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TEORI AUTOIMUNITAS A.

Pendahuluan Dalam keadaan normal, sistem imun dapat membedakan antigen tubuh sendiri dari antigen asing, karena tubuh mempunyai toleransi terhadap sel antigen! tetapi pengalaman klinis menun"ukkan bah#a adakalanya timbul reaksi autoimunitas! Reaksi autoimunitas adalah reaksi system imun terhadap antigen sel "aringan sendiri! Antigen tersebut disebut autoantigen, sedang antibody yang dibentuk disebut autoantibody! Sel autoreakti adalah lim osit yang mempunyai reseptor untik autoantigen! $ila sel tersebut memberikan respons autoimun, disebut sel lim osit reakti %S&R'! (ada orang normal , meskipun S&R berpasangan dengan autoantigen, tidak selalu ter"adi respon autoimun, karena ada system yang mengontrol reaksi autoimun! )adang*kadang tidak "elas apakah autoantibody tersebut merupakan penyebab atau timbul sekunder akibat suatu penyakit! Oleh karena itu harus dibedakan antara enomena autoimun dengan penyakit autoimun! Reaksi autoantibody dan autoantigen yang menimbulkan kerusakan "aringan dan ge"ala*ge"ala klinis disebut penyakit autoimun, sedangkan bila tidak disertai ge"ala klinis disebut enomena autoimun! $urnett menga"ukan teori orbidden +lones, yang menyatakan bah#a tubuh men"adi toleran terhadap "aringannya sendiri oleh karena sel*sel yang autoreakti selama perkembangan embriologiknya akan musnah! B.Teori-teori autoimunitas ,!Teori se-uestered antigen atau hidden antigen Se-uestered aatau hidden antigen adalah antigen yang karena sa#ar anatomi+ tdk pernah berhubungan dengan system imun misalnya antigen sperma, lensa mata, dan sara pusat! $ila sa#ar tersebut rusak, dapat timbul penyakit autoimmun .!Teori de esiensi immun /ilangnya sel toleran+e mungkin disebabkan oleh karena adanya gangguan system lim oid! (enyakit autoimmune sering ditemukan bersamaan dengan de esiensi imun, misalnya pada lan"ut usia 0!Determinan antigen baru (embentukan autoantibody dapat di+etuskan oleh karena timbul deterrminan antigen baru pada protein normal! 1ontoh autoantibody yang timbul akibat hal tersebut ialah a+tor rematoid %2R'! 2R dibentuk terhadap determinan antigen yang terdapat pada immunoglobulin 3!Reaksi silang dengan mikroorganisme )erusakan "antung pada demam reumatik anak diduga ter"adi akibat produksi antigen terhadap streptokok A yang bereaksi silang dengan miokard penderita

4!5irus sebagai pen+etus autoimunitas 5irus yang terutama menggin eksi system lim oid dapat tmempengaruhi mekanisme kontrol imunologik sehingga ter"adi autoimunitas 6!Autoantibodi dibentuk sekunder akibat kerusakan "aringan Autoantibodi terhadap "antung ditemukan pada "antung in ark! (ada umumnya kadar autoantibody disini terlalu rendah untuk dapat menimbulkan penyakit autoimmun! Autoantibody dapat dibentuk pula terhadap antigen mitokondria pada kerusakan hati atau "antung! (ada tuber+ulosis dan tripanosomiasis yang menimbulkan kerusakan luas pada berbagai "aringan, dapat pula ditemukan autoantibody terhadap antigen "aringan dalam kadar gula yang rendah 1!(embagian (enyakit Autoimmun (enyakit autoimmun dapat dibagi mena"di . golongan yaitu organ spesi ik dan non spesi ik Organ spesi ik Non organ spesi ik Tiroiditis hasimoto Miksidema primer Tirotoksotosis Anemia pernesiosa 7astritis kronik autoimun (enyakit Addison Menopause premature Disbetes "u8enile Sindorm goodpasture Miastenia gra8is In ertilitas pada pria (em igus 8ulgaris (em igoid O talmia simpatis U8eitis 8agogenik S+lerosis multiple Anemia hemolitik autoimun (urpura trombositopenik idiopatik &eu+openia idiopatik Sirosis bilier primer /epatitis kronik akti dengan /$sAg negati8e Sirosis kriptogenik 1olitis ulserati Sindrom S"9gren Arthritis rematoid

