Cas e Report
A Cas e of Pol yart eri t i s Nodos a Di agnos ed at Chol ecys t ect omy
Hi deo K
OMITA,Tomoyos hi O
KAMOTO,and Kat s uhi ko Y
ANAGADivision of Gastr oenter ology and Hepatology, Depar tment of Inter nal Medicine, The Jikei Univer sity School of Medicine
Depar tment of Sur ger y, The Jikei Univer sity School of Medicine
ABSTRACT
A 63‑year ‑ol d woman was admi t t ed wi t h pyr exi a and r i ght upper quadr ant pai n. Al t hough chol ecys t i t i s had pr evi ous l y been di agnos ed,t he caus e was unknown,and s ympt oms per s i s t ed and wer e accompani ed by anemi a and l eukocyt os i s . To r el i eve t he s ympt oms and t o cor r ect t he anemi a and l eukocyt os i s at t r i but ed t o chr oni c chol ecys t i t i s ,l apar os copi c chol ecys t ect omy was per f or med. Hi s t opat hol ogi cal exami nat i on r eveal ed i nfil t r at i on i nt o t he mus cul ar l ayer of t he gal l bl adder by eos i nophi l s ,l ymphocyt es ,and pl as ma cel l s i n as s oci at i on wi t h necr ot i zi ng vas cul i t i s , a f eat ur e char act er i s t i c of pol yar t er i t i s nodos a. Pol yar t er i t i s nodos a was l ocal i zed t o t he gal l bl ad- der;hence,t he chol ecys t i t i s appear ed t o r epr es ent t he pr i mar y pr es ent at i on of pol yar t er i t i s nodos a, r at her t han a compl i cat i on of s ys t emi c vas cul ar di s eas e. Pol yar t er i t i s nodos a was di agnos ed at chol ecys t ect omy at an ear l y s t age,wi t h t he r es ul t i ng ear l y t r eat ment yi el di ng a f avor abl e cl i ni cal out come. ( Ji kei kai Med J 2005;52:87‑91)
Key wor ds:chol ecys t i t i s ,pol yar t er i t i s nodos a,necr ot i zi ng angi t i s
I
NTRODUCTION
Pol yar t er i t i s nodos a i s a s ys t emi c col l agen di s - eas e i nvol vi ng t he gal l bl adder i n 10% of 40% of cas es at aut ops y ,wi t h acal cul ous chol ecys t i t i s obs er ved i n 2% t o 9% of cas es . Al t hough we have f r equent l y di agnos ed pol yar t er i t i s nodos a when eval uat i ng cas es of chol ecys t i t i s of unknown or i gi n,onl y a s i ngl e cas e i n whi ch s ympt oms of mononeur i t i s mul t i pl ex appear - ed af t er s ur ger y has been r epor t ed. Under t he cl as s i ficat i on s ys t em of Chen,gal l bl adder vas cul i t i s mos t f r equent l y pr es ent s as a mani f es t at i on of s ys - t emi c pol yar t er i t i s nodos a and pr es ent l es s f r equent l y i n s uch di s eas es as s ys t emi c l upus er yt hemat os us , Wegner ʼ s gr anul oma,and ot her col l agen‑as s oci at ed di s eas es . I n addi t i on,i s ol at ed gal l bl adder vas cul i t i s can occur wi t h l ocal i zed pol yar t er i t i s nodos a. I n t hi s
l at t er cas e,al t hough t he caus e of t he pyr exi a and i nflammat i on i n l ocal i zed chol ecys t i t i s i s unknown,
s ur gi cal r es ect i on can be per f or med f or bot h t r eat - ment and di agnos i s .
C
ASER
EPORT
A 63‑year ‑ol d woman wi t h a hi s t or y of r epeat ed epi s odes of abdomi nal pai n and pyr exi a dur i ng t he pr evi ous s ever al mont hs was admi t t ed t o our hos pi t al ʼ s depar t ment of s ur ger y f or a det ai l ed eval ua- t i on. Phys i cal exami nat i on r eveal ed a hei ght of 155 cm,a body wei ght of 43 kg,a bl ood pr es s ur e of 122/60 mm Hg,l ow‑gr ade pyr exi a(37. 5° C),and pal e pal pe-
br al conj unct i vae. Phys i cal exami nat i on of t he ches t , abdomen,ner vous s ys t em,and s ki n yi el ded no abnor - mal findi ngs . Res ul t s of l abor at or y s t udi es wer e
Recei ved f or publ i cat i on,Januar y 28,2005 込田 英夫，岡本 友好，矢永 勝彦
Mai l i ng addr es s:Hi deo K
OMITA,Di vi s i on of Gas t r oent er ol ogy and Hepat ol ogy,Depar t ment of I nt er nal Medi ci ne,The Ji kei Uni ver s i t y School of Medi ci ne,3‑25‑8 Ni s hi ‑Shi mbas hi ,Mi nat o‑ku,Tokyo 105‑8461,Japan.
