Este o boala autoimuna, in care sistemul imun ataca propriile glande cu secretie interna

Sindromul SjogrenEste o boala autoimuna, in care sistemul imun ataca propriile glande cu secretie interna. Sindromul este unul dintre cele mai prevalente in SUA, afectand nu mai putin de 4.000.000 americani. 9 din 10 pacienti sunt femei. Varsta medie de aparitie este de 40 de ani, desi sindromul Sjogren poate aparea la orice varsta, atat la femei cat si la barbati.La circa 50% din cazuri, sindromul apare singur, in rest apare in asociatie cu alte boli ale tesutului conjunctiv. Cele mai comune 4 afectiuni care coexista cu acest sindrom sunt: artrita reumatoida, lupusul eritematos sistemic, sclerodermia si polimiozita/dermatomiozita. Uneori, cercetatorii se refera la primul tip ca fiind "Sjogren primar", iar la al doilea tip ca fiind "Sjogren secundar". Toate tipurile sunt sistemice si afecteaza intregul organism.

Simptomele caracteristice sunt uscarea conjunctivei si a mucoasei bucale. De asemenea mai poate provoca si deshidratarea altor organe, cum ar fi rinichii, tractul intestinal, vasele sangvine, plamanii, ficatul, pancreasul si sistemul nervos central. Multi pacienti prezinta oboseala si dureri articulare. Simptomele se pot mentine in platou, se pot remite sau se pot inrautati. In timp ce unii pacienti au simptome usoare, altii sufera simptome care le stanjenesc viata de zi cu zi.

Diagnosticarea precoce si tratamentul sunt importante pentru prevenirea complicatiilor. Simptomele sindromului Sjogren se pot suprapune sau mima simptomele altor afectiuni cum ar fi lupusul, artrita reumatoida, fibromialgia, sindromul de oboseala cronica si scleroza multipla. Mai mult, uscaciunea se poate instala si din alte motive, de exemplu ca un efect secundar al medicatiei (ex: antidepresive, antihipertensive).

In plus, din cauza absentei tuturor simptomelor simultan si a faptului ca sindromul Sjogren poate afecta mai multe aparate si sisteme ale organismului, medicii trateaza deseori fiecare simptom individual si le incadreaza in boala sistemica. In medie, timpul scurs de la aparitia bolii pana la instalarea simptomatologiei este de 6 ani. Reumatologii au ca prima responsabilitate diagnosticarea si monitorizarea sindromului Sjogren.

Odata ce a fost suspicionat sindromul, orice medic va cere o serie de analize ale sangelui, care includ:- Anticorpii anti-nucleari (Acestia sunt anticorpi care actioneaza impotriva componentelor normale ale nucleilor celulari. Aproximativ 70% din sindroamele Sjogren au rezultat pozitiv la analiza.)- anticorpii SSA si SSB (Se mai numesc si RO, respectiv LA si sunt deseori identificati in sindromul Sjogren. 70% dintre pacienti au rezultat pozitiv pentru SSA si 40% pentru SSB.)- FR (factorul reumatid) (Acest test imunologic indica o boala reumatoida. In sindromul Sjogren 60-70% dintre pacienti au rezultat pozitiv.)- VSH (Aceasta investigatie masoara gradul de inflamatie. Un VSH ridicat poate indica o dereglare inflamatorie, incluzand sindromul Sjogren.)- Ig (Acestea sunt proteine prezente in mod normal in sange, dar au de obicei titruri crescute in sindromul Sjogren.)Ulterior se pot cere mai multe teste oftalmologice sau stomatologice.

Testele oftalmologice includ:- testul Schrimer (care masoara productia lacrimala)- colorarea cu rosu bengal (pentru identificarea celulelor anormale de la suprafata oculara)

Testele dentale includ:- fluxul glandei parotide (masoara cantitatea de saliva produsa intr-o periada de timp data)- scintigrafia salivara (masoara functia glanedlor salivare)- sialografia (radiografia ductelor glanedlor salivare)- biopsia de buza (pentru a confirma infiltratul limfocitar in glandele salivare mici)

Multe dintre simptomele sindromului Sjogren pot si tratate cu medicamente OTC. Exista de asemenea si solutii pentru simptomele acestiu sindrom care exclud medicatia. De exemplu folosirea unui umidificator. Pacientii invata frecvent unii de la ceilalti in centre de intrajutorare. Sentimentul de izolare, faptul ca nu cunosc si alti oameni cu acelasi sindrom, poate atfel fi depasit cu usurinta.

Medicatia include lacrimi artificiale, saliva artificiala, lotiuni pentru piele fara miros, sprayuri nazale saline, lubrifianti vaginali. Fundatia pentru Sindromul Sjogren are o lista la zi a acestor medicamente. Fundatia ofera de asemenea sfaturi pentru o rutina zilnica in Ghidul pentru Sindromul Sjogren.

