Kaum Nqe Lus Nug Thiab Lus Teb Txog Purpura Nephritis

Henoch-Schoenlein purpura, tseem hu ua Henoch-Schoenlein purpura (HSP), tam sim no hu ua IgA vasculitis (IgAV). Nws yog ib qho kab mob vasculitis uas tshwm sim los ntawm neutrophil infiltration ntawm phab ntsa ntawm arterioles, capillaries, venules, thiab glomeruli nrog IgA1 deposition. IgAV kev koom tes ntawm ob lub raum hu ua IgAV nephritis, uas yog qhov peb tau paub ua "Henoch-Schoenlein purpura nephritis". Nws yog tus cwj pwm los ntawm kev mob glomerulonephritis. Lub raum pathology qhia tau hais tias fluorescent staining tuaj yeem nthuav tawm granular deposition ntawm IgA nyob rau hauv glomerular mesangial cheeb tsam. IgAV nephritis zoo li tsis tshua muaj tshwm sim hauv cov neeg laus nephrology departments. Hais txog cov xwm txheej saum toj no, kuv muaj ob peb lo lus nug thiab tau pom cov lus teb hauv cov ntaub ntawv. Kuv xav qhia rau koj paub.

Nyem rau Cistanche rau mob raum

Q1 Yog tias tus neeg mob tsis tau mus rau lub raum biopsy, nws puas tuaj yeem kuaj tau tias yog IgAV nephritis?

Yog lawm. Xyoo 2023 IgAV raum raug mob kev kuaj mob thiab cov txheej txheem kho mob [1] hais tias kev kuaj mob ntawm IgAV raum raug mob yog nyob ntawm daim tawv nqaij ib txwm (touchable daim tawv nqaij purpura yam tsis muaj thrombocytopenia thiab coagulopathy), mob caj dab / arthralgia, mob plab plab thiab / lossis melena thiab lub raum. Qhov muaj tsawg kawg yog ob ntawm cov kev kho mob no ua rau kev kuaj mob yooj yim. Txawm li cas los xij, thawj lub cim ntawm kwv yees li 1/4 ntawm cov neeg mob tsis yog pob liab liab. Daim tawv nqaij los yog lub raum biopsy paub meej tias leukocytic vasculitis nrog qhov tseem ceeb IgA deposition, uas tuaj yeem paub meej tias IgAV. Tus neeg mob no muaj cov tawv nqaij ecchymoses, nrog rau hematuria thiab proteinuria, thiab tuaj yeem kuaj tau IgAV nephritis. Tsis tas li ntawd, lub raum pathology qhia tias IgA 4+ tso rau hauv thaj chaw glomerular mesangial, ntxiv kev lees paub qhov kev kuaj mob ntawm IgAV nephritis.

Q2 IgAV yog kab mob vasculitis. Vim li cas tus neeg mob qhov kev tshwm sim ntawm daim tawv nqaij yog txwv rau cov ceg qis thiab tsis yog lwm qhov ntawm lub cev?

Cov kab mob IgAV yog tsim thaum IgA1 tso rau ntawm phab ntsa ntawm cov hlab ntsha me, uas ua rau cov phab ntsa puas thiab ua rau cov tshuaj tiv thaiv. Cov qe ntshav liab dhau los ntawm cov hlab ntsha tsis zoo, tsim ecchymoses thiab purpura. Thaum cov qe ntshav liab ntau dhau thiab sib sau hauv qab ntawm daim tawv nqaij, cov tshuaj tiv thaiv kab mob ua rau cov ntaub so ntswg edema, ua rau palpable purpura[2]. Cov kab mob sib txawv thiab cov duab yog tsim nyob ntawm txoj kab uas hla ntawm cov hlab ntsha cuam tshuam. Cov kab mob feem ntau yog faib ua symmetrically thiab muaj ntau dua nyob rau hauv qhov distal ntawm cov ceg qis, ko taw, pob tw, thiab lwm yam, thiab tsis tshua muaj nyob rau hauv lub taub hau, ntsej muag, pob tw, tes, thiab lwm yam. Qhov no tej zaum yuav yog vim cov ntshav khiav qeeb. nyob rau hauv qis qis ntawm cov ceg, uas ua rau nws yooj yim dua rau IgA tso rau hauv cov hlab ntsha me hauv cov khoom no[2-4].

Q3 Puas yog IgAV lesions xav tau daim tawv nqaij biopsy?

