IgG4-related tubulointerstitial nephritis accompanied with cystic formation

Transcription

1 Fukuhr et l. BMC Urology 2014, 14:54 CASE REPORT IgG4-relte tuulointerstitil nephritis ompnie with ysti formtion Open Aess Hieo Fukuhr 1, Yoshinori Tniguhi 2, Mnu Mtsumoto 3, Noto Kuro 4, Stoshi Fukt 1, Keiji Inoue 1*, Shimpei Fujimoto 2, Yoshio Ter 2 n Tro Shuin 1 Astrt Bkgroun: An immunogloulin G4 (IgG4)-relte isese is importnt isese in ifferentil ignosis of tumors in kiney, pnres, lung n other orgns. The imging finings of IgG4-relte kiney iseses re usully expresse s efet ontrst region, while ysti formtion in kiney is extremely rre. Here, we report se of IgG4-relte tuulointerstitil nephritis with renl ysti hnge use y the nrrowing or ostrution of olleting ut in renl meull. Cse presenttion: Aominl ontrste CT sn showe mm ysti tumor t the upper pole of the right kiney n multiple low-ttenution res in the left kiney. 18 F-fluoroeoxygluose (FDG)-PET/CT sn showe moerte FDG umultion of ysti tumor in mrginl lesion. In ition, FDG-PET/CT sn lso showe moerte FDG umultion in the pnreti oy. Lprosopi right nephretomy ws performe. Histologil exmintion ws revele lymphoplsmyti infiltrte with fol firosis n severe nrrowing or ostrution of lumen of olleting ut in renl meull. Furthermore, the IgG4 positive plsm ells infiltrte exeeing 10 ells per one high-power fiel in renl meull. The rtio of IgG4-plsm ells to IgG-positive plsm ells ws out 50%. The serum level of IgG4 ws lso elevte (218 mg/l). Bse on these finings, we finlly ignose IgG4-relte tuulointerstitil nephritis with renl ysti hnge. Conlusion: IgG4-relte kiney isese might use ysti formtion y severe nrrowing n ostrution of olleting ut. Keywors: IgG4-relte tuulointerstitil nephritis, Renl yst hnge, Colleting ut Bkgroun An immunogloulin G4 (IgG4)-relte isese is newlypropose linil isese entity hrterize y elevte serum IgG4 n IgG4 positive plsm ell infiltrtion in vrious orgns. IgG4-relte isese ws first esrie s utoimmune pnretitis (AIP) n hs susequently een esrie in other orgns [1-3]. The ffete ommon site is onsiere to e pnres, liver, slivry gln, lung, rest, prostte n kiney. The histologil hrteristis re lymphoplsmyti infiltrte, IgG4 plsm ell n firosis [4]. Imging feture is often esrie s n interstitil lesion [5]. However, we sometimes enounter pseuotumor formtion whih is not esy to istinguish from mlignnt tumor [6,7]. Herein, we report se of IgG4- * Corresponene: keiji@kohi-u..jp 1 Deprtment of Urology, Kohi Meil Shool, Kohsu, Oko, Nnkoku, Kohi , Jpn Full list of uthor informtion is ville t the en of the rtile relte tuulointerstitil nephritis with renl ysti hnge use y the nrrowing or ostrution of olleting ut in renl meull. Cse presenttion A 63-yer-ol womn ws referre to Kohi University Hospitl with renl tumor isovere y meil exmintion, inientlly isovere on ompute tomogrphy (CT) sn. There ws no previous meil history n fmily history of kiney isese. Her vitl signs were norml vlue. Bloo eletrolytes, proteinogrm, renl funtion n hepti enzymes showe ll norml vlue. Solule IL-2 reeptor ws slightly elevte (703 U/ml (norml, )). The other tumor mrkers were ll within the norml rnge. Aominl ontrste CT sn showe mm ysti tumor t the upper pole of the right kiney n multiple lowttenution res in the left kiney (Figure 1) Fukuhr et l.; liensee BioMe Centrl Lt. This is n Open Aess rtile istriute uner the terms of the Cretive Commons Attriution Liense ( whih permits unrestrite use, istriution, n reproution in ny meium, provie the originl work is properly reite. The Cretive Commons Puli Domin Deition wiver ( pplies to the t me ville in this rtile, unless otherwise stte.

