8heumaLology

8A Chronlc/sysLemlc lnflammaLory arLhrlLls wlLh lnfllLraLlon of synovlal [olnLs by lnflammaLory cells and
progresslve eroslon of arLlcular carLllage and bone
O ynovlal PyperLrophy granulaLlon Llssue on arLlcular carLllage (annus Iormat|on) caused
by [olnL lnflammaLlon

Lpldemlology mlddle age women nLA Dk4
Cllnlcal #s ** symmettlc polyottbtltls see Motoloq 5tlffoess w/ loslJloos ooset tbot lmptoves os tbe
Joy ptoqtesses (-/) low qtoJe fevet fotlqoe ooJ mololse
O Dsoolly beqlooloq wltb tbe smoll jolots (le lll Mcl wtlst) osoolly wlll locloJe koee ookles
elbows sboolJets blps
O Never ulP join invo/vement
O Dlnar uevlaLlon of flngers
O ouLanleres ueformlLy 99 flelon w/ u9 hypereLenslon
O wan neck ueformlLy lleed MC9 w/ 99 hypereLenslon and u9 flelon
O Cervlcal plne ubluaLlon (C1 and C2) 8A pLs musL have cervlcal splne lmaglng prlor Lo
surgery as rlsk of subluaLlon durlng lnLubaLlon may be faLal

troorticu/or
coostltotloool Mololse fevet oootexlo fotlpoe
Cutaneous O kln becomes Lhln w/ easy brulslng
O mall vessel vascullLlc changes/ulceraLlons lnvolvlng palms flngerLlps nall folds
O kheumato|d Nodu|es (pathagnomon|c for kA) subcuLaneous palnless nodules
over eLensor surfaces (elbows occlpuL sacrum) and someLlmes vlsceral organs
u|monary O 9leural Lffuslons vety low ln vety low qlocose low complemeot level ofteo
coofoseJ wltb empyemo
O 9ulmonary llbrosls see honeycomblng on Cx8 and resLrlcLlve paLLern on 9l1s
O 9ulmonary nfllLraLes
O 8heumaLlc nodules may cavlLaLe Lhe lungs and creaLe a nldus for lnfecLlon
O Cap|ans syndrome ls a rare subseL of pulmonary nodulosls characLerlzed by Lhe
developmenL of nodules and pneumoconlosls followlng slllca eposure
Card|ac O 8heumaLlc nodules ln Lhe PearL can cause conducLlon problems (hearL block
and bundle branch blocks)
O 9erlcardlLls see ln 40 of pLs
O 9erlcardlal Lffuslons
O 9erlcardlLls (consLrlcLlve) very rare
Cccuar O lecrlLls/eplsclerlLls
O cleromalacla sofLenlng of Lhe sclera LhaL may acLually perforaLe and cause
bllndness
O eraLocon[uncLlvlLls slcca (s[ogren's syndrome) dry eyes and no Lears and dry
mucous membranes ln general
Nervous
System
O Mononeur|t|s Mu|t|p|ex presence of sysLemlc vascullLls of smallmedlum slzed
vessels causes lnfarcL (soJJeo ooset) Lo nerve pleus
O neuropaLhy ls Lyplcally asynchronous and assymeLrlc"

e/tys
5yndrome
O 1rlad of 8A neuLropenla plenomegaly
O Also see anemla of chronlc dlsease LhrombocyLopenla and lymphadenopaLhy
O ssocloteJ w/ blqb tltets of kl ooJ exttoottlcolot Jlseose
O ncreased suscepLlblllLy Lo lnfen

8|ood O Anemla of Chronlc ulasease mlld normocyLlc normochromlc anemla
O 1hrombocyLosls

Vascu||t|s M|crovascu|ar Vascu||t|s may progress Lo MesenLerlc vascullLls 9An or oLher vascular
syndromes



L5
O ncreased 8l LlLers (gM anLl lc gC) 73 of pLs (noLspeclflc!)
O (+) AnA 40
O (+)Ant|CC Ab 9S
O nc L8/C89 (nonspelflc)
O ynovlal [olnL asplraLe Lurbld fluld decreased vlscoslLy wlLh lncrease WC counL (30003000)

kkay Larly 5oft tlssoe swellloq ooJ joxtoottlcolot Jemloetlllzoloo

Lote loint spoce norrowinq ond erosions {physico/
deformity of joint)

