Kev Koom Tes Ntawm IgG4-RD-txog rau raum Kab Mob Thiab Vasculitic Neuropathy

ali.ma@wecistanche.com

Benjamin Jiang, Zarife Sahenk, Anjali Satoskar, Miriam Freimer, thiab Isabelle Ayoub

Abstract

IgG4-Cov kab mob ntsig txog yog ib qho kev tiv thaiv kab mob sib kis kab mob uas muaj cov kab mob sib kis los ntawm cov ntaub so ntswg infiltration ntawm lgG4-cov plasma zoo thiab cov ntshav siab IgG4 concentrations. Peripheral neuropathy yog atypical tshwm sim ntawm tus kab mob no. Peb piav qhia txog qhov xwm txheej txawv ntawmvasculitic neuropathyhauv tus neeg mob nrogIgG4- hais txogmob raum. Ib tug poj niam muaj hnub nyoog 55-xyoo-laus hais tias muaj sab ceg tsis muaj zog zuj zus mus rau ob sab ceg tsis muaj zog, mob thiab loog ntawm ob txhais ceg, thiab kev tsis pom kev. Nws tau raug soj ntsuam yav dhau los rau kev poob phaus thiab ntshav ntshav nrog CT scan ntawm lub plab vim muaj kev txhawj xeeb txog kev mob qog noj ntshav. Abnormal txhim khu kev qhalub raumtau pom, thiab kuaj ua haujlwm thiabraumbiopsy tau zoo ib yam nrogIgG4- muaj kab mob. Myeloperoxidase antineutrophil cytoplasmic antibodies kuj zoo. Ua ke nrog tus neeg mob asymmetric ceg tsis muaj zog thiab mob neuropathy, qhov no ua rau muaj kev txhawj xeeb rau vasculitis. Sural paj hlwb biopsy paub tseebvasculitic neuropathy. Cov kev tshawb fawb tsis ntev los no tau pom tias muaj kev sib tshooj hauv cov yam ntxwv kho mob ntawmIgG4-Cov kab mob ntsig txog thiab antineutrophil cytoplasmic antibody-associated vasculitides, uas paub tias ua rauvasculitic neuropathy. Cov kws kho mob yuav tsum paub txog lub koom haum no, thiabIgG4-Cov kab mob ntsig txog yuav tsum tau txiav txim siab hauv kev kuaj mob sib txawv hauv cov neeg mob uas muaj kab mob peripheral neuropathy hauv cov ntsiab lus kho mob zoo.

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Taw qhia

IgG4- kab mob ntsig txog (IgG4-RD) yog ib qho kev tiv thaiv kab mob sib kis kab mob uas ua rau mob fibrosis thiabIgG4-zoo plasma cell infiltration ntawm cov ntaub so ntswg cuam tshuam thiab feem ntau cuam tshuam nrog cov ntshav siab lgG4 concentrations. Thawj tau piav qhia hauv cov neeg mob uas muaj tus mob autoimmune pancreatitis, txij li thaum nws tau pom tias muaj kev cuam tshuam rau ntau yam kabmob xws lilub raum, aorta, glands lacrimal, thiab salivary qog [1]. Kev koom tes hauv neurologic tsis tshua muaj thiab feem ntau tshwm sim raws li pachymeningitis lossis hypophysitis. Peripheral neuropathy tau raug tshaj tawm txawm hais tias nws yog qhov tsis tshua pom tshwm sim [2]. Peb piav qhia tus neeg mob nrog kev kuaj mob thawj zaugIgG4- hais txograumkab mob(IgG4-RKD) uas tau nthuav tawm nrog mob peripheral neuropathy thiab pom tias muajvasculitic neuropathyntawm sural paj hlwb biopsy.

Case piav qhia

Ib tug poj niam muaj hnub nyoog 55- xyoo uas muaj keeb kwm ntawm kev tsis haum rhinitis thiab mob hawb pob tshwm sim nrog sab ceg tsis muaj zog, uas tom qab ntawd nce mus rau ob sab ceg tsis muaj zog. Nws tau tshaj tawm txog 14 kg ntawm kev poob phaus uas tsis tau npaj siab rau xyoo dhau los, thiab kev soj ntsuam kuaj pom pom tias muaj hlau tsis txaus ntshav ntshav. Qhov no tau hais txog kev mob qog noj ntshav thiab ua rau CT scan ntawm lub plab uas ua rau pom qhov loj ntawm ob sab ntawm lub plab.lub raumnrog heterogeneous zoo sib xws li thiab poob ntawm ib txwm corticomedullary sib txawv.Lub raumbiopsy pom storiform fibrosis thiab plasma cellrich interstitial o nrogIgG4immunostaining qhia pawg ntawmIgG4-positive plasma cells (Daim duab 1), qhia txogIgG4- RKD. Ntshav creatinine yog 1.1 mg / dL. Tsis muaj cov protein lossis cov active sediment hauv cov zis. Kev soj ntsuam kuaj mob ntxiv (cov ntsiab lus hauv Table 1) yog qhov tseem ceeb rau cov ntshav qab zibIgG4qib 177 mg/dL(2.4-121 mg/dL), IgE qib 1,309.7IU/mL(1.5-165.3IU/mL), erythrocyte sedimentation rate (ESR) ntawm 77 (<30 mm/h),="" positive="" myeloperoxidase-antineutrophil="" cytoplasmic="" antibodies(mpo-anca)by="" elisa,and="" antinuclear="" antibody="" ≥1:1,280.antidouble="" stranded="" dna(antidsdna)antibody="" was="" negative,and="" complement="" levels="" were="">

