Lub raum tsis ua haujlwm sai tshwm sim los ntawm IgG4- Kab mob ntsig txog
Mar 18, 2022
Hu rau: Audrey Huaudrey.hu@wecistanche.com
Kev loj hlob sai rau raum tsis ua haujlwm cuam tshuam nrog Perirenal Capsular Lesion Vim IgG4-Cov Kab Mob Sib Kis
Nobuhide Endo, Hitomi Shimizu, Tomoki Tanaka, Yukiko Nakase, Tomohiro Kawazoe thiab Tomoharu Watanabe
Taw qhia
Lub raum kev koom tes hauvIgG4-cov kab mob ntsig txog(IgG4-RD) tuaj yeem tshwm sim raws li tubulointerstitial nephritis (TIN), glomerular lesions sawv cev los ntawm membranous nephropathy, loj qhov txhab, thiab retroperitoneal fibrosis (1). Ob peb tus yam ntxwv imaging txawv txav tau raug tshaj tawm (2). Ntau qhov chaw qis qis ntawm qhov sib piv-txhim kho kev suav tomography (CT) feem ntau pom. Cov kab mob loj heev kuj tsis tshua muaj thiab yuav tsum tau paub qhov txawv ntawm cov qog malignant. Hydronephrosis txuam nrog retroperitoneal fibrosis yog lwm qhov txawv txav. Cov kab mob perirenal yog qhov tsis tshua muaj kev nthuav qhia ntawm IgG4-RD(IgG4-cov kab mob ntsig txog) thiab ob peb txoj kev tshawb fawb tau qhia txog lawv qhov tseem ceeb hauv kev kho mob kom ntxaws (3, 4). Peb tshaj tawm ib rooj plaub ntawm kev loj hlob sairaumua tsis tiavnrog rau cov duab txawv txawv txawv, nrog rau cov nqaij mos nyob ib ncig ntawm lub raum thiab retroperitoneal fibrosis.
TSEEM CEEB NTAWM CISTANCHE: KHO KIDNEY SAIB XYUAS THIAB txhim kho raum ua haujlwm
Case Report
Ib tug txiv neej Nyij Pooj muaj hnub nyoog 71- xyoo uas muaj mob ntsws thiab pleural effusion raug xa mus rau peb lub tsev kho mob. Thaum xub thawj, nws tau kuaj pom tias muaj lub plawv tsis ua haujlwm vim qhov hnyav mitral regurgitation. Txawm hais tias nws cov tsos mob zoo dua nrog cov tshuaj diuretics, nws tau txhim khoraumua tsis tiavthiab raug xa mus rau peb lub tuam tsev nephrology. Ntawm qhov kev nthuav qhia, kev kuaj lub cev qhia cov hauv qab no: ntshav siab, 150/61 mmHg; lub plawv dhia, 85 beats / min; lub cev kub, 37-38 degree . Nws tsis muaj pob liab liab, lymphadenopathy, lossis edema ntawm cov ceg. Hnub 23 ntawm kev mus pw hauv tsev kho mob, tus neeg mob qhov kev sim kuaj tau pom tias lub raum tsis ua haujlwm nrog cov ntshav creatinine (Cr) qib ntawm 5.27 mg / dL, uas yog 1.34 mg / dL thaum nkag. Nws cov ntshav dawb suav yog 7,300 / mm3 thiab nws qib hemoglobin yog 10.0 g / dL. Nws qib C-reactive protein (CRP) tau nce me ntsis (3.44 mg / dL). Kev kuaj zis tsis pom muaj proteinuria lossis microscopic hematuria. Kev ntsuam xyuas kev tiv thaiv kab mob qhia pom cov ntshav nce siab IgG4, tsis muaj IgG nce (263 mg / dL thiab 1,339 mg / dL, raws li). Nws cov qib serum ntxiv (C3, C4, thiab CH50) nyob hauv qhov qub. Kev ntsuam xyuas rau autoantibodies, suav nrog cov tshuaj tiv thaiv nuclear, tiv thaiv neutrophil cytoplasmic, anti-SS-A, thiab cov tshuaj tiv thaiv SS-B, tsis zoo. Nws soluble interleukin{29}} receptor (SIL-2R) qib tau nce siab (5,316 U/mL). Tus neeg mob qhov kev kuaj tau raug muab sau tseg hauv Table. Contrast-enhanced CT pom tau tias diffuse cov ntaub so ntswg infiltration nyob rau hauv sab laug perirenal qhov chaw, nrog rau me me infiltration nyob rau hauv txoj cai perirenal qhov chaw thiab sab xis-sab hydronephrosis vim periaortic fibrosis. Txawm yog txoj cairaumyog atrophic thiab pom hydronephrosis, sab laugraumtau kho tsawg dua li txoj cai. Chest CT qhia me ntsis av-iav opacities nyob rau hauv ob sab ntsws ntsws. Tsis muaj qhov loj ntawm cov qog nqaij hlav. Tsis tas li ntawd, tsis muaj qhov pom tseeb ntawm lub raum txawv txav tau pom ntawm qhov sib piv-txhim kho CT scan ua tiav 8 xyoo ua ntej nws mus pw hauv tsev kho mob.