1x
egln wlLh hlgh dose nAus (+/ 1ylenol and corLlcosLerolds) Lhen Laper Lo lowesL dose posslble for
sympLom rellef and Lherapy lnlLlaLlon (uMA8us Lake aL leasL 6wks Lo sLarL worklng)

uMA8us Lo slow Lhe progresslon of dlsease

I|rst L|ne
Metbottexote O Lw5 6lv wl1n L1 5uPPL,N11lN!
O lvet 1oxlclty
O ooe mottow 5opptessloo
O lotetstltlol poeomooltls polmoooty flbtosls
O ral Dlcers (sLomaLlLls) mlld alopecla
O C upseL
nyJtoxycbolotpoloe
(ose lo mllJ coses ot lo
comblootloo)
O etino 1oicity /oss of vision
8equlres eye eam q6mo
5olfosolozloe O ooe mottow toxlclty
O neolysls wltb C6lu Jeflcleocy
O kosb



5econd Line 1heropy {or in combinotion with ,ethotreote)

1Nl loblbltots
(lofllxlmob oJolloomob
eotetocept)
eoctivotion of /otent 1 Lw5 u PPu l51!
ncreased succespLlblllLy Lo lnfecLlon
otl cu20
(ketlxlmob)
locteoseJ soccesptlblllty to lofectloo
eflooomlJe nepotlc 1oxlclty myelosopptessloo
cyclospotloe



DISLASL SLkUM MAkkLk
L O AnA (screenlng
purposes)
O Ant|dsDNA
O Ant|SmAb (mosL
speclflc for L)
O f you don#L have a poslLlve AnA you
probably don#L have upus buL Lhe
opposlLe ls noL Lrue!
O AnLldsunA speclflc for renal
lnvolvemenL ln L
O May cause false poslLlve 898
(syphalus)
urug nduced upus O Ant|n|stoneAb 1 Pydralazlne
2 9rocalnamlde
3 nP
4 MeLhyldopa
3 Culnedlne
6 Chlorpromazlne
**u/C drugsd/c lupus
8heumaLold ArLhrlLls O (+) 8l (73)
O AnLlCC9 Ab
O PA u84
O (AnA ln 40)
AnLlCC9 Ab ls Lhe besL marker seen early and
lL ls speclflc
9olymyoslLls/
uermaLomyoslLls
O AnA
O Ant|Io1 Ab
O C9/Aldsolase
AnLl!o Ab ls assoclaLed wlLh lung flbrosls
esL lnlLlal LesL ls C9 and a|do|ase
Ankyloslng pondyllLls O Seronegat|ve
arthropathy
(negat|ve kI)
O nLA827
9A8 ls Lhe mneumonlc
1 9sorlaLlc ArLhrlLls
2 Ankylosklng pondylyLls
3 nflammaLory owel ulsease
4 8leLer arLhrlLls
cleroderma
O Ant|sc|70 ANA (aka
Ant|1opo|somerase
Ab)

C8L1 syndrome O Ant|Centromere Ab
Mled ConnecLlve
1lssue
MC1u
O Ant| kN ANA
[ogren#s O Ant|ko (Ant|SSA)
ANA
O Ant|La (Ant|SS8)
ANA




L
AuLoanLlbody producLlon lmmune comple deposlLlon complemenL acLlvaLlon vascullLlc/Llssue
descLrucLlon
1ypes of Lupus
1) SLL
2) D|sco|d Lupus (sk|n |es|ons w]o system|c d|sease)
3) Drug |nduced Lupus
4) ANAnegat|ve Lupus assoc|ated f|nd|ngs
a Arthr|t|s keynaud's phemenon subacute cutaneous |upus
b Sero|ogy Ant|ko (SSA) Ab pos|t|ve ANA neg
c k|sk of neonata| |upus |n |nfants of affected women

Const|tut|ona| Iat|gue (common) ma|a|se fever anorex|a
Cutaneous O Ma|ar kash
O D|sco|d kash (Scar|ng erythematous ra|sed patches w|th kerotot|c sca||ng)
O hotosens|t|v|ty face and hands
O Cra| and nasopharyngea| u|cers
Muscku|oske|eta| O Io|nt pa|n (90 of pp|e often f|rst symptom and assoc w] f|are)
O ArLhralgla myalgla and frank arLhrlLls may lnvolve Lhe
small [olnLs of Lhe hands wrlsLs and knees
O ymmeLrlc noneroslve wlLh paln LhaL ls dlsproporLlonaLe
Lo swelllng
O x 8ay flndlngs are normal and lnvolve 99 MC9 WrlsLs
nees leeL