2 Fukuhr et l. BMC Urology 2014, 14:54 Pge 2 of 5 e f g h Figure 1 Imging finings.. Aominl ompute tomogrphy sn shows mm ysti lesion in right kiney (rrows).. PET/CT sn showe moerte FDG umultion of ysti lesion in right kiney (rrows).. PET/CT sn showe moerte FDG umultion of lung tumor (rrows).. PET/CT sn showe moerte FDG umultion of pnreti oy (rrows). e. Diffusion-weighte MRI showe high intensity re in pnreti oy (rrows). f. MRCP showe irregulr nrrowing in min pnreti ut (rrows). g. PET/CT sn emonstrte erese of FDG umultion in pnreti oy (rrows). h. PET/CT sn emonstrte erese of FDG umultion in lung tumor (rrows). 18 F-fluoroeoxygluose (FDG)-Positron Emission Tomogrphy/Compute Tomogrphy (PET/CT) sn showe moerte FDG umultion of ysti tumor in mrginl lesion (Figure 1). In ition, FDG-PET/CT sn lso showe moerte FDG umultion in the pnreti oy n smll lung mss (Figure 1, ). Diffusionweighte mgneti resonne imges (MRI) showe high intensity re n fol enlrgement on pnreti oy.

3 Fukuhr et l. BMC Urology 2014, 14:54 Furthermore, min pnreti ut showe irregulr nrrowing on mgneti resonne holngio pnretogrphy (MRCP) (Figure 1e n f), initing utoimmune pnretitis. Pge 3 of 5 Multiple low-ttenution re in left kiney fitte the imging finings of tuulointerstitil nephritis. However, right renl ysti tumor h iffuse wll thikening n wek enhnement of the ysti wll on CT sn n renl ② ③ e f g h ① Figure 2 Mrosopi n mirosopi finings.. The mrosopi finings of renl ysti tumor (rrows).. low power loupe imges of 3 prts (①; ysti vity, ②; renl ortex, ③; renl meull).. H&E stin. Mrke lymphoplsmyti infiltrte n storiform firosis.. Storiform firosis with Azn stining e. Immunohistohemil result. A signifint mount of IgG4-positive plsm ells infiltrtes in renl meull. f. Colleting ut ws ompresse longituinry in renl ortex (rows). g. Colleting ut ws ilte in renl in renl meull (rrows). h. Colleting ut eme nrrowe n ostrute in renl meull (rrows).

4 Fukuhr et l. BMC Urology 2014, 14:54 Pge 4 of 5 ell rinom with ysti hnge ws suspete. In imging test, it ws very iffiult to istinguish from tuulointerstitil nephritis n mlignnt renl ysti tumor. Ultimtely, we me preopertive ignosis s suspiious of renl ell rinom with ysti hnge n then performe lprosopi right nephretomy. The mrosopi finings of ysti wll were gry white olor n no hemorrhge ws oserve insie (Figure 2 n ). Histologil exmintion ws revele lymphoplsmyti infiltrte with storiform firosis in renl ortex (Azn stining positive) (Figure 2 n ). Immunohistohemilly, the IgG4 positive plsm ells infiltrte exeeing 10 ells per one high-power fiel (Figure 2e). The rtio of IgG4-plsm ells to IgG-positive plsm ells ws out 50%. Colleting ut ws ompresse longituinlly y severe inflmmtion n firosis in renl ortex roun yst (Figure 2f). In ontrst, olleting ut h teneny to ilte in renl meull jent to renl ortex n ostrut in renl meull wy from renl ortex (Figure 2g n h). In itionl immunohistohemil nlysis of yst wll showing olleting ut mrkers, epithelil memrne ntigen (EMA), the pire ox (PAX) 2 n PAX8, were positive in the lining ell of the yst wll (Figure 3 n ). While proximl tuule mrkers, CD10 n renl ell rinom mrker ntigen (RCC-M), were negtive (Figure 3 n ). Also, ysti wll h no signifint mlignnt omponents. Bse on pthologil results fter surgery, then we nlyze store preopertive serum retrospetively. As result, the ptients h hypoomplementemi n polylonl gmmopthy with elevte levels of serum IgG (1934 mg/l) n IgE (1061 IU/ml). The serum level of IgG4 ws lso elevte (218 mg/l). Finlly, we ignose IgG4-relte tuulointerstitil nephritis with renl ysti hnge oring to ignosti lgorithm of the Jpn Soiety of Nephrology. After the opertion, the ptient reeives steroil therpy. Orl prenisolone t initil ose of 30 mg/y ws ministrte fter surgery. Six month fter therpy, serum level of IgG4 returns to norml level (28.1 mg/l) n FDG-PET/CT sn showe ispperne of FDG umultion in pnreti oy n lung mss (Figure 1g n h). Disussion To our knowlege, renl ysti formtion of IgG4-relte isese in our se is the first reporte se in English litertures. The imging finings of IgG4-relte kiney iseses re usully expresse s efet ontrst region se minly on interstitil lesion. While, the mss formtion is lso foun in some ses [6,7]. These ses re iffiult to istinguish from renl ell rinom y imging finings. The imging finings of IgG4-relte kiney iseses hve some vritions. In the representtive imging stuy y Tkhshi et l., they tegorize IgG4-relte kiney Figure 3 Immunostining finings.. Immunohistohemil result. PAX2 highlights ells lining in the yst wll (olleting ut mker).. EMA highlights ells lining in the yst wll (olleting ut mker).. RCC-M negtive ells lining in the yst wll (proximl ut mker).. CD10 negtive ells lining in the yst wll (proximl ut mrker).