Dermatomiositis S+leroderma &E dis+oid &upus eritermatosus sistemik %S&E' D!)riteria (enyakit Autoimun )riteria untuk menegakkan diagnosis penyakit autoimmun adalah sebagai berikut : ,!(enyakit timbul akibat adanya respons autoimun .!Ditemukan autoantibody 0!(enyakit dpat ditimbulkan oleh bahan yang diduga merupakan antigen 3!(enyakit dapat dipindahkan dari satu binatang ke binatang yang lain melalui serum atau lim osit yang hidup What is the immune system? The immune system is the body;s means o prote+tion against mi+roorganisms and other < oreign< substan+es! It is +omposed o t#o ma"or parts! One +omponent, $ lympho+ytes, produ+es antibodies, proteins that atta+k < oreign< substan+es and +ause them to be remo8ed rom the body= this is sometimes +alled the humoral immune system! The other +omponent +onsists o spe+ial #hite blood +ells +alled T lympho+ytes, #hi+h +an atta+k < oreign< substan+es dire+tly= this is sometimes +alled the +ellular immune system! It takes time or both +omponents o the immune system to de8elop! T lympho+ytes be+ome prote+ti8e, and antibodies are de8eloped a ter a person is e>posed to spe+i i+ < oreign< threats! O8er a li etime, the immune system de8elops an e>tensi8e library o identi ied substan+es and mi+roorganisms that are +ataloged as ?threat@ or ?not threat!@ 5a++inations utiliAe this pro+ess to add to the library! They e>pose a personBs immune system to #eakened or ina+ti8ated orms o ba+teria and 8iruses that +an no longer +ause disease, so that the personBs immune system #ill re+ogniAe them and +reate antibodies that #ill be ready to prote+t against the in e+tious orms o these mi+roorganisms i the person +omes in +onta+t #ith them in the uture! Normally, the immune system +an distinguish bet#een ?sel @ and ?not sel @ and only atta+ks those tissues that it re+ogniAes as ?not sel !@ This is usually the desired response, but not al#ays! Chen a person is gi8en an organ transplant, the immune system #ill +orre+tly re+ogniAe the ne# organ as ?not sel @ %unless it is rom an identi+al t#in' and #ill atta+k it in a pro+ess +alled re"e+tion! To pre8ent re"e+tion, the transplant patient must take drugs that redu+e the a+ti8ity o the immune system %immunosuppressants' or the rest o his li e!

What are autoimmune disorders? Autoimmune disorders are diseases +aused by the body produ+ing an inappropriate immune response against its o#n tissues! Sometimes the immune system #ill +ease to re+ogniAe one or more o the bodyBs normal +onstituents as ?sel @ and #ill +reate autoantibodies D antibodies that atta+k its o#n +ells, tissues, andEor organs! This +auses in lammation and damage and it leads to autoimmune disorders! The +ause o autoimmune diseases is unkno#n, but it appears that there is an inherited predisposition to de8elop autoimmune disease in many +ases! In a e# types o autoimmune disease %su+h as rheumati+ e8er', a ba+teria or 8irus triggers an immune response, and the antibodies or T*+ells atta+k normal +ells be+ause they ha8e some part o their stru+ture that resembles a part o the stru+ture o the in e+ting mi+roorganism! Autoimmune disorders all into t#o general types: those that damage many organs %systemi+ autoimmune diseases' and those #here only a single organ or tissue is dire+tly damaged by the autoimmune pro+ess %lo+aliAed'! /o#e8er, the distin+tions be+ome blurred as the e e+t o lo+aliAed autoimmune disorders re-uently e>tends beyond the targeted tissues, indire+tly a e+ting other body organs and systems! Some o the most +ommon types o autoimmune disorders in+lude:

Systemic Autoimmune Diseases Rheumatoid arthritis (RA) and Juvenile RA (JRA) (joints; less commonly lung, skin)

Localized Autoimmune Diseases y!e " Dia#etes $ellitus (!ancreas islets)

Lu!us %Systemic Lu!us (ashimoto)s thyroiditis, *raves) &rythematosus' (skin, joints, disease (thyroid) kidneys, heart, #rain, red #lood cells, other) Scleroderma (skin, intestine, less commonly lung) +eliac disease, +rohn)s disease, ,lcerative colitis (*- tract)

Sjogren)s syndrome (salivary glands, $ulti!le sclerosis. tear glands, joints) *ood!asture)s syndrome (lungs, kidneys) /egener)s granulomatosis (#lood vessels, sinuses, lungs, kidneys) 0olymyalgia Rheumatica (large muscle grou!s) *uillain34arre syndrome (nervous system) Addison)s disease (adrenal) 0rimary #iliary cirrhosis, Sclerosing cholangitis, Autoimmune he!atitis (liver) em!oral Arteritis 1 *iant +ell Arteritis (arteries o2 the head and neck)