Daim duab 1. CT scan ntawm lub plab thiab lub raum biopsy.A: CT scan ntawm lub plab nrog iodinated contrast qhia asymmetric o ntawm lub raum sab laug, nrog heterogeneous zoo sib xws li thiab poob ntawm ib txwm corticomedullary sib txawv ob sab. Cov kev tshawb pom zoo sib xws tau muaj nyob rau hauv lub raum txoj cai tab sis mus rau qib qis dua. B: Lub raum biopsy qhia focally accentuated lymphoplasmacytic inflammatory infiltrates nrog storiform fibrosis ntawm hematoxylin thiab eosin stain ntawm × 10 magnification, nrog (C)IgG4-cov ntshav plasma zoo (ntse taub hau) qhia txog cov tshuaj tiv thaiv kab mob immunohistochemical staining rauIgG4ntawm formalin-taw paraffin-embedded cov ntaub so ntswg ntawm × 20 magnification.

Tus neeg mob tau pom tom qab hauv tsev kho mob neurology ua ntej pib siv tshuaj tiv thaiv kab mob. Los ntawm lub sijhawm no, nws txhais ceg tsis muaj zog tau tshwm sim tau 8 lub hlis, thiab nws kuj tau tshaj tawm 6 lub hlis ntawm qhov mob kub hnyiab thiab loog ntawm nws sab sab. Kev kuaj xyuas yog qhov tseem ceeb rau ob tog tsis muaj zog ntawm pob taws dorsiflexion thiab plantarflexion, poob ntawm pinprick thiab kev vibration siab distal rau pob taws, thiab ib qho kev txav mus los. Kev ntsuam xyuas electrodiagnostic pom qhov ntev-dependent sensorimotor axonal polyneuropathy. Muab cov qauv asymmetric pib ntawm ceg tsis muaj zog, qhov hnyav ntawm nws qhov mob, thiab qhov zoo MPO-ANCA, muaj kev txhawj xeeb rauvasculitic neuropathy.

Table 1. Pertinent laboratory results

Biopsy ntawm sab laug sural paj hlwb tau ua, uas pom qhov hnyav myelinated thiab unmyelinated fiber ntau poob nyob rau hauv tag nrho cov fascicles, ib tug recanalized epineurial hlab ntsha, thiab ntom perineurial mononuclear cell infiltrates, raws li zoo raws livasculitic neuropathy. IgG4immunohistochemical staining pom tau tias qee qhov tawgIgG4- cov ntshav plasma zoo (Daim duab 2). Baseline positron emission tomography (PET) scan rau kev ntsuam xyuas ntawm kev koom tes hauv nruab nrog cev ntxiv tsis tau pom zoo los ntawm tus neeg mob lub tuam txhab pov hwm.

Table 2. Cov ntsiab lus qhia.

Tus neeg mob tau kho nrog prednisone 40 mg txhua hnub thiab muaj kev daws teeb meem ntawm qhov mob neuropathic thiab txhim kho txoj hauv kev tsis pub dhau 4 lub lis piam. Prednisone tau raug txiav tawm hauv 6 lub hlis. Nws kuj tau txais IV immunoglobulin 0.5 g / kg / hnub rau 4 koob tshuaj tom qab ntawd 0.4 g / kg txhua lub lim tiam rau 8 lub lis piam, tom qab ntawd rituximab 1 g txhua 6 lub hlis. Kev ntsuam xyuas tom qab pom tau tias muaj kev txhim kho nyob rau hauv qis qis qis zog thiab ib txwm mus. Thaum 6 lub hlis ntawm kev kho mob, ntshavIgG4theem normalized mus rau 57.1 mg / dL, IgE qib txo mus rau 283 IU / mL, thiab ESR normalized mus rau 25 mm / h. Nws lub raum ua haujlwm tau zoo li qub.