Tsis yog 2019 European IgAV kev pom zoo hais tias: Rau cov neeg mob uas muaj pob khaus atypical thiab / lossis thaum lwm yam kev kuaj mob tsis suav nrog, yuav tsum tau kuaj xyuas daim tawv nqaij xws li IgA-tshwj xeeb immunofluorescence staining; Txawm hais tias daim tawv nqaij biopsy yog qhov tseem ceeb rau kev lees paub IgAV, cov neeg mob uas raug purpura nyob rau hauv qis qis thiab pob tw feem ntau tsis xav tau daim tawv nqaij biopsy.

Q4 Puas yog lub raum pathological immunofluorescence staining nyob rau hauv cov neeg mob uas muaj IgAV nephritis tsuas yog IgA deposits?

Lub raum biopsy ntawm IgAV nephritis paub meej tias leukocytic vasculitis nrog feem ntau IgA deposits. Txawm li cas los xij, 2019 European IgAV kev pom zoo hais tias: Qhov tsis muaj IgA immunofluorescence staining hauv biopsy tsis suav nrog kev kuaj mob ntawm IgAV. Cov kev tshawb fawb tau tshaj tawm tias tsawg heev ntawm IgAV nephritis raum pathology feem ntau yog IgG deposition, thiab cov kab mob tshwm sim ntawm cov neeg mob no hnyav dua li cov neeg uas feem ntau IgA deposition, feem ntau nrog cov tshuaj tiv thaiv GBM zoo [5].

Q5 Ntxiv rau daim tawv nqaij thiab ob lub raum, dab tsi lwm yam kabmob cuam tshuam los ntawm IgAV?

Ntxiv rau qhov cuam tshuam rau ntawm daim tawv nqaij thiab lub raum, IgAV feem ntau cuam tshuam rau lub plab zom mov thiab pob qij txha. Cov tsos mob xws li mob plab, plab zom mov los ntshav, ntshav hauv cov quav, mob caj dab lossis mob pob qij txha. Nws kuj tuaj yeem cuam tshuam rau lub ntsws, lub hauv nruab nrab ntawm lub paj hlwb, txiav txiav, thiab lwm yam, tshwm sim raws li hnoos, mob hauv siab, hemoptysis, cerebral hemorrhage, cerebral ischemic infarction, pancreatitis, qaug dab peg, kev hloov hauv kev nco qab, thiab lwm yam.

Q6 Puas yog IgAV thiab IgA nephropathy yog tib yam kab mob?

Tsis yog. IgAV feem ntau yog hais txog vasculitis tshwm sim los ntawm IgA deposition nyob rau hauv cov phab ntsa ntawm cov hlab ntsha me. Clinically, nws yog feem ntau manifested li non-thrombocytopenic purpura, mob plab, pob qij txha o thiab mob, thiab raum kev koom tes. IgA nephropathy yog ib qho kev kuaj mob immunopathological. Cov tsos mob tseem ceeb hauv kev kho mob yog rov tshwm sim microscopic lossis macroscopic hematuria. Nws yog tus cwj pwm los ntawm kev tso tawm ntawm lub cev tiv thaiv kab mob los ntawm IgA nyob rau hauv glomerular mesangial cheeb tsam hauv lub raum immunopathology, nrog rau glomerular mesangial hyperplasia raws li kev hloov pauv hauv keeb kwm. Txij li thaum ob qho tib si IgAV thiab IgA nephropathy muaj cov yam ntxwv pathophysiological ntawm IgA deposition, thiab IgAV feem ntau nrog rau lub raum kev koom tes, ob qho tib si uas tau tshwm sim raws li glomerular mesangial proliferation thiab tseem ceeb IgA deposition nyob rau hauv mesangial cheeb tsam, tus tshiab vasculitis naming kev pom zoo ntawm 012. Chapel Hill Conference tau hais txog IgA nephropathy rau IgAV lesions txwv rau lub raum.

Wu Xiaochuan et al. [6] qhia tias:

(1) Tsis muaj qhov sib txawv hauv etiology ntawm IgAV thiab IgA nephropathy. Ob leeg tuaj yeem tshwm sim los ntawm kev tiv thaiv kab mob hauv lub raum vim muaj kab mob, tiv thaiv kab mob, kab mob sib kis, kab mob caj ces, thiab lwm yam.

