CASE REPORT IG4-d Chn Tubun Nph whu Aummun Pn nd h Tm Cu Rn Funn Yuk Tub 1, Fumh Akm 1, Tkh O 2, Jun Aj 1, Tkk Sk 3, Shnh Nh 4 nd Ih N 4 Ab W p n d mn wh hn ubun nph (TIN) hwn hh um mmunbun G4 (IG4) nnn. H um nn (S) v hd du nd m.9 m/dl 5.6 m/dl v 18 mnh. Rn bp hwd mkd IG4-pv pm nn n h num whu mu bnm nd IG4-d TIN w dnd. O pdnn dud h S nd IG4 v mmd. Th pn nd h IG4-d TIN n n n p pd bu hn v n pd whu nn un bnm, nd w hud nd hddn IG4-d TIN n hn n nun. K wd: IG4, ubun nph, IG4-d d (In Md 49: 1593-1598, 21) (DOI: 1.2169/nnmdn.49.3787) Indun Vu uv ubun nph (TIN) hv bn dnd, nudn, x, nu nd ummun ndn (1, 2). Rn, mn TIN d wh mmunbun G4 (IG4)- d d, uh ummun pn (AIP), hv bn pd (hn IG4-d TIN ) (3, 4). In h, vn h um IG4 v nd bundn IG4-pv pm nn n h n num w h. Fuhm, TIN hvn uh u whu h IG4-d d hv bn pd (5, 6); hwv, n m, h m u n unn b mn w n pnd n d. W pn h pn wh TIN hwn hh um IG 4 nnn nd mkd IG4-pv pm nn n h n num whu bvu h n nvvmn d wh IG4-d d, nudn h m u n unn b mn um nn (S) v 5.6 m/dl. C Rp A 76--d Jpn mn w d u hp h vun n nun n Ob 28. H S v hd du nd m.9 m/dl 5.6 m/dl v 18 mnh wh n mpm, xp pp. Th p md h nudd wnd hm nd hpnn, nd h mdn nudd mdpn b nd n nh hm. H hd vd md mn h ndn wh pd bd xmnn h md nun n 2. Th S v hd bn b ppxm.8.9 m/dl un Ap 27, nd hn hd du nd (F. 1A). Th hn v hd du dd m 29 IU/L 112 IU/L, wh n nun hd pd. Mumn um IG v nd un hd n bn pmd b. On xmnn, h w b, wh bd Dpmn Inn Mdn, N Pu Cn Hp, Ju, Dpmn Ph, N Pu Cn Hp, Ju, Dpmn Inn Mdn, Nk Rd C Hp, Nk nd Dvn Cn Nph nd Rhum, N Unv Gdu Sh Md nd Dn Sn, N Rvd pubn Ap 9, 21; Apd pubn M 13, 21 Cpndn D. Yuk Tub, ub.@m.m 1593
In Md 49: 1593-1598, 21 DOI: 1.2169/nnmdn.49.3787 A Admn 5 8 S [m/dl] 4.5 4 3.5 3 2.5 2 S GFR 7 6 5 4 3 GFR [ml/mn/1.73m 2 ] 1.5 1 2.5 1 Fb-5 Jun-5 O-5 Fb-6 Jun-6 O-6 Fb-7 Jun-7 O-7 Fb-8 Jun-8 O-8 B 4 Rn 3 25 bp PSL 6 2 15 12.5 1 7 3 5 25 S [m/dl] 4 3 2 S IG IG4 2 15 1 IG [m/dl], IG4 [m/dl] 1 5 O-8 Nv-8 D-8 Jn-9 Fb-9 M-9 Ap-9 M-9 Jun-9 Ju-9 Au-9 Sp-9 Fu1. Tmuhum nn(s)vndmdmun (GFR)bmnnhpn.SvdundndGFRdu dndvppxm18mnh.gfr wudbhmujpn mnw:gfr(ml/mn/1.73m 2 )=194 S - 1.94 A -.287 (A).Cnu mnnhpn.opdnn(psl)nnd4m/ddudhs, mmunbung (IG),ndmmunbunG4(IG4)v(B). pu 15/72 mmh. H hd h ubmndbu m, whh w 1 m n dm nd m ppn. Th m hd