Hnub 26 ntawm kev nkag, nwsraum tsis ua haujlwmua rau muaj kev nce siab ntawm Cr thiab CRP (Cr 8.59 mg / dL, CRP 8.94 mg / dL) thiab cov tsos mob ntawm uremia, xws li xeev siab thiab qaug zog. Hemodialysis tau pib nrog vascular nkag catheter. Raws li cov kev tshawb pom no, peb txiav txim siab tias nws txoj kev vam meejraumua tsis tiavraug ntxias los ntawm IgG4-RD(IgG4-cov kab mob ntsig txog). Txawm li cas los xij, qhov tseeb etiology tsis paub. Yog li ntawd, peb tau ua ib qho laparoscopic biopsy ntawm sab laugraumthiab retroperitoneum.
CovraumKev kuaj ntshav pom tsis muaj tubulointerstitial nephritis tab sis diffuse me me wrinkling ntawm hauv qab daim nyias nyias ntawm glomerular capillaries. Tsis muaj lwm yam kab mob glomerular, xws li membranous nephropathy lossis glomerulonephritis, tau pom. Immunofluorescence microscopy tsis pom muaj qhov tseem ceeb ntawm IgG, IgA, IgM, C3, C4, lossis Clq. Electron microscopy qhia tias tsis muaj qhov tseem ceeb ab-normalities. CovraumCov ntaub so ntswg capsule tau thickened nrog inflammatory infiltration ntawm lymphocytes thiab plasma hlwb, ntxiv rau fibrosis. Immunohistochemical staining qhia qhov nruab nrab IgG4-cov ntshav plasma zoo suav ntawm kwv yees li 20 / siab zog thiab IgG4 / IgG-zoo cell piv ntawm 70 feem puaraumtshuaj ntsiav. Retroperitoneal biopsy qhia pom cov ntaub so ntswg ntom ntom nrog cov lymphocytes, macrophages, thiab cov ntshav plasma, uas zoo ib yam nrog peritoneal fibrosis. Tsis muaj cov cim qhia ntawm malignancy. Ua ke, lubraumcapsular lesions txuam nrog IgG4-RD(IgG4-cov kab mob ntsig txog)raug suav hais tias yog qhov laj thawj tseem ceeb ntawmraum tsis ua haujlwm.
Prednisolone (30 mg; 0.5 mg/kg) tau muab rau hauv tsev kho mob hnub 32, ua raws li kev sib tw ntawm 2- mus rau 3- lub lim tiam. Cov tsos mob ntawm kev mob tshwm sim, xws li ua npaws thiab nws qib CRP tau kho sai. Nwsraumkev ua haujlwmmaj mam txhim kho, thiab hemodialysis raug txiav tawm hauv tsev kho mob hnub 45. Nwslub raum ua haujlwmtau tswj xyuas zoo tom qab 6 lub hlis (Cr 1.5-2.0 mg/dL). Ua raws li CT pom tau tias kev txhim kho ntawm cov kab mob perirenal capsular thiab retroperitoneal fibrosis.
TSEEM CEEB NTAWM CISTANCHE: KHO KIDNEY SAIB XYUAS THIAB txhim kho raum ua haujlwm
Kev sib tham
IgG4-RD(IgG4-cov kab mob ntsig txog)yog kab mob fibroinflammatory uas cuam tshuam rau ntau yam kabmob, ua rau lub cev tsis ua haujlwm (5). Qhov tseem ceeb tshaj plawsraumkab mobtshwm sim los ntawm IgG4-RD(IgG4-cov kab mob ntsig txog)yog tubulointerstitial nephritis, uas ua rauraumua tsis tiavtsis pom muaj proteinuria lossis hematuria (1). Glomerular lesions, xws li membranous nephropathy nrog proteinuria kuj tau tshaj tawm. Lub raum pelvic thiab periureteral lesions los yog retroperitoneal fibrosis feem ntau ua rau postrenal raum tsis ua haujlwm. Qee cov neeg mob uas muaj IgG4-RD(IgG4-cov kab mob ntsig txog)muaj mob me me los yog tsis muaj tsos mob, vim lawv qhov txhab mob maj mam.
