Chronic systemic inflammatory disease

Symmetrical, deforming peripheral polyarthritis

Presentation
Painful, swollen and stiff small joints of hand /
feet Higher prevalance in females; typically from
30s oes not affect !P joints
"orse in the morning
Pea# onset in $0s
%cute flares superimposed over moderate progression
&arly
'oi nt sw e lling (s ymm e tr ical ) C P, P !P wr ist*, infla mm a tio n, s yno vitis
)ulti System autoimmune disease, with autoanti+odies against
a variety of autoantigens
Signs
,ater 'oint damage, deformity, ulnar deviation of fingers, dorsal wrist su+lu-ation
Swan nec# and .outonniere deformities of fingers, /0shaped deformities of thum+
1odules on el+ows, fi+rosing alveolitis, pleural effusion, 2aynaud3s, carpal
Polycloncal .0cell secretion of pathogenic autoanti+odies
and formation of immune comple-es
4 yp ica lly w o m en of child +ea ring age
Common in %fro Cari++eans and %sians
Pathology
&pidemiology
&-tra articular
5elty3s Syndrome
tunn e l, pe ripe rh al ne uropa thy
Complication of 2%
4riad of seropostive 2%, neutropenia, and splenomegaly
&rythematous raised patches
.utterfly rash
2h 5actor
Positive in 678 cases
%naemia of chronic disease
w ith #e ra to tic s ca les
iscoid rash 2heumatoid %rthritis !nvestigations
.loods
!nfla mm a tion ca uses ra ised p la te le ts, C 2 P a nd & S 2
Photosensitivity
9ral ulcers
1on erosive arthritis
:02ay
So ft tiss ue sw e lling, ju-ta a rtic ular os teope nia, +o ny e rosio ns
2 ap id ly red uce infla mm a tion / co ntro ls s ym p to m s in s ho rt te rm

Steroids
P le uritis or pe rica rd itis
Serositis
iagnosing S,& ($ or more* ;seful for acute flares
Help control symptoms, try different 1S%!s as response varies
Per sis ta nt p ro te inu ria or ce llular cas ts
Se i<ures of ps yc hosis
2enal disorder
C1S isorder
1S%!s
Paracetamol = wea# opiates use is limited
2educe inflammation
C9:0> inhi+itors where indicated
Haematological disorder
!mmunological disorder
&arly use of disease modifying agents improve symptoms / long term
outcomes
"ee#ly dose; avoid in liver disease, pregnancy
9 ver ?78 cases a re % 1 % = ive
Se nsi tive test
%nti0nuclear anti+ody
2elapsing remitting condition
S,&
Connective Tissue
Disease 1
)anagement )ethotre-ate S&@ oral ulcers, nausea, lethargy, myelosuppression, hepatoto-icity
%nti0inflammatory effects are due to effects on 415 pathways
S&@ myelosuppression, nausea, rash, low sperm count
9ther features@ fatigue, pyre-ia during flares, weight loss, myalgia, alopecia,
2aynaud3s
Clinical 5eatures
)%
Sulfasala<ine
2educes synthesis of inflammatory mediators and cyto#ines (no immunosupressant action*
% spec ific test
ou+le stranded
1% ecreased
complement
)onitoring activity Aold
; su ally via !) injec tion, high to-icity
S & @ m ye los upp ressio n, re nal to -ici ty, ras h, p ho tose n si tivity
Can occur secodary to S,& (>78 of
%ntiphospholipid anti+odies are
present
Coagulation defect, ,ivedo reticularis, 9+stetric
(recurrent miscarriage 0 give ,)"H to prevent
m isca rriage *, 4h ro m o+oc ytope nia
C,94
features
%ntiphospholipid syndrome
,imited systemic sclerosis
C2&S4 syndrome
Calcinosis (su+ cut tissues*, 2aynaud3s, o&sophageal
and gut dysmotility, Sclerdactyly and 4elangiectasia (non
+lanching haemorrhages*
S#in involvement is limited to face, hands and feet
!f jo int / s#in s ym p to m s a re un co ntro lled +y
1 S % ! s
S & @ irr e ve rsi+ le re tinopa thy
High factor sun+loc#
Hydro-ychloroBuine
4reatment
Systemic Sclerosis
%ssociated with
anticentromere anti+odies and
pulmonary H4
iffuse s#in involvement, whole +ody when severe
,ow dose m ay +e of va lue in c hro nic d isease
High dose prednisolone with severe flares
Steroids iffuse systemic sclerosis
)ore profound organ fi+rosis@ lung,
cardiac and renal disease
Prognosis is often poor
4herapy
Currently no cure
!mmunosuppressive regimens are used for organ involvement
or progressive s#in disease
2egular %C&0! reduces ris# of renal crisis
C2P typically normal, &S2 raised ,east to-ic, although possi+ly least effective
9ver 70 drugs including isonia<id
Hydro-ychloroBuine
S& @ ra sh, re tinopa thy
isease remits if the drug is stopped
9CP may worsen idiopathic S,&
rug induced lupus
Sc le rode rm a 0 s#in fi+ rosis
cases* or as primary disease
5eatures Cascular disease
Connective 4issue iseaseDmmap 0 0E/0?/>0F0 0