Seoros|t|s nflammaLlon of pleura and perlcardlum ChesL 9aln w/ effuslons
kena| perslsLenL proLelnurla (300mg/day) or cellular casLs on urlnalysls
4 Mesanglal dlsease (mlnlmal lnvolvmenL llmlLed Lo mesanglum)
4 local (mlld lnvolvemenL of kldney parenchyma)
4 ulffuse (MembranoprollferaLlve wldespread lnvolvemenL of glomerull)
4 Membraneous (see Lhlckenlng of glomerular basemenL membrane)
May see ked ce|| casts or hematur|a
(Most common organ |nvo|ved)
Card|ac O 9erlcardlLls
O LndocardlLls (elbmansacks endocardlLls ls a serlous compllcaLlon)
O myocardlLls

u|monary O |eur|t|s
O 9leural effuslons
O 9neumonlLls pulmonary flbrosls
O Alveolar Pemmorage and 9nA posslble
nemato|og|c O PemolyLlc Anemla
O Anemla or reLlculocyLosls of chronlc dlsease
O ymphopenla eukopenla 1hrombocyLopenla
O 1hrombus formaLlon
O 8aynouds phenomenon
Immuno|og|c ANA +
O Ck any of the fo||ow|ng
1) AnLldsunA (30)
2) AnLlm Ab (40)
3) lalse poslLlve LesL for syphalus
4) 9oslLlve L preparaLlon AnAs blnd Lo nuclel of damaged cells
produclng oJles
A|so
ComplemenL ususally decreased
AnLlcardlollpan A and upus AnLlcoagulanL presenL ln
hypercoagulable sLaLes
ko (SSA) and La (SS8) are found ln 1330 and assoc w/
1) [ogren#s
2) ubacuLe cuLaneous
3) neonaLal upus (congenlLal hearL block)
4) ComplemenL ueff (C2 and C4)
S) AnAneg lupus

GI n/v dyspepsla dysphagla 9Du
CNS elzures psychosls depresslon headaches 1A CvA
Cther 9hoLophobla 8eLlnal dlsease (coLLon wool spoLs) llndness con[uncLlvlLls
abdomlnal paln

1x
1) NSAIDs
2) Avo|d Sun Lxposure
3) Cort|costero|ds
4) nydroxych|oroqu|ne for sk|n art|cu|ar rena| sx's
S) Cytotox|c Agents (cyc|ophospham|de) for act|ve g|omeru|onephr|t|s
6) Mon|ter and treat kena| Inx and n1N
7) Ant|coagu|at|on |f needed for hypercoagu|ab|e states


o|ymyos|t|s]Dermatomyos|t|s progresslve dlsease wlLh skeleLal mm lnflammaLlon
O neLhlrd of pple wlLh polymyoslLls also have uermaLomyoslLls
**nC8LALu 8 l8 MACnAnC?
xs
O 9rogresslve and ?MML18C 98xMA mm weakness LhaL beglns ln Lhe legs develops over
weeks Lo monLhs!
O Myalglas and muscle Lenderness
O uysphaglas lf esophageal lnvolvemenL
O Interst|t|a| Lung D|seasedyspnea and poor oxygenat|on saturat|on
O Sc|erodacty|y| 1hlckenlng of Lhe skln on Lhe flngers and hands
O aLer on muscle aLrophy

Cutaneous Man|festat|ons of Dermatomyos|t|s
O 8ed PelloLroplc 8ash symmeLrlc confluenL purplered macular erupLlon of Lhe eyellds and
perlorblLal Llssue
O LryLhroderma vloleL dlscoloraLlon of perlorblLal reglon
O hawl lgn" 8ash lnvolvlng shoulders upper chesL back made worse wlLh Dv llghL
O CoLLron#s 9apules 9apular rash wlLh scales on Lhe dorsum of Lhe hand and aL Lhe bony
promlnence (ofLen mlsLaken for psorlasls)
O Mechanlcs Pands" 8ough eryLhemaLous cracked skln on flnger Llps and laLeral aspecLs of
flngers (w/ LelangloacLlslas)