. here is still some de#ate as to 5hether $S is an autoimmune disease

2or a more +omplete list o autoimmune +onditions, 8isit the (atient In ormation page o the Ameri+an Autoimmune Related Diseases Asso+iation, In+! In some +ases, a person may ha8e more than one autoimmune disease= or e>ample, persons #ith Addison;s disease o ten ha8e type , diabetes, #hile persons #ith s+lerosing +holangitis o ten ha8e ul+erati8e +olitis! In some +ases, the antibodies may not be dire+ted at a spe+i i+ tissue or organ= or e>ample, antiphospholipid antibodies +an rea+t #ith +lotting proteins in the blood, leading to ormation o blood +lots #ithin the blood 8essels %thrombosis'! Autoimmune disorders are diagnosed, e8aluated, and monitored through a +ombination o autoantibody blood tests, blood tests to measure in lammation and organ un+tion, +lini+al presentation, and through non*laboratory e>aminations su+h as F*rays! There is +urrently no +ure or autoimmune disorders, although in rare +ases they may disappear on their o#n! Many people may e>perien+e lare*ups and temporary remissions in symptoms, others +hroni+ symptoms or a progressi8e #orsening! Treatment o autoimmune disorders is tailored to the indi8idual and may +hange o8er time! The goal is to relie8e symptoms, minimiAe organ and tissue damage, and preser8e organ un+tion! Ne# treatments and a greater understanding o autoimmune disorders are being resear+hed! (atients should talk to their do+tors and to any spe+ialists they are re erred to about their treatment options!

What is lupus? What are the types of lupus?


&upus is an autoimmune disease +hara+teriAed by a+ute and +hroni+ in lammation o 8arious tissues o the body! Autoimmune diseases are illnesses that o++ur #hen the body;s tissues are atta+ked by its o#n immune system! The immune system is a +omple> system #ithin the body that is designed to ight in e+tious agents, su+h as ba+teria and other oreign mi+robes! One o the #ays that the immune system ights in e+tions is by produ+ing antibodies that bind to the mi+robes! Patients with lupus produce abnormal antibodies in their blood that target tissues within their own body rather than foreign infectious agents. Because the antibodies and accompanying cells of inflammation can affect tissues anywhere in the body, lupus has the potential to affect a variety of areas! Sometimes lupus +an +ause disease o the skin, heart, lungs, kidneys, "oints, andEor ner8ous system! Chen only the skin is in8ol8ed, the +ondition is +alled lupus dermatitis or +utaneous lupus erythematosus! A orm o lupus dermatitis that +an be isolated to the skin, #ithout internal disease, is +alled dis+oid lupus! Chen internal organs are in8ol8ed, the +ondition is re erred to as systemi+ lupus erythematosus %S&E'! $oth dis+oid and systemi+ lupus are more +ommon in #omen than men %about eight times more +ommon'! The disease +an a e+t all ages but most +ommonly begins rom .G to 34 years o age! Statisti+s demonstrate that lupus is some#hat more re-uent in A ri+an Ameri+ans and people o 1hinese and Hapanese des+ent!

What causes lupus? Is it hereditary?


The pre+ise reason or the abnormal autoimmunity that +auses lupus is not kno#n! Inherited genes, 8iruses, ultra8iolet light, and +ertain medi+ations may all play some role! 7eneti+ a+tors in+rease the tenden+y o de8eloping autoimmune diseases, and autoimmune diseases su+h as lupus, rheumatoid arthritis, and autoimmune thyroid disorders are more +ommon among relati8es o patients #ith lupus than the general population! Some s+ientists belie8e that the immune system in lupus is more easily stimulated by e>ternal a+tors like 8iruses or ultra8iolet light! Sometimes, symptoms o lupus +an be pre+ipitated or aggra8ated by only a brie period o sun e>posure! It also is kno#n that some #omen #ith S&E +an e>perien+e #orsening o their symptoms prior to their menstrual periods! This phenomenon, together #ith the emale predominan+e o S&E, suggest that emale hormones play an important role in the e>pression o S&E! This hormonal relationship is an a+ti8e area o ongoing study by s+ientists!

ore recently, research has demonstrated evidence that a !ey en"yme#s failure to dispose of dying cells may contribute the development of $%&. The en"yme, '(ase), normally eliminates what is called *garbage '(A* and other cellular debris by chopping them into tiny fragments for easier disposal. +esearchers turned off the '(ase) gene in mice. The

mice appeared healthy at birth, but after si, to eight months, the ma-ority of mice without '(ase) showed signs of $%&. Thus, a genetic mutation in a gene that could disrupt the body#s cellular waste disposal may be involved in the initiation of $%&.
In S&E, the body;s immune system produ+es antibodies against itsel , parti+ularly against proteins in the +ell nu+leus! S&E is triggered by en8ironmental a+tors that are unkno#n! <All the key +omponents o the immune system are in8ol8ed in the underlying me+hanisms< o S&E, a++ording to Rahman, and S&E is the prototypi+al autoimmune disease! The immune system must ha8e a balan+e %homeostasis' bet#een being sensiti8e enough to prote+t against in e+tion, and being too sensiti8e and atta+king the body;s o#n proteins %autoimmunity'! 2rom an e8olutionary perspe+ti8e, a++ording to 1ro#, the population must ha8e enough geneti+ di8ersity to prote+t itsel against a #ide range o possible in e+tion= some geneti+ +ombinations result in autoimmunity! The likely en8ironmental triggers in+lude ultra8iolet light, drugs, and 8iruses! These stimuli +ause the destru+tion o +ells and e>pose their DNA, histones, and other proteins, parti+ularly parts o the +ell nu+leus! $e+ause o geneti+ 8ariations in di erent +omponents o the immune system, in some people the immune system atta+ks these nu+lear*related proteins and produ+es antibodies against them! In the end, these antibody +omple>es damage blood 8essels in +riti+al areas o the body, su+h as the glomeruli o the kidney= these antibody atta+ks are the +ause o S&E! Resear+hers are no# identi ying the indi8idual genes, the proteins they produ+e, and their role in the immune system! Ea+h protein is a link on the autoimmune +hain, and resear+hers are trying to ind drugs to break ea+h o those links! I.JI0KJI3GJ S&E is a +hroni+ in lammatory disease belie8ed to be a type III hypersensiti8ity response #ith potential type II in8ol8ement