Cov txheej txheem kuaj mob dav dav rau IgG4-RD(IgG4-cov kab mob ntsig txog) are:(i) serum IgG4>135 mg/dL;(ii)>40% of IgG-positive plasma cells being IgG4 positive and >10 hlwb/HPF ntawm biopsy(6). Txawm li cas los xij, qhov rhiab heev thiab qhov tshwj xeeb ntawm cov ntshav ntshav IgG4 tsis txaus. Cov kev tshawb pom histopathological yuav tsum tau txhais nyob rau hauv cov ntsiab lus ntawm kev soj ntsuam thiab hluav taws xob tshawb pom, vim hais tias IgG4- cov hlwb zoo kuj pom nyob rau hauv ntau lwm yam mob, xws li malignancies, Castleman kab mob, granulomatosis nrog polyangiitis, thiab Sjogren's syndrome (7). Tsis ntev los no, American College of Rheumatology/European League Against Rheumatism classification specifications nrog rau cov yam ntxwv kho mob, serologic, radiologic, thiab pathologic nta tau tshaj tawm tias muaj kev ntseeg tau zoo (8). Cov xwm txheej tam sim no tau ua raws li cov qauv no txaus.
Cov kab mob hauv lub raum tau zoo nyob rau thawj lub hlis tom qab mus pw hauv tsev kho mob, qhov no, yog atypical ntawm IgG4-RD(IgG4-cov kab mob ntsig txog). Ua ntej lub raum biopsy, peb xav tias lub ntsiab ua rauraumua tsis tiavyog interstitial nephritis vim tias, tshwj tsis yog kev kuaj pom ntawm cov zis 2-microglobulin, cov txiaj ntsig ntawm urinalysis yuav luag zoo li qub. Txawm li cas los xij, pathological tseem ceeb ntawm perirenal capsular lesion tsis meej. Kev kuaj mob histopathological tsis pom muaj cov tsos mob ntawm interstitial nephritis lossis glomerular lesions, tshwj tsis yog muaj qhov sib txawv me me ntawm cov ntaub hauv qab daus ntawm glomerular capillaries, qhia txog lub raum ischemia, txawm hais tias IgG4- cov kab mob ntsig txog yuav tau ploj mus thaum lub sijhawm kuaj ntshav. Lub raum capsule tau koom nrog hauv o thiab fibro-sis nrog cov nplua nuj IgG4-cov hlwb zoo, uas yog raws li IgG4-RD(IgG4-cov kab mob ntsig txog).
Ntawm qhov sib piv-txhim kho CT, qhov sib txawv ntawm lub raum sab laug tsis muaj zog dua li ntawm lub raum sab xis, txawm tias atrophy ntawm lub raum sab xis. Qhov kev tshawb pom no kuj qhia tau tias txo qis perfusion ntawm sab laug b. Cov nqaij mos ntawm lub raum capsules ncig lub raum sab laug nkaus, nrog rau lub raum hilum.