bn m 2 m 25 pvu, nd hd du dud n z. S mpm w bn. Un n dmn hwd un pn xn.3 /d nd β2-mbun 24.8 m/l. Un dmn hwd 1 4 d bd /hh-pw d whu. Un ph hwd n mnn pn h hn. Lb xmnn hwd h wn u: wh bd un 6.6 1 3 /μl wh 1.6% nph; hmbn, 1.4 /dl; nm ; bd u nn, 46.4 m/dl; S, 5.4 m/dl; p mnn, 9 IU/L; nn mnn, 11 IU/L; kn phph, 174 IU/L; bubn,.7 m/dl; m, 69 IU/L; C-v pn,.4 m/dl; hm- 1594
In Md 49: 1593-1598, 21 DOI: 1.2169/nnmdn.49.3787 A B C D E F F u 2. L hm p nd n h n b p.d u n n n p m nd mph w h n b nd ubu phw b v d( AE).Th m u h w dn bn m ( F).( A:H m x n nde n( HE) n n, wm n n ( B =5 μm),b:h m x n nde n n n 4,CE:P d dm h n m n v ( PAM) M n h m n[ C] 1,[ D] 2,[ E] 4,F:PAM n,h hm n n ( B =1 μm) ). bn A1, 5.5%; IG, 2,963 m/dl; IA, 94 m/dl; IM, 41 m/dl; IE, 125 IU/mL; IG1, 1,29 m/dl; IG2, 1,51 m/dl; IG3, 14 m/dl; IG4, 1,8 m/dl; C3, 6 m/ dl; C4, 17 m/dl; nd CH5, 35 U/mL. Th w n mnn mmph. Annu, n-dna, n-r, n-l, nd n-nuph pm nbd w nv. Pn mpud mph (CT) h nk pv hwd nm-zd kdn (11.2 nd 12. m) nd n bnm ndn n pn, b du, mph nd nd pnum. Cn mdum-nhnd CT w n pmd bu v n nun. Gum - nph hwd n bnm um-67 umun n kdn nd h n, huh um nph w pmd 7 d d hp. Nk unph hwd d v ndu d n h nm h ubmndbu nd. Th ndu w hmnu nd hph nn h wh mxm dm 1 m w nd. Bp h ndu w n pmd. A n bp pmn hwd du n nn pm nd mph whu nph. In b wh ubu ph w bvd (F. 2A-E). Th pmn h mp nnd 6 1595
In Md 49: 1593-1598, 21 DOI: 1.2169/nnmdn.49.3787 A B C Fu3. ImmunnnCD138(A),IG (B)ndIG4(C)nhnnum.NumuCD138-pv(pm)ndhnum (A).Appxmhh IG-pvnnhnum wpvig4(b,c).a-c:hh-mnn(b=1μm). mu, nn whh hwd b. A mu hwd n mn bnm. Th w n bvu ndp xp pn nd n p w hknn (F. 2F). Immunun hwd du nn IG nd bnn n h num. Th w n ppn dpn IG, IA, IM, bnn, C3 C1q n h mu. En mp hwd n-dn dp n h ubu bmn mmbn. N mu w vb n mp. Immunhhm w pmd CD138 (pm mk), IG nd IG4 n pn u (F. 3A-C). I vd h numu pm hd nd h num nd ppxm h h IGpv nn h num w pv IG4. IG4-d TIN whu bvu h n nvvmn d wh IG4-d d w dnd. O pdnn n n d 4 m/d dud h S, IG, nd IG4 v (F. 1B). A h mnh hp, h S, IG nd IG4 v hd dd 2.6 m/dl, 1,6 m/dl nd 389 m/dl, pv, nd h ubmndbu m dppd. Th d pdnn w du pd nd h w vn 5 m pdnn d wh d S v 2.1 m/dl Mh 21. Dun Rn, IG4-d d, pnd b AIP, h bn nzd. Th d h mmn u w: vd um IG4 v, bundn nn d n b IG4-pv pm, nd mkd mpvmn