What is drug-induced lupus?


DoAens o medi+ations ha8e been reported to trigger S&E! /o#e8er, more than KGL o this <drug*indu+ed lupus< o++urs as a side e e+t o one o the ollo#ing si> drugs: hydralaAine %used or high blood pressure', -uinidine and pro+ainamide %used or abnormal heart rhythms', phenytoin %used or epilepsy', isoniaAid %INydraAid, &aniaAidJ used or tuber+ulosis', d*peni+illamine %used or rheumatoid arthritis'! These drugs are kno#n to stimulate the immune system and +ause S&E! 2ortunately, drug*indu+ed S&E is in re-uent %a++ounting or less than 4L o all patients #ith S&E' and usually resol8es #hen the medi+ations are dis+ontinued!

What are the symptoms and signs of lupus?


(atients #ith S&E +an de8elop di erent +ombinations o symptoms and organ in8ol8ement! 1ommon +omplaints and symptoms in+lude atigue, lo#*grade e8er, loss o appetite, mus+le a+hes, arthritis, ul+ers o the mouth and nose, a+ial rash %<butter ly rash<', unusual sensiti8ity to sunlight %photosensiti8ity', in lammation o the lining that surrounds the lungs %pleuritis' and the heart %peri+arditis', and poor +ir+ulation to the ingers and toes #ith +old e>posure %Raynaud;s phenomenon'! 1ompli+ations o organ in8ol8ement +an lead to urther symptoms that depend on the organ a e+ted and se8erity o the disease! Skin mani estations are re-uent in lupus and +an sometimes lead to s+arring! In dis+oid lupus, only the skin is typi+ally in8ol8ed! The skin rash in dis+oid lupus o ten is ound on the a+e and s+alp! It usually is red and may ha8e raised borders! Dis+oid lupus rashes are usually painless and do not it+h, but s+arring +an +ause permanent hair loss! O8er time, 4L*,GL o patients #ith dis+oid lupus may de8elop S&E! O8er hal o the patients #ith S&E de8elop a +hara+teristi+ red, lat a+ial rash o8er the bridge o their nose! $e+ause o its shape, it is re-uently re erred to as the <butter ly rash< o S&E! The rash is painless and does not it+h! The a+ial rash, along #ith in lammation in other organs, +an be pre+ipitated or #orsened by e>posure to sunlight, a +ondition +alled photosensiti8ity! This photosensiti8ity +an be a++ompanied by #orsening o in lammation throughout the body, +alled a < lare< o the disease!

Typi+ally, this rash +an heal #ithout permanent s+arring #ith treatment!

Most patients #ith S&E #ill de8elop arthritis during the +ourse o their illness! Arthritis in S&E +ommonly in8ol8es s#elling, pain, sti ness, and e8en de ormity o the small "oints o the hands, #rists, and eet! Sometimes, the arthritis o S&E +an mimi+ that o rheumatoid arthritis %another autoimmune disease'! More serious organ in8ol8ement #ith in lammation o++urs in the brain, li8er, and kidneys! Chite blood +ells and blood*+lotting a+tors also +an be +hara+teristi+ally de+reased in S&E, kno#n as leu+openia and thrombo+ytopenia, respe+ti8ely! &eu+openia +an in+rease the risk o in e+tion and thrombo+ytopenia +an in+rease the risk o bleeding! In lammation o mus+les %myositis' +an +ause mus+le pain and #eakness! This +an lead to ele8ations o mus+le enAyme le8els in the blood! In lammation o blood 8essels %8as+ulitis' that supply o>ygen to tissues +an +ause isolated in"ury to a ner8e, the skin, or an internal organ! The blood 8essels are +omposed o arteries that pass o>ygen*ri+h blood to the tissues o the body and 8eins that return o>ygen*depleted blood rom the tissues to the lungs! 5as+ulitis is +hara+teriAed by in lammation #ith damage to the #alls o 8arious blood 8essels! The damage blo+ks the +ir+ulation o blood through the 8essels and +an +ause in"ury to the tissues that are supplied #ith o>ygen by these 8essels! In lammation o the lining o the lungs %pleuritis' and o the heart %peri+arditis' +an +ause sharp +hest pain! The +hest pain is aggra8ated by +oughing, deep breathing, and +ertain +hanges in body position! The heart mus+le itsel rarely +an be+ome in lamed %+arditis'! It has also been sho#n that young #omen #ith S&E ha8e a signi i+antly in+reased risk o heart atta+ks rom +oronary artery disease! )idney in lammation in S&E +an +ause leakage o protein into the urine, luid retention, high blood pressure, and e8en kidney ailure! This +an lead to urther atigue and s#elling o the legs and eet! Cith kidney ailure, ma+hines are needed to +leanse the blood o a++umulated poisons in a pro+ess +alled dialysis! In8ol8ement o the brain +an +ause personality +hanges, thought disorders %psy+hosis', seiAures, and e8en +oma! Damage to ner8es +an +ause numbness, tingling, and #eakness o the in8ol8ed body parts or e>tremities! $rain in8ol8ement is re erred to as lupus +erebritis! Many patients #ith S&E e>perien+e hair loss %alope+ia'! O ten, this o++urs simultaneously #ith an in+rease in the a+ti8ity o their disease! The hair loss +an be pat+hy or di use and appear to be more like hair thinning! Some patients #ith S&E ha8e Raynaud;s phenomenon! In these patients, the blood supply to the ingers andEor toes be+omes +ompromised upon e>posure to +old, +ausing blan+hing, #hitish andEor bluish dis+oloration, and pain and numbness in the e>posed ingers and toes!

