-modulating drugs (eg, hydroxychloro-quine, methotrexate, azathioprine,cyclophosphamide, gold compounds).Primary care providers must do arisk-benefit analysis for each patientbefore prescribing such agents.

Disease manifestations of arthralgias,myalgias, lymphadenopathy, and fatigueare generally treated with sahcylates,NSAIDs, and frequently hydroxychloro-quine. In fact, the lattermost agent isone of the most commonly prescribedmedications for patients with Sjogren'ssyndrome.^ Although corticosteroidshave no therapeutic role in the glandularaspects of Sjogren's, they are useful inpatients who have life-threatening com-plications of the disease,' including vas-culitis, skin lesions, pneumonitis,neuropathy, and nephritis. Thesepatients are usually very ill, and are hos-pitalized at this point in their illness.Tapering of the steroids must be done withcaution; drugs such as hydroxychloro-quine, azathioprine, and methotrexate areuseful during the tapering process.̂ Manypatients with secondary Sjogren's andunderlying connective tissue disease aretreated with low-dose corticosteroids.Immunomodulating agents are used toinduce remission in patients with acuteinflammatory processes of rheumaticconditions and internal organ involve-ment. By controlling the disease process,these agents may reduce the need forcorticosteroids and allow for their even-tual discontinuation.'"

PrognosisAs with most chronic progressive ill-nesses, the rate of progression ofSjogren's syndrome, either primary orsecondary, is highly variable. In general,the oral and ophthalmologic manifesta-tions of the syndrome do not progress.Some patients experience only mildxerostomia and xerophthalmia, whichthey are able to manage with topicalagents. Others experience cycles of goodhealth alternating with episodes of seri-ous illness that are manifested by pro-gression of the disease. This progressionmay include blurred vision, constant eyediscomfort, recurrent candidal infection,swollen parotid glands, hoarseness, dys-phagia, and difficulty eating. Theseproblems, along with debilitating fatigueand joint pain, contribute to a decreasedquality of life. They may interfere withsimple activities such as watching televi-sion or reading, as well as with patients'ability to work or drive. Furthermore,patient's with primary Sjogren's syndromeare at increased risk for lymphoprolifera-tive disorders, including non-Hodgkin'slymphoma."* Patients with splenomegaly,bilateral parotid enlargement, and a his-tory of radiation treatment are at especial-ly high risk. Secondary Sjogren's canprogress to damage the vital organs of thebody, complicating the illness and thecourse of treatment.

Patient EducationLike any chronic illness, Sjogren's syn-drome can have a profound impact onpatients and family members. All maybeneflt from a close relationship with theprimary care provider, as well as a struc-tured support organization. Helpingpatients to identify their concerns isparamount in their ability to cope withthe illness. Integrating relaxation tech-niques and referral to a mental healthprofessional may be beneficial for selectpatients. As direct caregivers, NPs play akey role in educating patients withSjogren's syndrome. As the disease pro-gresses, they are well suited to counseland coach patients through these transi-tions and to educate patients to maxi-mize the effectiveness and efficiency oftheir treatment regimens. By instructingpatients about proper oral hygiene to

minimize the risk of oral candidiasis andabout proper eye care to prevent dry-ness, NPs can have a profound impacton patient adherence and quality of care.

ResearchMuch research has been performed onthe genetic basis of Sjogren's syndromeand the nature of the immune compo-nent. New data are suggestive of viruses(eg, retroviruses, EBV, HCV) playing arole in pathogenesis. Numerous clinicaltrials are now recruiting patients toincrease clinicians' understanding ofhormonal underpinnings, diagnostictechniques, pharmacotherapy, and thelong-term effects of the disease.

ConclusionSjogren's syndrome is one of the mostcommon autoimmune diseases, yet itcan go undiagnosed for 8 to 10 yearsbecause of the nature of the signs ands^Tuptoms. NPs must be able to recog-nize the clinical features of the syn-drome, to recommend appropriatestudies to diagnosis it, to begin treat-ment, and to make appropriate referrals.Easing symptoms and preventing com-plications are essential to improvinglong-term outcomes in the populationaffected by Sjogren's syndrome.Understanding family dynamics andencouraging family involvement arevital components to the care of anypatient with a chronic illness. NPs mustthink globally in order to educatepatients, families, collaborating clini-cians, and researchers to advance therecognition and treatment of thisautoimmune disease.

Susan DeNisco is an assistant clinical pro-fessor and coordinator of the Family NursePractitioner Program at Sacred HeartUniversity in Fairfield, Connecticut, andshe practices at Southwest CommunityHealth Center in Bridgeport, Connecticut.Linda Ferro is a nurse practitioner in theSection of Endocrinology at NorwalkHospital, Norwalk, Connecticut.

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'.8 No. 5 Moy2004 Tbe American Journal for Nurse Proctitioners m 2 1

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