E‑mai l:komi t a＠j i kei . ac. j p
87
nor mal except f or a hi gh t i t er of C‑r eact i ve pr ot ei n (10. 4 mg/dL),hypoal bumi nemi a(2. 0 g/dL),and l eu- kocyt os i s (10, 700/mm ) wi t h eos i nophi l i a (6. 8% ; Tabl e 1). Ul t r as onogr aphy and abdomi nal comput ed t omogr aphy r eveal ed a di s t ended gal l bl adder(Fi g.1).
Endos copi c r et r ogr ade chol angi opancr eat ogr aphy
r eveal ed i s ol at ed di s t ens i on of t he gal l bl adder(Fi g.2) wi t h no evi dence of common bi l e duct di l at at i on.
Becaus e of t he pos s i bi l i t y of an as s oci at ed l eukemi a or ot her mal i gnancy,gal l i um s ci nt i gr aphy and bone mar r ow as pi r at i on bi ops y wer e per f or med. Gal l i um s ci nt i gr aphy r eveal ed di ffus e accumul at i on t hr ough-
out t he s kel et al s ys t em,but t he r es ul t s of bone mar - r ow as pi r at i on bi ops y wer e i nconcl us i ve. Al t hough t he caus es of t he pyr exi a and upper quadr ant pai n wer e uncl ear ,we s us pect ed t hes e s ympt oms mi ght be due t o chr oni c i nflammat or y chol ecys t i t i s and,t her e-
f or e,per f or med l apar os copi c chol ecys t ect omy. Hi s - t opat hol ogi cal exami nat i on of t he r es ect ed s peci men r eveal ed t he pr es ence of vas cul ar angi t i s(Fi g.3).
Af t er s ur gi cal r es ect i on,t he abdomi nal pai n r es ol ved, but t he pyr exi a and as s oci at ed el evat ed l evel s of i nflammat or y pr ot ei ns i n t he s er um r emai ned.
Ther ef or e,t o es t abl i s h a defini t i ve di agnos i s ,t he pat i ent was t r ans f er r ed t o t he depar t ment of r heumat ol ogy f or a mor e compr ehens i ve eval uat i on.
Ser ol ogi cal anal ys es yi el ded t he f ol l owi ng val ues:
per i nucl ear ant i neut r ophi l i c cyt opl as mi c ant i body(P‑
H.K
OMITA
,et al . Vol .52,No.3 88
Tabl e 1. Labor at or y Dat a on admi s s i on
Hemat ol ogy Coagul at i on Bl ood Chemi s t r y Ur i nal ys i s
WBC 10, 700/mm PT 80% TP 6. 3 g/dl pH 7. 0 RBC 300×10/mm APTT 36. 5 s ec Al b 2. 0 g/dl SG 1. 046 Hb 8. 2 g/dl TT 54% T‑Bi l 0. 3 mg/dl Pr ot ei n (−)
Ht 24. 8% HPT 75% D‑Bi l 0. 0 mg/dl Sugar (−) Pl t 46. 8×10/mm AST 12 I U/1 Ur obi l i nogen (−)
Gr an 78. 2% ALT 6 I U/1 Acet on (−)
Lymph 9. 6% ALP 324 I U/1 Bi l i r ubi n (−) Eos i no 6. 8% LDH 140 I U/1 Occul t (−)
Mono 5. 0% UN 9 mg/dl
Bas o 0. 4% Cr 0. 4 mg/dl Na 138 mmol /1 K 3. 6 mmol /1 Cl 100 mmol /1 CRP 10. 4 mg/dl
WBC;Whi t e Bl ood Cel l ,RBC;Red Bl ood Cel l ,Hb;Hemogl obi n,Ht;Hemat ocr i t ,Pl t;
Pl at el et s ,Gr an;Gr anul ocyt e,Lymph;Lymphocyt e Eos i no;Eos i nocyt e,Mono;Monocyt e, Bas o;Bas ocyt e,PT;Pr ot hr ombi n t i me,APTT;Act i vat ed par t i al t hr ombopl as t i n t i me,TT;
t hr ombi n t i me,HPT;Hepapl as t i n t es t ,TP;Tot al pr ot ei n,Al b;Al bumi n,T‑Bi l;Tot al Bi l i r - ubi n,D‑Bi l;Di r ect Bi l i r ubi n,AST;Al ani ne Ami not r ans f er as e,ALT;As par at e Ami not r ans f er - as e,ALP;Al kl i ne Phos phat as e,LDH ;Lact at e Dehydr ogenas e,UN ;Ur ea ni t r ogen,Cr;
Cr eat i ni n,Na;Nat r i um,K;Pot as s i um,Cl;Chl or i de,CRP;C‑r eact i ve pr ot ei n
Fi g.1. Endos copi c ul t r as onogr apy. The gal l bl adder
was enl ar ged, and debr i s was i dent i fied. A
s t r ongl y echoi c l es i on cons i s t ent wi t h a gal l s t one
was i dent i fied. The ar r owhead i ndi cat es debr i s .