How is lupus diagnosed?


Sin+e patients #ith S&E +an ha8e a #ide 8ariety o symptoms and di erent +ombinations o organ in8ol8ement, no single test establishes the diagnosis o systemi+ lupus! To help do+tors impro8e the a++ura+y o the diagnosis o S&E, ,, +riteria #ere established by the Ameri+an Rheumatism Asso+iation! These ,, +riteria are +losely related to the symptoms dis+ussed abo8e! Some patients suspe+ted o ha8ing S&E may ne8er de8elop enough +riteria or a de inite diagnosis! Other patients a++umulate enough +riteria only a ter months or years o obser8ation! Chen a person has our or more o these +riteria, the diagnosis o S&E is strongly suggested! Ne8ertheless, the diagnosis o S&E may be made in some settings in patients #ith only a e# o these +lassi+al +riteria, and treatment may sometimes be instituted at this stage! O these patients #ith minimal +riteria, some may later de8elop other +riteria, but many ne8er do! The ,, +riteria used or diagnosing systemi+ lupus erythematosus are

malar %o8er the +heeks o the a+e' <butter ly< rash,

dis+oid skin rash %pat+hy redness #ith hyperpigmentation and hypopigmentation that +an +ause s+arring

photosensiti8ity %skin rash in rea+tion to sunlight Iultra8iolet lightJ e>posure

mu+ous membrane ul+ers %spontaneous ul+ers o the lining o the mouth, nose, or throat

arthritis %t#o or more s#ollen, tender "oints o the e>tremities

pleuritis or peri+arditis %in lammation o the lining tissue around the heart or lungs, usually asso+iated #ith +hest pain upon breathing or +hanges o body position

kidney abnormalities %abnormal amounts o urine protein or +lumps o +ellular elements +alled +asts dete+table #ith a urinalysis

brain irritation %mani ested by seiAures I+on8ulsionsJ andEor psy+hosis',

blood*+ount abnormalities %lo# +ounts o #hite or red blood +ells, or platelets, on routine blood testing

immunologi+ disorder %abnormal immune tests in+lude anti*DNA or anti*Sm ISmithJ antibodies, alsely positi8e blood test or syphilis, anti+ardiolipin antibodies, lupus anti+oagulant, or positi8e &E prep test', and

antinu+lear antibody %positi8e ANA antibody testing Iantinu+lear antibodies in the bloodJ'!

In addition to the ,, +riteria, other tests +an be help ul in e8aluating patients #ith S&E to determine the se8erity o organ in8ol8ement! These in+lude routine testing o the blood to dete+t in lammation % or e>ample, tests +alled the sedimentation rate and 1*rea+ti8e protein', blood*+hemistry testing, dire+t analysis o internal body luids, and tissue biopsies! Abnormalities in body luids and tissue samples %kidney, skin, and ner8e biopsies' +an urther support the diagnosis o S&E! The appropriate testing pro+edures are sele+ted or the patient indi8idually by the do+tor!

What is the treatment for systemic lupus?