In cea de-a 85-a sesiune generala a Asociatiei Internationale pentru Cercetare Stomatologica s-a discutat posibilitatea de a diagnostica sindromul Sjogren pe baza testarii salivei indivizilor. Cercetatorii au evidentiat faptul ca, in locul biopsiilor si al analizelor de sange, saliva poate fi folosita in diagnosticare. Pacientii cu sindrom Sjogren primar au de 40 de ori mai multe sanse sa faca limfoame decat oamenii sanatosi.

Cercetatorii de la Universitatea din California - Los Angeles au identificat o serie de markeri salivari care sunt prezenti in saliva celor cu aceasta afectiune. Folosind tehnologii genetice, au descoperit ca saliva poate oferii informatii concrete si sigure. Este vorba aici de saliva completa, adica un amestec intre saliva din cavitatea bucala si saliva obtinuta din glandele salivare. Mai mult, acesti markeri indica si o distructie celulara glandulara, activitate a raspunsului imun antiviral sau celule programate pentru apoptoza care sunt implicate in sindromul Sjogren. Aceasta metoda de diagnosticare are un avantaj net fata de metodele folosite pana acum si va putea fi folosita in mod curent intr-un viitor apropiat.www.sjogrens.org;www.medicalnewstoday.com

Exista doua tipuri de sindrom Sjgren:

Sindromul Sjgren primar, in care inflamarea glandelor nu se asociaza cu alte tulburari ale tesutului conjunctiv;

Sindromul Sjgren secundar, care se asociaza cu patologii ca:

poliartrita reumatoida;

lupusul eritematos sistemic;

sclerodermia.

Sindromul Sjogren este boal autoimun, n sensul c organismul produce anticorpi mpotriva propriilor celule sau esuturi. Se manifest n principal prin uscciune la nivelul ochilor i la nivelul gurii. Simptomele pe care le descriu pacienii sunt de senzaie de nisip sau alt corp strin n ochi, de arsur la nivelul ochilor, absena lacrimilor n situaii n care ar fi natural ca acestea s apar. n ceea ce privete simptomele de uscciune bucal, pacienii relateaz dificulti n mestecarea i nghiirea alimentelor, necesitatea de a bea ap n timp ce mnnc alimente, probleme dentare de tipul cariilor multiple i frecvente", a explicat medicul.

Potrivit acestuia, dei n general boala este perceput ca fiind rar, nu este chiar aa, prevalena sa crescnd semnificativ odat cu vrsta. Astfel, boala nu este frecvent la adultul tnr, la 20-30 de ani, dar prevalena ncepe s creasc spre vrsta de 40-50 de ani, n special n rndul femeilor.

"Studiile de prevalen, adic de frecven a bolii n populaie, raporteaz date variabile de la o ar la alta sau n funcie de metoda de diagnostic folosit, dar majoritatea autorilor sunt de acord cu creterea prevalenei odat cu vrsta", a susinut dr. Jurcu.

El a precizat c SUUMC poate aborda integrat aceast afeciune att din punctul de vedere al stabilirii diagnosticuluim ct i din punctul de vedere al tratamentului.

"Noi suntem unul dintre spitalele din ar n care putem aborda integrat sindromul Sjogren. Spun asta pentru c diagnosticul i tratamentul necesit deseori o colaborare ntre mai multe specialiti medicale. Diagnosticul necesit o evaluare oftalmologic special, cu msurarea secreiei lacrimale i examene speciale pentru a se vedea dac exist afectare la nivelul corneei. Fiind o boal autoimun n care sunt implicai autoanticorpi, diagnosticul de bazeaz pe dozarea n snge a unor anticorpi specifici (Ro sau SS-A i La sau SS-B). n unele cazuri, se poate face biopsia de glande salivare minore, util pentru a detecta modificrile specifice bolii de la acest nivel. Astfel, n diagnostic sunt implicai alturi de interniti sau reumatologi, medici oftalmologi, specialiti n chirurgie oro-maxilo-facial care ne ajut cu biopsiile de glande salivare minore, serviciul de anatomie patologic care este extrem de important n interpretarea biopsiilor, laboratorul n care realizm analizele necesare diagnosticului", a mai afirmat Ciprian Jurcu.

El a precizat c n cadrul Spitalului Militar exist o colaborare foarte bun cu Secia de medicin nuclear, unde s-a efectuat, la unii pacieni, scintigrafia de glande salivare, pentru a vedea dac funcia glandelor salivare este afectat.

"n plus, unii pacieni pot avea i alte manifestri ale bolii, cum ar fi cele pulmonare sau neurologice, ceea ce nseamn c este nevoie i de o colaborare cu seciile respective. n spitalul nostru, pacienii au acces la toate aceste servicii necesare pentru a putea stabili un diagnostic corect", a mai spus doctorul internist.