Daim duab 2. Sural paj hlwb biopsy.A: 1-micron plastic-embedded sections uas qhia txog lub nkoj recanalized nyob rau hauv lub epineurium thiab ob fascicles qhia (B) loj fiber ntau poob thiab (C) acellularity/fibrosis nyob rau hauv lub endoneurium. D: Cov hlab ntsha khov tshiab uas qhia qhov mob hauv lub perineurium ntawm 1 fascicle thiab (E) ib lub nkoj uas tau muab tshem tawm nrog qee cov perivascular mononuclear hlwb. F: Immunohistochemical staining rauIgG4qhiaIgG4-cov hlwb zoo (xub xub) ntawm × 20 magnification, nrog (G) × 40 magnification ntawm tib lub hom phiaj.

Kev sib tham

Tus neeg mob qhov kev sib xyaw ua ke ntawm cov kev tshawb pom histopathologic, kev tshawb pom radiographic, thiab cov ntshav siabIgG4theem ua tau raws li cov txheej txheem kom meejIgG4-RD raws li cov txheej txheem kuaj mob dav dav nrog rau cov txheej txheem kuaj mob tshwj xeeb rauIgG4-Cov kab mob hauv lub raum[3,4].Vasculitic neuropathyyog atypical feature ntawmIgG4-RD. Aortitis tuaj yeem pom, tab sis qhov mob ntawm cov hlab ntsha me me tau xav tias tsis tshua muaj [1]. Txawm li cas los xij, muaj ib lub cev loj hlob ntawm cov ntaub ntawv qhia txog kev sib koom ua ke ntawmIgG4-RD thiab vasculitis, nrog lossis tsis muaj neuropathy.

ANCA-associated vasculitides (AAV), uas suav nrog microscopic polyangitis (MPA), eosinophilic granulomatosis nrog polyangiitis (EGPA), thiab granulomatosis nrog polyangiitis (GPA), yog cov kab mob vasculitides feem ntau cuam tshuam nrog neuropathy uas tuaj yeem muaj cov yam ntxwv kho mob zoo ib yam li.IgG4-RD, thiab yuav tsum tau txiav txim siab hauv qhov kev kuaj mob sib txawv ntawmIgG4-RD [5]. Cov kev tshawb fawb tsis ntev los no qhia tias qhov zoo sib xws tau dhau los ntawm kev sib koom ua haujlwm kho mob thiab tau ua pov thawj ANCA zoo rau cov neeg mob.IgG4-RD, histopathologic thiab serologic nta ntawmIgG4-RD hauv cov neeg mob AAV, thiab cov neeg mob tau kuaj pom ob qho tib siIgG4-RD and AAV concomitantly [5 6,7,8].

Hauv ib txoj kev tshawb fawb, cov kws tshawb fawb tau tshuaj xyuas sural paj hlwb biopsy cov qauv ntawm 149 cov neeg mob uas muaj cov kab mob neuropathy uas muaj pawg ntawm cov kab mob inflammatory ntawm histopathology.29 cov neeg mob muaj.IgG4-zoo cell infltration ntawm histopathology, uas 20 kuj tau elevated serumIgG4theem.23 ntawm 29 tus neeg mob yav dhau los tau kuaj pom thawj zaugvasculitic neuropathyxws li MPA lossis EGPA [5].

Lwm cov ntaub ntawv tshaj tawm tau piav qhia txog tus neeg mob uas tau kuaj pom tias muaj EGPA hauv kev teeb tsa ntawm ntau yam mononeuropathies thiab purpura, nrog rau cov tawv nqaij biopsy uas qhia txog leukocytoclastic vasculitis. Tus neeg mob kuj tau nthuav cov qog lacrimal thiab submandibular thiab tau kuaj pom nrog Mikulicz tus kab mob, qhov tshwm sim ntawmIgG4-RD [6]. Lwm cov kev tshawb fawb tau piav qhia cov neeg mob ntsib cov txheej txheem rau ob qho tib siIgG4-RD thiab AAV thaum tsis muaj cov paj hlwb kev koom tes [7,8].

Xaus

Kev sib raug zoo ntawmIgG4-RD thiabvasculitic neuropathytseem tsis tau muaj tag nrho delineated. Tus neeg mob nyob rau hauv cov ntaub ntawv no ua tau raws li cov txheej txheem kom meejIgG4-RD, thiab kuj muaj ib tugvasculitic neuropathynrog MPO-ANCA zoo. Cov kws kho mob yuav tsum paub txog qhov muaj peev xwm ntawm qhov kev sib koom ua ke no thiab nkag siab tias qhov zoo ANCA tsis suav nrog kev kuaj mob.lg4 ua- RD, thiab ib yam nkaus, cov ntshav siabIgG4Qib tsis suav nrog kev kuaj mob ntawm AAV. Ntxiv rau,IgG4-RD yuav tsum raug txiav txim siab nyob rau hauv kev kuaj mob sib txawv hauv cov neeg mob uas muaj kab mob peripheral neuropathy thiab cov pov thawj ntawm cov kab mob inflammatory.

Cov ntaub ntawv

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