There is no permanent +ure or S&E! The goal o treatment is to relie8e symptoms and prote+t organs by de+reasing in lammation andEor the le8el o autoimmune a+ti8ity in the body! Many patients #ith mild symptoms may need no treatment or only intermittent +ourses o antiin lammatory medi+ations! Those #ith more serious illness in8ol8ing damage to internal organ%s' may re-uire high doses o +orti+osteroids in +ombination #ith other medi+ations that suppress the body;s immune system! (atients #ith S&E need more rest during periods o a+ti8e disease! Resear+hers ha8e reported that poor sleep -uality #as a signi i+ant a+tor in de8eloping atigue in patients #ith S&E! These reports emphasiAe the importan+e or patients and physi+ians to address sleep -uality and the e e+t o underlying depression, la+k o e>er+ise, and sel *+are +oping strategies on o8erall health! During these periods, +are ully pres+ribed e>er+ise is still important to maintain mus+le tone and range o motion in the "oints! Nonsteroidal antiin lammatory drugs %NSAIDs' are help ul in redu+ing in lammation and pain in mus+les, "oints, and other tissues! E>amples o NSAIDs in+lude aspirin, ibupro en %Motrin', napro>en %Naprosyn', and sulinda+ %1linoril'! Sin+e the indi8idual response to

NSAIDs 8aries among patients, it is +ommon or a do+tor to try di erent NSAIDs to ind the most e e+ti8e one #ith the e#est side e e+ts! The most +ommon side e e+ts are stoma+h upset, abdominal pain, ul+ers, and e8en ul+er bleeding! NSAIDs are usually taken #ith ood to redu+e side e e+ts! Sometimes, medi+ations that pre8ent ul+ers #hile taking NSAIDs, su+h as misoprostol %1ytote+', are gi8en simultaneously! 1orti+osteroids are more potent than NSAIDs in redu+ing in lammation and restoring un+tion #hen the disease is a+ti8e! 1orti+osteroids are parti+ularly help ul #hen internal organs are a e+ted! 1orti+osteroids +an be gi8en by mouth, in"e+ted dire+tly into the "oints and other tissues, or administered intra8enously! Un ortunately, +orti+osteroids ha8e serious side e e+ts #hen gi8en in high doses o8er prolonged periods, and the do+tor #ill try to monitor the a+ti8ity o the disease in order to use the lo#est doses that are sa e! Side e e+ts o +orti+osteroids in+lude #eight gain, thinning o the bones and skin, in e+tion, diabetes, a+ial pu iness, +atara+ts, and death %ne+rosis' o the tissues in large "oints! /ydro>y+hloro-uine %(la-uenil' is an antimalarial medi+ation ound to be parti+ularly e e+ti8e or S&E patients #ith atigue, skin in8ol8ement, and "oint disease! 1onsistently taking (la-uenil +an pre8ent lare*ups o lupus! Side e e+ts are un+ommon but in+lude diarrhea, upset stoma+h, and eye*pigment +hanges! Eye*pigment +hanges are rare but re-uire monitoring by an ophthalmologist %eye spe+ialist' during treatment #ith (la-uenil! Resear+hers ha8e ound that (la-uenil signi i+antly de+reased the re-uen+y o abnormal blood +lots in patients #ith systemi+ lupus! Moreo8er, the e e+t seemed independent o immune suppression, implying that (la-uenil +an dire+tly a+t to pre8ent the blood +lots! This as+inating study highlights an important reason or patients and do+tors to +onsider (la-uenil or long*term use, espe+ially or those S&E patients #ho are at some risk or blood +lots in 8eins and arteries, su+h as those #ith phospholipid antibodies %+ardiolipin antibodies, lupus anti+oagulant, and alse*positi8e 8enereal disease resear+h laboratory test'! This means not only that (la-uenil redu+es the +han+e or re* lares o S&E, but it +an also be bene i+ial in thinning the blood to pre8ent abnormal e>+essi8e blood +lotting! (la-uenil is +ommonly used in +ombination #ith other treatments or lupus! 2or resistant skin disease, other antimalarial drugs, su+h as +hloro-uine %Aralen' or -uina+rine, are +onsidered and +an be used in +ombination #ith hydro>y+hloro-uine! Alternati8e medi+ations or skin disease in+lude dapsone and retinoi+ a+id %Retin*A'! Retin*A is o ten e e+ti8e or an un+ommon #art*like orm o lupus skin disease! 2or more se8ere skin disease, immunosuppressi8e medi+ations are +onsidered as des+ribed belo#! Medi+ations that suppress immunity %immunosuppressi8e medi+ations' are also +alled +ytoto>i+ drugs! Immunosuppressi8e medi+ations are used or treating patients #ith more se8ere mani estations o S&E, su+h as damage to internal organ%s'! E>amples o immunosuppressi8e medi+ations in+lude methotre>ate %Rheumatre>, Tre>all', aAathioprine %Imuran', +y+lophosphamide %1yto>an', +hlorambu+il %&eukeran', and +y+losporine %Sandimmune'! All immunosuppressi8e medi+ations +an seriously depress