Potrivit acestuia, odat stabilit diagnosticul, pacienilor li se prezint, pe de o parte, reguli de via generale pentru a proteja ochii i mucoasa bucal, iar pe de alt parte, se stabilete schema de tratament care se bazeaz pe topice locale la nivel ocular, pe stimulente ale secreiei lacrimale i salivare, imunosupresoare sau tratament cortizonic. Schema de tratament este stabilit n funcie de particularitile fiecrui caz i adaptat acestora.

Dr. Jurcu a mai precizat c tratamentul nu este unul foarte scump i se supune regulilor generale de compensare.

"Trebuie precizat faptul c diagnosticul este stabilit de ctre medicul specialist de medicin intern sau reumatologie dup analiza atent a datelor cazului i dup excluderea altor cauze de uscciune ocular sau bucal. Vreau s subliniez faptul c nu ntotdeauna aceste simptome sunt datorate sindromului Sjogren, existnd o multitudine de alte situaii sau boli n care mai pot fi ntlnite. Tocmai de aceea diagnosticul este unul complex i trebuie efectuat doar ctre medicul specialist", a artat Jurcu.

Potrivit acestuia, boala nu este una foarte grav, dar poate fi invalidant datorit simptomelor de uscciune sau atunci cnd exist manifestri sistemice.

"De asemenea, oboseala cronic, uneori sever, este una dintre problemele principale ale pacienilor. n plus, exist anumite riscuri pe care le implic boala, pacienii necesitnd o supraveghere atent n acest sens", a susinut dr. Jurcu.

Pacienii care au boala prezint un risc crescut pentru apariia limfoamelor comparativ cu populaia general.

"Acest sindrom exist n form sa primar, adic neasociat unei alte boli sau n forma secundar, asociat cu alte boli autoimune cum ar fi lupusul eritematos sistemic, poliartrit reumatoid, sclerodermia sau bolile autoimune tiroidiene", a adugat Ciprian Jurcu.

Nu se cunosc cauzele apariiei bolii, potrivit medicului, existnd pn n acest moment mai multe teorii, astfel c nu au fost identificate modaliti speciale de prevenie.

De asemenea, medicul militar a subliniat importana adresrii la medicul specialist atunci cnd exist simptome care ar putea sugera boala, pentru a se demara un proces de diagnostic specific i pentru a se stabili o schem de tratament adaptat.

AGERPRES/(A autor: Roberto Stan, editor: Florin Marin)

Dry Eye and Sjgrens Syndrome

Millions of Americans suffer from dry eye. There are two main causes: decreased secretion of tears by the lacrimal (tear-producing) glands and loss of tears due to excess evaporation. Both can lead to ocular surface discomfort, often described as feelings of dryness, burning, a sandy/gritty sensation, or itchiness. Visual fatigue, sensitivity to light, and blurred vision are also characteristic of dry eye.

About TearsNormal healthy tears contain a complex mixture of proteins and other components that are essential for ocular health and comfort (Figure 1). Tears are important because they:

Provide nutrients and support the health of cells in the cornea.

Lubricate the ocular surface.

Protect the exposed surface of the eye from infections.

Clear vision depends on even distribution of tears over the ocular surface.

For Sjgrens syndrome patients, inflammation of tear-secreting glands reduces tear production, resulting in chronic dry eye. In addition, changes in the composition of tears contribute to dry eye (Figure 2). In people with dry eye, thin spots in the tear film may appear and the tears no longer adequately protect and support the health of ocular surface tissues.

Diagnosis of Dry EyeTreatment options for dry eye depend on its causes and severity, so it is important to be examined by an eye doctor who is trained to diagnose and treat ocular diseases. The doctor may use tests to assess tear production, tear stability, and tear distribution. A slit-lamp examination using dyes that temporarily stain unhealthy tissue will reveal any abnormality or damage to the ocular surface. These tests typically cause little discomfort and are performed in the doctors office.

Treatments for Dry Eye DiseaseArtificial tears are available over the counter. They can provide temporary relief from dry eye symptoms. Artificial tears contain water, salts, and polymers but lack the proteins found in natural tears (Figure 3). Patients who frequently use drops should choose a brand without preservatives or one with special non-irritating preservatives. Artificial tears are used to treat mild forms of dry eye or to supplement other treatments.

Punctal occlusion blocks the small openings in the eyelid that normally drain tears away from the eye. Usually this is done by inserting plugs made of silicone or other materials into the openings. This simple procedure helps to retain the patients tears on the ocular surface for a longer time. It can improve symptoms and increase comfort for some patients.

Cyclosporine ophthalmic emulsion (Restasis) treats an underlying cause of chronic dry eye by suppressing the inflammation that disrupts tear secretion. Many patients report a noticeable increase in tear production and comfort with continued use of Restasis.