Labs
1) ncreased Ck and a|do|ase
a 1he creaLlne klnase level ls Lhe mosL senslLlve and speclflc lL usually ls 330 Llmes above
Lhe reference level
2) ncreased uP A1 A1 creaLnlne
3) AnA freq poslLlve
4) Ant|Io1 Ant|bod|es 9oslLlve ln paLlenLs wlLh lnLersLlLlal lung dlsease
S) Musc|e 8|opsy nflammaLory cells and muscle degeneraLlon
a o|ymyos|t|s nflammaLory Cells WI1nIN muscle fasclcles (Lndomyseal)
b Dermatomyos|t|s nflammaLory cells SUkkCUNDING muscle fasclcles (9erlvascular and
9erlmyslal)
6) ,6 5pootooeoos llbtlllotloos

1 Plgh dose corLlcosLerolds meLhoLreaLe or azaLhloprlne for 46wks +/ vC

o|ymy|ag|a kheumat|ca ee muscle 9An wlLhouL any muscle weakness
Llderly women
Lrongly AssoclaLed wlLh 1emporal ArLerlLls
#s
1) llaLeral Plp and houlder 9aln
a evere mornlng sLlffness and afLer perlods of lnacLlvlLy
b 9aln on movemenL (unable Lo llfL shoulders)
c ulfflculLy geLLlng ouL of bed
2) ConsLlLuLlonal ympLoms lD malalse depresslon uneplalned wL loss anemla
3) !olnL paln and swelllng posslble synovlLls of knees wrlslLs hands
a 1enosynovlLls and synovlLls around shoulder may lead Lo roLaLor cuff Lenderness or
adheslve capsullLls

abs LSk S0 (often 100) Negat|ve kI
1x low dose corLlcosLerolds lmmedlaLe workup for 1emporal ArLerlLlLls

Seronegat|ve Spondy|oarthropath|es
ll setooeqotlve spooJyloottbtopotbles bove tbe followloq lo commoo
1) Neqotlve kl
2) 5ttooq ssoc w/ n27
J) Ollqoottbtltls
4) otbesltls loflommotloo ot sltes of losettloo of fosclo llqomeot ot teoJoo to booe
5) loflommototy ttbtltls oxlol ooJ soctollloc jolots
6) xttoottlcolot feototes eyes sklo CD ttoct
7) lomlllol pteJlsposltloo look fot lMnx of lu 5 lsotlosls


Ankyloslng pondyllLls Chronlc lnflammaLory dlsease of splne and pelvls (bllaLeral
sacrollllLus)
a ee fuslon of splne ln ascendlng manner
b Malesfemales usually ln laLe Leens early 20s40s

Cllnlcal leaLures
O n|p and Low 8ack a|n that |s worse |n the morn|ng and after |nact|v|ty |mproves over
course of the day
O a|nfu| kyphos|s that |s re||eved by bend|ng forward
O 9osslble llmlLed of splne/hlp/chesL
O 9osslble Anter|or Uve|t|s

CompllcaLlons resLrlcLlve lung dlsease Cauda Lqulna syndrome plnal fracLure wlLh cord ln[ury
sLeoporosls sponylodlsclLls

keact|ve Arthr|t|s
O ssymetric lnflammaLory o/iqoorthritis of Lhe lower eLremeLles LhaL may be mlgraLory
O 14 weeks afLer sysLemlc lnfecLlon (C or CD)
O commonly assoclaLed organlsms 5olmooello 5blqello compyloboctot cblomyJlo etslolo
O eiters 5yndrome ArLhrlLls ureLhrlLls ocular lnflammaLlon (con[uncLlvlLls anLerlor uvelLls)

sor|at|c Arthr|t|s een ln 1020 of people wlLh 9sorlasls
O 1yplcally pL w/ skln manlfesLaLlons for monLhs Lo yrs prlor Lo [olnL lnvolvemenL
O ASSML1kIC and CLAk1ICULAk w] Upper LxtremetyLL Sma|| Io|ntsLarge Io|nts
O DI [o|nts and sp|ne common|y affected worse |n morn|ng
O !olnL llne paln paln wlLh sLress on [olnLs
O oss|b|e Anter|or Uve|t|s
O nall plLLlng
8adlology shows hlghly desLrucLlve leslons of 99 and u9 [olnLs (pencll ln cup")

SCLLkCDLkMA