blood*+ell +ounts and in+rease risks o in e+tion and bleeding! Other side e e+ts are spe+i i+ or ea+h drug! 2or e>amples, Rheumatre> +an +ause li8er to>i+ity, #hile Sandimmune +an impair kidney un+tion! In re+ent years, my+ophenolate mo etil %1ell+ept' has been used as an e e+ti8e medi+ation or lupus, parti+ularly #hen it is asso+iated #ith kidney disease! 1ell+ept has been help ul in re8ersing a+ti8e lupus kidney disease %lupus renal disease' and in maintaining remission a ter it is established! Its lo#er side*e e+t pro ile has ad8antage o8er traditional immune*suppression medi+ations! In S&E patients #ith serious brain or kidney disease, plasmapheresis is sometimes used to remo8e antibodies and other immune substan+es rom the blood to suppress immunity! Rarely, S&E patients +an de8elop seriously lo# platelet le8els, thereby in+reasing the risk o e>+essi8e and spontaneous bleeding! Sin+e the spleen is belie8ed to be the ma"or site o platelet destru+tion, surgi+al remo8al o the spleen is sometimes per ormed to impro8e platelet le8els! Other treatments ha8e in+luded plasmapheresis and the use o male hormones! (lasmapheresis has also been used to remo8e proteins %+ryoglobulins' that +an lead to 8as+ulitis! End*stage kidney damage rom S&E re-uires dialysis andEor a kidney transplant! Most re+ent resear+h is indi+ating bene its o ritu>imab %Ritu>an' in treating lupus! Ritu>imab is an intra8enously in used antibody that suppresses a parti+ular #hite blood +ell, the $ +ell, by de+reasing their number in the +ir+ulation! $ +ells ha8e been ound to play a +entral role in lupus a+ti8ity, and #hen they are suppressed, the disease tends to#ard remission! This may parti+ularly help ul or patients #ith kidney disease! At the .GGM national Rheumatology meeting, there #as a paper presented suggesting that lo#*dose dietary supplementation #ith omega*0 ish oils +ould help patients #ith lupus by de+reasing disease a+ti8ity and possibly de+reasing heart*disease risk!

Systemic Lupus (cont.) .n this Article


Chat is lupusN Chat are the types o lupusN Chat +auses lupusN Is it hereditaryN Chat is drug*indu+ed lupusN Chat are the symptoms and signs o lupusN /o# is lupus diagnosedN Chat is the treatment or systemi+ lupusN /o# +an a lupus patient help pre8ent disease a+ti8ity % lares'N /o# +an lupus a e+t pregnan+y or the ne#bornN Chat does the uture hold or patients #ith lupusN Chere +an one get more in ormation about lupusN Systemi+ &upus At A 7lan+e Patient 'iscussions/ &upus * Symptoms at Onset o Disease Systemi+ &upus 7lossary

Systemi+ &upus Inde>

What does the future hold for patients with lupus?


O8erall, the outlook or patients #ith systemi+ lupus is impro8ing ea+h de+ade #ith the de8elopment o more a++urate monitoring tests and treatments! The role o the immune system in +ausing diseases is be+oming better understood through resear+h! This kno#ledge #ill be applied to design sa er and more e e+ti8e treatment methods! 2or e>ample, +ompletely re8ising the immune system o patients #ith e>tremely aggressi8e treatments that 8irtually temporarily #ipe out the immune system is being e8aluated! 1urrent studies in8ol8e immune eradi+ation #ith or #ithout repla+ement o +ells that +an re*establish the immune system %stem +ell transplantation'! It should be noted that patients #ith S&E are at a some#hat in+reased risk or de8eloping +an+er! The +an+er risk is most dramati+ or blood +an+ers, su+h as leukemia and lymphoma, but is also in+reased or breast +an+er! This risk probably relates, in part, to the altered immune system that is +hara+teristi+ o S&E! Comen #ith S&E appear to be at in+reased risk or heart disease %+oronary artery disease' a++ording to re+ent reports! Comen #ith S&E should be e8aluated and +ounseled to minimiAe risk a+tors or heart disease, su+h as ele8ated blood +holesterol, -uitting smoking, high blood pressure, and obesity! D/EA %dehydroepiandrosterone' has been help ul in redu+ing atigue, impro8ing thinking di i+ulties, and impro8ing -uality o li e in patients #ith S&E! Re+ent resear+h indi+ates that D/EA has been sho#n to impro8e or stabiliAe signs and symptoms o S&E! D/EA is +ommonly a8ailable in health* ood stores, pharma+ies, and many gro+eries! &andmark resear+h has sho#n +learly that oral +ontra+epti8es do not in+rease the rate o lares o systemi+ lupus erythematosus! This important inding is opposite to #hat has been thought or years! No# #e +an reassure #omen #ith lupus that i they take birth* +ontrol pills, they are not in+reasing their risk or lupus lares! NOTE: $irth*+ontrol pills or any estrogen medi+ations should still be a8oided by #omen #ho are at in+reased risk o blood +lotting, su+h as #omen #ith lupus #ho ha8e phospholipid antibodies %in+luding +ardiolipin antibody and lupus anti+oagulant'!