wh d hp (7). Vu d, uh Mkuz d, hn n dn, n hn nd pn b hv bn pd mnn IG4-d d d, nd TIN h bn nzd mnn h IG 4-d d n mn p (8, 9). W hv pnd h n u pn wh IG4- d TIN wh w n mpd wh h bvu IG4-d d, nudn h m u n unn b nd mn. Mn p IG4-d TIN hv d u, uh vn h um IG4 v, bundn IG4-pv pm nn n h n num, nd vb pn d hp (4, 1). On h h hnd, h m u n unn n IG4-d TIN h n bn d bu h numb p md, wh w pnn h m u n unn b nd mn n d. Tb 1 hw 16 IG 4-d TIN n whh h m u n unn b mn w hwn n h. Th m 1596
In Md 49: 1593-1598, 21 DOI: 1.2169/nnmdn.49.3787 Tb1. TmCuhSum CnnLvbndTmnn16CIG4-RdTubunNph C N. Cu S v b mn Tmn Cu S v mn Oh IG4-d n Rn bp 1 1.5 4.8 n 2 mnh -PSL 4 m/d 4.8 2. n 3 mnh SSG wn TIN+MN 15 2 2.7 3.4 n 3 mnh -PSL4 m/d 3.4 2.4 n 7 mnh AIP TIN+MN 13 3 1.2 3. n 3 mnh NA NA AIP TIN 9 4.9 2.2 n 7 mnh -PSL 4 m/d 2.2 1.6 n 3 wk AIP TIN 16 5 1. 3.9 n 12 mnh mpsl 32 m/d 3.9 1.5 n 2 mnh AIP TIN 17 6 1.2 1.4 n 14 mnh n mn dh * AIP TIN+MPGN k 18 Rn 7.9 5.6 n 18 mnh -PSL 4 m/d 5.6 2.4 n 2 mnh nn TIN pn 8 1.2 6.2 n 2 mnh -PSL 2 m/d n mnnn HD AIP TIN+MN 11 9 1.2 2.5 n 2 -PSL 3 m/d mpvd AIP TIN 4 1 1.7 3.6 n 7 -PSL 3 m/d 3.6 3. n 6 mnh AIP, SC TIN 4 11 NA mpsl mpu+-psl 5 m/d 2.5.6 n 3 mnh Mkuz' d TIN 12 12 NA -PSL 4 m/d 1.4 1.2 n 4 wk nn TIN 5 13 NA -PSL 5 m/d 1.8 1.3 n 5 nn TIN 5 14 NA -PSL 6 m/d 1.1 1.1 n 9 AIP TIN 5 15 NA -PSL 4 m/d 1.5 1.2 n 4 mnh nn TIN+MN 6 16 NA mpsl pu+-psl n n 2.3 AIP TIN 3 Abbvn: NA, n vb; S, um nn [m/dl]; -PSL, pdnn; mpsl, mh pdnn; HD, hmd; SSG, ubmndbu v nd; AIP, ummun pn; SC, n hn; TIN, ubun nph; MN, mmbnu nphph; MPGN, mmbnpv munph. *Dh m mpm pnumn u n unn n h p dvdd n w p: u, n whh n u pd pd n w mnh, nd hn, n whh n u pd w v n m. Hwv, n hn, w n whh n u pd du v n pd pd pd h nd n pd bu h m u n unn b mn w n dd n h p. In h pn, n unn du dd v 18 mnh (F. 1A). Th u u h hn x n IG4-d TIN, nd w hud b w IG4-d TIN pn w v n pd whu nn un bnm. Th m u n unn d hp n IG4-d TIN vb n m p, huh h w pn wh IG4-d TIN wh w n b dnnu hmd mn d hp (11). On h h hnd, h n-m pn n unn n IG4-d TIN un, bu h bvn pd pnd n w h nd whn v mnh. Ahuh h m p n whh n unn h bn b v, n ddn u, h numb md (3, 5, 12). I un whh n m mun d n mnnn hp n IG4-d TIN. Pn wh hv bn mkn p v h n m wh mnnn hp nd dvpd TIN dnnun d hp h d upp h np h