ANCA)/myel oper oxi das e(MPO),79 EU;I gE,290 U/mL;I gG,2, 717 mg/dl;I gA,391 mg/dl;I gM,119 mg/dl;C , 6. 0 mg/dl;C ,＜40 mg/dl;C ,
＞4. 0 mg/dl;C, 90 mg/dl;C , 20. 5 mg/dl;CH , 48. 6;ant i nucl ear ant i body,40;r heumat oi d f act or , 232. 8 I U/ml;RAPA 1280;negat i ve,RNP;negat i ve, Sm ;negat i ve, SS‑A;negat i ve, AMA;negat i ve, AMA‑M Ab;＜5. 0, SMA;negat i ve, cyt opl as mi c ant i neut r ophi l i c cyt opl as mi c ant i body,＞10;and ant i phos phol i pi d ant i body,negat i ve.
Dur i ng t he eval uat i on,s ympt oms of l at er al par - es t hes i a devel oped i n t he upper ext r emi t i es .
El ect r omyogr aphy s ugges t ed mononeur i t i s mul t i pl ex.
A ki dney bi ops y was al s o per f or med at t hi s t i me but s howed no evi dence of vas cul i t i s . Becaus e of t he pr es ence of bot h a hi gh‑gr ade f ever and mononeur i t i t s mul t i pl ex,as wel l as hi s t ol ogi cal findi ngs of necr ot i z- i ng angi t i s and s er ol ogi cal findi ngs of P‑ANCA/MPO and hi gh t i t er s of C‑r eact i ve pr ot ei n,I gG,and I gE,
and eos i nophi l i a,t he di agnos i s of cl as s i c pol yar t her - i t i s nodos a was made. The pyr exi a,mononeur i t i s mul t i pl ex,and hi s t ol ogi cal evi dence of fibr i noi d ne- cr ot i zi ng angi t i s of s mal l mus cul ar ar t er i es wer e cons i s t ent wi t h t he di agnos t i c cr i t er i a f or pol yar t er i t i s
Fi g.2. Chol angi opancr eat ogr aphy r eveal ed i s ol at ed di s t ens i on of t he gal l bl adder . No pancr eat i c or bi l e duct i nvol vement was not ed. The s ol i d ar r owhead i ndi cat es t he cys t i c duct and t he dot t ed ar r owhead s hows pancr eat i c duct .
Fi g.3. Mi cr os copi c eval uat i on of t he r es ect ed gal l bl adder r eveal ed pr onounced i nfil t r at i on by l ymphocyt es and
eos i nophi l s . Necr ot i zi ng angi t i s and fibr i n i nfil t r at i on wer e al s o not ed i n bl ood ves s el s .
nodos a es t abl i s hed by t he Mi ni s t r y of Heal t h,Labor and Wel f ar e of Japan. On t he bas i s of t hi s di agnos i s , pr edni s ol one(60 mg/day)and cycl ophos phami de(50 mg/day) wer e admi ni s t er ed, pr oduci ng mar ked i mpr ovement s i n t he pat i ent ʼ s s ympt oms(pyr exi a and s er ol ogi cal i nflammat or y r eact i on)and gener al condi -
t i on over t he next s ever al days . However ,dys es t h- es i a and weaknes s of t he l ower ext r emi t i es wer e not al l evi at ed by t hi s t r eat ment .