TSEEM CEEB NTAWM CISTANCHE: KHO KIDNEY SAIB XYUAS THIAB txhim kho raum ua haujlwm
Raws li cov kev tshawb pom no, peb tau txiav txim siab tias cov kab mob inflammatory nyob ib ncig ntawm lub raum sab laug txo qhov perfusion ntawm lub raum ua rau lub raum tsis ua haujlwm. Nplooj raum yog ib yam kab mob pathological uas sab nraud ntawm lub raum vim hematoma lossis loj qhov txhab ua rau lub raum ischemia, ua rau kub siab thiabraumua tsis tiav(9). Xav tias retroperitoneal fibrosis ua rau compression ntawm lub ureter thiab impairs lub ureteral passage, nws yog tsim nyog los txiav txim siab tias perirenal o thiab fibrosis vim IgG 4- cuam tshuam cov kab mob uas ua rau lub raum tsis ua haujlwm thiabraumua tsis tiavlos ntawm tib lub mechanism raws li nyob rau hauv lub raum Page. Perirenal mos cov ntaub so ntswg infiltration txuam nrog IgG4-RD(IgG4-cov kab mob ntsig txog)tsis tshua muaj (2). Choj et al. thiab Chen et al. qhia txog perirenal capsule infiltra-tion vim IgG4-RD(3,4).Lub raumua tsis tiavyog me me thiab tsis tau sai heev nyob rau hauv cov ntaub ntawv no, thiab cov ncauj lus kom ntxaws mechanism ntawm kev loj hlob ntawmraum tsis ua haujlwmtsis tau piav. Tsis muaj qhov xwm txheej ceev ceevraumua tsis tiavxav tau kev lim ntshav ib ntus, raws li tau tshwm sim hauv cov ntaub ntawv tam sim no, tau tshaj tawm. Feem ntau, hauv cov neeg mob uas muaj IgG 4-RD, CRP theem qis thiab cov tsos mob tshwm sim qeeb. Nws tau raug tshaj tawm tias qhov siab CRP yog txuam nrog peri aortitis lossis periarteritis hauv cov neeg mob uas muaj IgG4-RD(IgG4-cov kab mob ntsig txog), thiab tus neeg mob tam sim no kuj muaj cov kab mob periaortic (10). Qhov siab CRP los yog kub taub hau qis hauv cov ntaub ntawv tam sim no tuaj yeem cuam tshuam txog kev mob vascular kuj. Txawm hais tias feem ntau ntawm Page raum qhia tau tias mob ntshav siab lossis mob raum tsis zoo, qee qhov mob hnyavraum ua tsis tiavtau raug tshaj tawm rau cov neeg mob uas muaj lub raum nyob ib leeg, xws li cov neeg mob uas tau hloov lub raum (11).Qhov no, lub raum sab xis tau ua tsis zoo los ntawm atrophy thiab hydronephrosis. Txawm li cas los xij, vim tsis muaj ntaub ntawv qhia txog tus neeg mob lub xeev ua ntej nws qhov kev nthuav qhia tam sim no, nws tseem tsis tau paub meej tias qhov atrophy ntawm lub raum sab xis tau tshwm sim los ntawm lwm yam uas tsis yog hydronephrosis. Raws li cov kev tshawb pom no, ntxiv rau txoj cai raum tsis zoo, txuas ntxiv compression ntawm lub raum sab laug los ntawm cov kab mob perirenal tau pom tias tau ua rau lub raum tsis zoo thiab ua rau muaj kev vam meej.raum tsis ua haujlwm. Rau qhov zoo tshaj plaws ntawm peb txoj kev paub, qhov no yog thawj daim ntawv tshaj tawm los piav qhia txog lub raum tsis ua haujlwm sai sai los ntawm cov kab mob perirenal cuam tshuam nrog IgG4-RD(IgG4-cov kab mob ntsig txog)hauv kev nthuav dav.
IgG4-RD(IgG4-cov kab mob ntsig txog)tuaj yeem cuam tshuam rau ntau yam kabmob thiab tuaj yeem ua rau mob thiab fibrosis, ua rau muaj ntau yam tsos mob. Malignant lymphoma tuaj yeem suav hais tias yog ib qho kev kuaj mob sib txawv hauv cov neeg mob uas muaj perirenal lossis retroperitoneal soft-tissue masses. Nyob rau hauv cov ntaub ntawv no, peb tau ua ib lub raum biopsy kom tsis txhob malignant lymphoma, txiav txim siab cov ntshav siab L-2R qib. Txawm li cas los xij, lub raum biopsy yog ib txoj kev tawm tsam thiab feem ntau ua tsis tau yog tias tus neeg mob qhov mob tsis zoo. Atypical imaging kev tshawb pom thiab cov tsos mob tshwm sim tuaj yeem ua rau muaj kev nkag siab zoo dua ntawm IgG4-RD(IgG4-cov kab mob ntsig txog), tso cai rau kom muaj txiaj ntsig zoo rau nws qhov kev kuaj mob thiab kev kho mob. Qhov xwm txheej atypical no qhia txog qhov sib txawv ntawm kev kho mob ntawm IgG4-RD(IgG4-cov kab mob ntsig txog).
TSEEM CEEB NTAWM CISTANCHE: KHO KIDNEY SAIB XYUAS THIAB txhim kho raum ua haujlwm
Cov ntaub ntawv
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3. Cho YJ, Jung WY, Lee SY, Song JS, Park HJ.Perirenal capsule and scrotal involvement inimmunoglobulin G4- cuam tshuammob raum: Kev tshuaj xyuas qhov xwm txheej. Rheumatol Int 38: 1941-1948, 2018.
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Nco tseg:cov saum toj no tsis yog ib daim ntawv teev tag nrho