mnnn hp n n IG4-d TIN (5, 11, 12). Indd, u pn h bn mkn p wh 5 m pdnn d d. On h h hnd, pn wh TIN dnnud d hp 8 mnh, huh h u dnnun w n hwn n h (13). In AIP pn, h pd p n m 6% 26% nd h n nnu nnn h m d mnnn hp (14). Fm h vwpn h mnn h IG4-d d, h p n IG 4-d TIN pn mh n b w (1, 11). Cu bvn n v h n m, d mnnn hp. I n umu IG4-d TIN wh dd n u nd vw hm h vu pn h n u nd h b mn mhd h d. In nun, h m u n unn n h pn nd h IG4-d TIN n n n p pd bu hn v n pd whu nn un bnm, nd w hud nd hddn IG4-d TIN n hn n nun pn m nph. Rn 1. Bk RJ, Pu CD. Th hnn p u ubun- nph. Nph D Tnpn 19: 8-11, 24. 1597
In Md 49: 1593-1598, 21 DOI: 1.2169/nnmdn.49.3787 2. Kz A, Fh AJ, Snm P, Nvn TE, Km Y, Bukwk RJ. R nbd ubun nph nn n humn n-ubu bmn mmbn nph d wh mmbnu nphph. Am J Md 93: 691-698, 1992. 3. Tkd S, Hk J, K T, Tkd C, Tkzku E. IG4- d dph ubun nph mpn ummun pn. Nph D Tnpn 19: 474-476, 24. 4. Ynd K, Mu K, Km K,. Tubun nph d wh IG4-d ummun d. Am J Kdn D 5: 455-462, 27. 5. Sk T, S A, Ymzk H,. Tubun nph d wh IG4-d m d. Cn Exp Nph 11: 168-173, 27. 6. Sk T, Im N, I T, Ymzk H, Nh S. Mmbnu nphph d wh IG4-d m d nd whu ummun pn. Cn Nph 71: 173-178, 29. 7. Kmw T, Okm A. IG4-d n d. Wd J Gn 14: 3948-3955, 28. 8. Ymm M, Tkhh H, Oh M,. A nw npuzn Mkuz d n IG4-d pm d. Md Rhum 16: 335-34, 26. 9. Cn LD, Chn SL, Dhpnd V,. Pudumu du IG4 mmun-mpx ubun nph d wh ummun pnn d. Am J Su Ph 31: 1586-1597, 27. 1. Sk T, Nh S, I T,. Rn n n IG4-d m d. Inn Md 46: 1365-1372, 27. 11. Sd Y, Hnm N, Hm H,. A IG4-d ubun nph hwn h pn n dunn u ummun pn. Nppn Jnz Gkk Sh 52: 73-79, 21 (n Jpn, Ab n Enh). 12. Ak A, S K, Ibh M,. A Mkuz d mpd wh v n nph d wh IG4. Cn Exp Nph 13: 367-372, 29. 13. Wn SJ, Jnkn DA, Bm CO. Nphph n IG4- d m d. Am J Su Ph 3: 1472-1477, 26. 14. Kmw T, Sk K. Thpu ummun pn. Adv Md S 53: 145-148, 28. 15. H P, Ru P, Smmn C, Wn K, Dw K. Au kdn nju nd pnu n pn wh db nd ubmndbu m. Am J Kdn D 54: 375-38, 29. 16. Muhm M, Tmzwk J, Gkmn JD. Chn ubun nph pnn mup n ndu nd pn nun. Am J Kdn D 49: 7-1, 27. 17. Uhm-Tnk Y, M Y, Kmu T,. Au ubun nph d wh ummun-d pn. Am J Kdn D 43: 18-25, 24. 18. Mm J, Hw Y, Fukuhm H,. Mmbnpv munph-k mu d nd nun ubun nph mpn IG4-d ummun pn. Inn Md 48: 157-162, 29. 21 Th Jpn S Inn Mdn hp://www.nk..jp/mndx.hm 1598