D
ISCUSSION
Chol ecys t i t i s has many pos s i bl e caus es ,i ncl udi ng adenocar ci noma of t he gal l bl adder ,di abet es mel l i t us , t or s i on of t he gal l bl adder ,unus ual bact er i al i nf ect i ons of t he gal l bl adder ,par as i t i c i nf es t at i on of t he gal l bl ad-
der ,s ar coi dos i s ,car di ovas cul ar di s eas e,t uber cul os i s , s yphi l i s ,and act i nomycos i s. Al l t hes e caus es had t o be r ul ed out i n our pat i ent bef or e r ar er condi t i ons , s uch as vas cul i t i s ,wer e cons i der ed. When no obvi ous or gani c findi ngs ar e appar ent and i s ol at ed chol ecys - t i t i s i s pr es ent ,t he di ffer ent i al di agnos i s s houl d i ncl ude pol yar t er i t i s nodos a and ot her al l er gi c vas - cul i di t i es , s uch as pol yar t er i t i s nodos a, al l er gi c gr anul omat ous vas cul i t i s ,Wegner ʼ s gr anul omat os i s ,
and gi ant cel l ar t hr i t i s .
Al t hough t he pr es ent pat i ent had i s ol at ed gal l - bl adder vas cul i t i s ,becaus e chol ecys t i t i s was compl i - cat ed by mononeur i t i s mul t i pl ex,we cons i der t hi s cas e t o r epr es ent“gal l bl adder vas cul i t i s as a mani f es - t at i on of pol yar t er i t i s nodos a. ” The s ympt om of mononeur i t i s mul t i pl ex was not pr es ent unt i l af t er s ur ger y. Al s o a s i ngl e cas e wi t h s uch a cl i ni cal cour s e has been r epor t ed pr evi ous l y,by Li vol s i et al ..
Accor di ng t o t he r epor t of Chen et al .,when chol ecys - t i t i s occur s as a pr i mar y mani f es t at i on(i . e. ,i s ol at ed pol yar t er i t i s nodos a),t he pr ognos i s af t er s ur ger y i s gener al l y f avor abl e. I n cont r as t ,pol yar t er i t i s nodos a cl as s i fied as“gal l bl adder vas cul i t i s as a mani f es t at i on of pol yar t er i t i s nodos a”gener al l y has a poor pr ogno- s i s wi t h many compl i cat i ons . However ,t he cl i ni cal out come i n t he pr es ent cas e,whi ch we have cat egor -
i zed as“gal l bl adder vas cul i t i s as a mani f es t at i on, ” was ext r emel y f avor abl e,des pi t e s ens or y di s t ur - bances ,as pr evi ous l y r epor t ed.
Pol yar t er i t i s nodos a pr ogr es s es r api dl y and has a 6‑mont h mor t al i t y r at e of 50% i f l ef t unt r eat ed.
Ear l y and aggr es s i ve t r eat ment i s t her ef or e r equi r ed t o i mpr ove t he pr ognos i s . Wi t h cor t i cos t er oi d t her -
apy al one,t he 3‑year s ur vi val r at e r anges f r om 52%
t o 60%. However ,wi t h t he advent of combi nat i on t her apy wi t h cor t i cos t er oi ds and i mmunos uppr es - s ant s ,r emi s s i on can be i nduced and mai nt ai ned,and t he 5‑year s ur vi val has been i mpr oved t o 80% . Our pat i ent has s ur vi ved f or 6 year s af t er t he di agnos i s of pol yar t er i t i s nodos a,dur i ng whi ch t i me t he di s eas e has been i n r emi s s i on. Thi s f avor abl e out come i s at t r i but abl e i n par t t o ear l y di agnos i s and appr opr i at e t her apy. Accor di ng t o t he cl as s i ficat i on of Chen et al .t hi s cas e does not r epr es ent“i s ol at ed gal l bl adder vas cul i t i s ”but r at her a“gal l bl adder vas cul i t i s as a mani f es t at i on. ” However ,des pi t e t he poor pr ognos i s as s oci at ed wi t h“gal l bl adder vas cul i t i s as a mani f es t a- t i on”i n t he pas t,our pat i ent had an ext r emel y good cl i ni cal cour s e af t er ear l y s ur gi cal r es ect i on and dr ug t r eat ment .
I n concl us i on,we r epor t a pat i ent wi t h pol yar t er - i t i s nodos a pr es ent i ng as acut e chol ecys t i t i s ,whi ch we cons i der t o be“gal l bl adder vas cul i t i s as a mani f es t a-
t i on”of pol yar t er i t i s nodos a. Becaus e ear l y t r eat - ment r es ul t ed i n a f avor abl e cl i ni cal out come,at ypi cal t ypes of chol ecys t i t i s ,s uch as acal cul ous chol ecys t i t i s , s houl d s ugges t t he pos s i bi l i t y of s ys t emi c vas cul i t i s ,as i n t he pr es ent cas e.
R
EFERENCES