Hydroxypropyl cellulose ophthalmic insert (Lacrisert) is a preservative-free prescription insert that dissolves gently over the course of a day, continually lubricating and protecting the eye while reducing moderate to severe dry eye symptoms with one application daily into the pocket of the lower eyelid.

Topically applied corticosteroids (cortisone) are occasionally prescribed to treat acute episodes of inflammation in dry eye. The use of these medications should be limited in frequency and duration to avoid potential complications of glaucoma and cataract.

Other Treatment Options and ConsiderationsCevimeline (Evoxac) and pilocarpine (Salagen) are medications taken orally to increase salivation in Sjgrens syndrome patients. Recent studies have shown some improvement in dry eye symptoms; however, tear production was either not increased or not measured in these studies. These medications are approved for treating dry mouth; treatment for dry eye is considered an off-label indication for use.

Because excess evaporation of the tearfilm can occur when there is irritation of the eyelids (conditions known as blepharitis or meibomian eyelid gland dysfunction), it is often helpful to maintain eyelid hygiene by using warm compresses and eyelid massage. Any infections of the eyelid margin should be treated with appropriate antibiotics as prescribed by the patients physician. Allergy and certain skin disorders (such as rosacea) also can aggravate dry eye and should be treated appropriately.

There is accumulating evidence to suggest that taking essential fatty acid supplements (Omega 3) by mouth may improve dry eye symptoms and signs. Further clinical trials are underway to confirm this potential benefit. Essential fatty acids are also available in flaxseed oil and fish oil supplements and in some over-the-counter products.

Ongoing clinical trials of other dry eye treatments may eventually result in new FDA-approved treatments for stimulating the production of specific tear components in dry eye patients.

Coping with Dry EyeMaking changes in your environment, habits, and medications can help minimize dry eye symptoms. Here are some suggestions:

Avoid environmental stresses that worsen dry eye, such as low humidity, drafts from air conditioners or fans, smoke, dust, or excessive makeup.

When possible, avoid taking drugs that cause dryness as a side effect, such as certain drugs for blood pressure regulation, antidepressants, and antihistamines (e.g. Benadryl). These drugs and others may decrease tear secretion and worsen dry eye. Your ophthalmologist can help determine whether any drugs you take may be contributing to your condition.

Try blinking on purpose or taking a short break with your eyes closed when reading or working at a computer. We tend to blink less often during these activities, potentially aggravating dry eye.

Wear special glasses or goggles to lessen dry eye. These items decrease tear evaporation by blocking air drafts and increasing humidity around the eyes. Increased humidity has proven to prevent the evaporation of natural and artificial tears.

Use specially-formulated ophthalmic gels or ointments. Although these may blur vision, they can be used overnight to keep eyes moist. Alternatively, use artificial tears before bedtime and in the morning.

Apply warm compresses on the eyes. Compresses can soothe dry, irritated tissues and improve secretion of oil from meibomian glands in the eyelids. Try applying them after waking in the morning and periodically during the day.

Keep your eyes lubricated throughout the day, even if you dont have dryness symptoms. Dont wait until your eyes hurt to seek treatment for dry eye because this could lead to damage to the eye. Patients should use one or more of the treatments listed above and ask their healthcare professional about any FDA-approved medications.

GlossaryBlepharitis: Inflammation of the eyelids, often decreasing secretions from meibomian glands. Excess evaporation of tears can result, leading to dry eye.

Cornea: The clear dome on the front of the eye that covers the pupil and iris. Clear vision depends on a healthy, undamaged cornea.

Lacrimal glands: Glands that secrete the water and most of the important proteins in tears.

Puncta: Small openings on the eyelids into which tears normally drain.

Meibomian glands: Glands in the eyelids that secrete oils. These oils form a thin layer on top of the tear film, retarding evaporation.

Tear film: Protects and lubricates the cornea and the rest of the ocular surface. Natural tears are mostly water containing a complex mixture of proteins and other components.

Dry Eye Brochures Available by Request

The contents of this page come from our Dry Eye Brochure which was created as a collaborative effort between the Foundations Publications Committee and Medical and Scientific Advisory Board. Publication was made possible by an educational grant from Allergan, makers of Restasis, and from the Allergan Foundation.

To download a copy of the Dry Eye Brochure, and to learn additional dry eye information, visit our Dry Eye Resources page.

If you are a doctor who is interested in having a packet of brochures to display in your office, contact the Sjgrens Syndrome Foundation office toll free at (800) 475-6473.

Pictures used with permission from Pflugfelder, SC; Beuerman, RW; Stern, ME, eds. Dry Eye and Ocular Surface Disorders. New York, NY: Marcel Dekker, Inc.; 2004.