Individuals with SLE can improve their prognosis by learning about the many aspects of the illness as wWhat are antinuclear antibodies?
Ce normally ha8e antibodies in our blood that repel in8aders into our body, su+h as 8irus and ba+teria mi+robes! Antinu+lear antibodies %ANAs' are unusual antibodies, dete+table in the blood, that ha8e the +apability o binding to +ertain stru+tures #ithin the nu+leus o the +ells! The nu+leus is the innermost +ore #ithin the body;s +ells and +ontains the DNA,

the primary geneti+ material! ANAs are ound in patients #hose immune system may be predisposed to +ause in lammation against their o#n body tissues! Antibodies that are dire+ted against one;s o#n tissues are re erred to as auto*antibodies! The propensity or the immune system to #ork against its o#n body is re erred to as autoimmunity! ANAs indi+ate the possible presen+e o autoimmunity and pro8ide, there ore, an indi+ation or do+tors to +onsider the possibility o autoimmune illness!

How is the A A test designed? What is it for?


The ANA test #as designed by Dr! 7eorge 2riou in ,K4M! The ANA test is per ormed using a blood sample! The antibodies in the serum of the blood are e,posed in the laboratory to cells. .t is then determined whether or not antibodies are present that react to various parts of the nucleus of cells. Thus, the term anti-*nuclear* antibody. 0luorescence techni1ues are fre1uently used to actually detect the antibodies in the cells, thus A(A testing is sometimes referred to as fluorescent antinuclear antibody test 20A(A3. The A(A test is a sensitive screening test used to detect autoimmune diseases

ell as +losely monitoring their o#n health #ith their do+tors!

What are autoimmune diseases?


Autoimmune diseases are +onditions in #hi+h there is a disorder o the immune system +hara+teriAed by the abnormal produ+tion o antibodies %auto*antibodies' dire+ted against the tissues o the body! Autoimmune diseases typi+ally eature in lammation o 8arious tissues o the body! ANAs are ound in patients #ith a number o di erent autoimmune diseases, su+h as systemi+ lupus erythematosus, S"ogren;s syndrome, rheumatoid arthritis, polymyositis, s+leroderma, /ashimoto;s thyroiditis, "u8enile diabetes mellitus, Addison disease, 8itiligo, perni+ious anemia, glomerulonephritis, and pulmonary ibrosis! ANAs +an also be ound in patients #ith +onditions that are not +onsidered +lassi+ autoimmune diseases, su+h as +hroni+ in e+tions and +an+er!

What other conditions cause A As to be produced?


ANAs +an be produ+ed in patients #ith in e+tions %8irus or ba+teria', lung diseases %primary pulmonary ibrosis, pulmonary hypertension', gastrointestinal diseases %ul+erati8e +olitis, 1rohn;s disease, primary biliary +irrhosis, al+oholi+ li8er disease', hormonal diseases %/ashimoto;s autoimmune thyroiditis, 7ra8e;s disease', blood diseases %idiopathi+ thrombo+ytopeni+ purpura, hemolyti+ anemia', +an+ers %melanoma, breast, lung, kidney, o8arian and others', skin diseases %psoriasis, pemphigus', as #ell as in the elderly and those people #ith a amily history o rheumati+ diseases!

!an medications cause A As to be produced?


Many medi+ations +an sometimes stimulate the produ+tion o ANAs, in+luding pro+ainamide %(ro+an SR', hydralaAine, and dilantin! ANAs that are stimulated by medi+ation are re erred to as drug*indu+ed ANAs! This does not ne+essary mean that any disease is present #hen these ANAs are <indu+ed!< Sometimes diseases are asso+iated #ith these ANAs, and they are re erred to as drug*indu+ed diseases!

A As are defined in certain patterns" What does this mean?


ANAs present di erent <patterns< depending on the staining o the +ell nu+leus in the laboratory: homogeneous or di use= spe+kled= nu+leolar= and peripheral or rim! Chile these patterns are not spe+i i+ or any one illness, +ertain illnesses +an more re-uently be asso+iated #ith one pattern or another! The patterns then +an sometimes gi8e the do+tor urther +lues as to types o illnesses to look or in e8aluating a patient! 2or e>ample, the nu+leolar pattern is more +ommonly seen in the disease s+leroderma! The spe+kled pattern is seen in many +onditions and in people #ho do not ha8e any autoimmune disease!

Are A As always associated with illness?


No! ANAs +an be ound in appro>imately 4L o the normal population, usually in lo# titers %lo# le8els'! These people usually ha8e no disease! Titers o lo#er than ,:OG are less likely to be signi i+ant! %ANA titers o less than or e-ual to ,:3G are +onsidered negati8e!' E8en higher titers are o ten insigni i+ant in patients o8er 6G years o age! Ultimately, the ANA result must be interpreted in the spe+i i+ +onte>t o an indi8idual patient;s symptoms and other test results! It may or may not be signi i+ant in a gi8en indi8idual!