5 Fukuhr et l. BMC Urology 2014, 14:54 Pge 5 of 5 isese into four types (1. mss or noule; 2. iffuse pthy; 3. kiney swelling; 4. pelvi wll thikening) [8]. This se emonstrte ysti formtion n i not orrespon to ny four types in imging inspetion. Then, ysti type of IgG4-relte kiney isese is extremely rre. We ssume the mehnism of ysti formtion in this se oring to pthologil exmintion: lymphoplsmyti lesion n storiform firosis in IgG4-relte kiney isese tens to our in renl ortex. But if inflmmtion n firosis spre to renl meull, we must onsier out ffet on olleting ut. In this se, the inflmmtion n firosis in renl ortex spre to renl meull n inue severe nrrowing or ostrution of lumen of olleting ut in renl meull (Figure 2h). Colleting ut in renl meull jent to renl ortex h teneny to ilte ue to mil inflmmtion n firosis (Figure 2g), wheres olleting ut in renl ortex roun yst ws ompresse longituinlly y sever inflmmtion n firosis n i not show iltion (Figure 2f). Thus, spre of inflmmtion n firosis from renl ortex inue trophy n nrrowing or ostrution of lumens of olleting ut in renl meull (Figure 2h). These finings provie tht the severe nrrowing n ostrution of olleting ut in renl meull followe y the iltion of proximl site of olleting ut system n finlly le to ysti formtion in renl ortex. In immunohistohemil nlysis of yst wll showing the ysti hnge, olleting ut mrkers, EMA, PAX2 n PAX8, were positive in the lining ell of the yst wll (Figure 3 n ). While proximl tuule mrkers, CD10 n RCC-M, were negtive (Figure 3 n ). Furthermore, the ostrution exists only in olleting ut of renl meull n there is no jent ysti lesion. One might point tht IgG4-relte kiney isese ourre with simple renl yst in this se. However, simple renl yst generlly rises from proximl origin [9] n she hs never etete renl normlity y helth hek. Therefore, we hypothesize tht IgG4-relte kiney isese might use ysti formtion y severe nrrowing n ostrution of olleting ut. If this ysti tumor h een ignose s n IgG4- relte kiney isese y neele iopsy efore surgery, we oul voi unneessry opertion n provie steroi therpy. Therefore, it is importnt to onsier ifferentil ignosis with IgG4-relte isese from mlignnt ysti isese similr to imging in this se. Further se investigtions on reltionship etween imging finings n pthologil results shoul e exmine in IgG4-relte kiney isese. Conlusion IgG4-relte kiney isese might use ysti formtion y severe nrrowing n ostrution of olleting ut. Consent Written informe onsent ws otine from the ptient for pulition of this se report n the ompnying imges. A opy of written onsent is ville for review y the Eitor-in-Chief of this journl. Arevitions IgG4: Immunogloulin G4; AIP: Autoimmune pnretitis; PAX: Pire ox; EMA: Epithelil memrne ntigen; RCC-M: Renl ell rinom mrker ntigen. Competing interests The uthors elre tht they hve no ompeting interests. Authors ontriutions HF rfte the report. YT, SF, KI, SF, YT n TS re for ptient n pprove the finl version of the mnusript. MM n NK performe histopthologil exmintions. All uthors reviewe the report n pprove finl version of the mnusript. Aknowlegements No soures of funing hve to e elre. Author etils 1 Deprtment of Urology, Kohi Meil Shool, Kohsu, Oko, Nnkoku, Kohi , Jpn. 2 Deprtment of Enorinology, Metolism n Nephrology, Kohi, Jpn. 3 Deprtment of Pthology, Kohi Meil Shool, Kohi, Jpn. 4 Deprtment of Dignosti Pthology, Kohi Re Cross Hospitl, Kohi, Jpn. Reeive: 14 Ferury 2014 Aepte: 9 July 2014 Pulishe: 20 July 2014 Referenes 1. Stone JH, Zen Y, Desphne V: IgG4-relte isese. 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