(2) Kev faib tawm ntawm IgAV suav nrog xya hom: cais hematuria, cais proteinuria, hematuria thiab proteinuria, mob nephritis, nephrotic syndrome, nephritis sai heev, thiab mob nephritis. Cov kev tshawb fawb soj ntsuam rov qab ntawm IgAV ua los ntawm ntau tus kws tshawb fawb hauv tsev tau pom tias qhov kev faib tawm ntau tshaj plaws yog nephrotic syndrome, tom qab ntawd los ntawm hematuria thiab proteinuria, thiab tsis tshua muaj hom mob nephritis thiab mob nephritis. Kev kho mob ntawm IgA nephropathy zoo ib yam li IgAV, tab sis asymptomatic hematuria lossis proteinuria yog qhov tshwm sim ntau tshaj, ua raws li nephrotic syndrome thiab mob nephritis.

(3) Kev tshawb fawb hauv tsev thiab txawv teb chaws tau pom tias hauv cov neeg mob uas muaj IgA nephropathy thiab IgAV nephritis, kev kuaj kab mob qhia pom glomerular IgA deposition. IgA nephropathy cov tshuaj tiv thaiv kab mob feem ntau yog tso rau hauv mesangium, tab sis hauv cov neeg mob uas muaj IgAV nephritis, IgA deposition nyob rau hauv glomerular capillary loops yog ntau tshaj li nyob rau hauv mesangium, thiab nyob rau hauv ob peb kis, muaj txawm tsis muaj IgA deposition nyob rau hauv lub mesangium. Feem ntau ntawm IgA nephropathy yog IgA nrog IgM thiab / lossis C3 deposition, thiab tsis muaj kev hloov pauv xws li IgG deposition raws li lub ntsiab deposit thiab capillary phab ntsa linear IgG deposition. Hauv cov menyuam yaus uas muaj IgAV nephritis, IgG tuaj yeem tso rau hauv glomerular immune deposits, thiab tej zaum tseem yog IgG lossis muaj cov kab tawm ntawm IgG ntawm phab ntsa capillary [5].

(4) Rau IgA nephropathy, qhov txawv txav glycosylated IgA1 molecules tau tso rau hauv ob lub raum, thiab tsis muaj cov ntaub ntawv qhia txog lawv cov khoom tso rau hauv cov kabmob uas tsis yog lub raum. IgAV tuaj yeem cuam tshuam rau ntawm daim tawv nqaij, plab hnyuv, ob lub raum, thiab cov pob qij txha, thiab qhov txawv txav glycosylated IgA1 molecules tau pom nyob rau hauv cov kabmob xws li ob lub raum, tawv nqaij, thiab plab hnyuv [7]. Cov kws tshawb fawb txawv teb chaws cov kev tshawb fawb txog qib ntawm cov ntshav qab zib tsis txawv txav glycosylated IgA1 molecules tso rau hauv ob kab mob tau pom tias tus naj npawb ntawm cov ntshav IgA1 molecules tso rau hauv IgA nephropathy siab dua li hauv IgAV, thiab hauv qee cov neeg mob IgAV, txawm tias qhov txawv txav glycosylated IgA1 tsis tuaj yeem ua tau. pom [8].

(5) Qhov kev cia siab ntev ntawm IgAV muaj feem cuam tshuam rau qib ntawm lub raum puas. Feem ntau, qhov kev cia siab yog qhov zoo. Qee qhov xwm txheej tshwm sim rau lub raum tsis tu ncua, thiab qhov tshwm sim ntawm qhov kawg ntawm lub raum kab mob kuj tsawg (kwv yees li 2%). Txawm li cas los xij, kev nce qib thiab kev kwv yees ntawm IgA nephropathy tsis zoo. Moriyama et al. [9] rov qab txheeb xyuas qhov kev kwv yees ntawm 1012 cov neeg mob uas muaj IgA nephropathy thiab pom tias kwv yees li 50% ntawm cov neeg mob tau nce mus rau theem kawg ntawm lub raum hauv 30 xyoo tom qab kuaj pom.

IgA nephropathy thiab IgAV muaj cov kab mob sib txawv, tab sis lawv txawv hauv kev kho mob, kab mob, thiab kev tshawb fawb.

Q7 Cov qauv kev kho mob rau IgAV nephritis yog dab tsi?

The 2021 KDIGO guidelines point out that there is currently no IgAV nephritis prevention and treatment program supported by randomized controlled studies in adults. For patients with rapidly progressive glomerulonephritis (RPGN), the treatment plan refers to ANCA-associated vasculitis; for other manifestations, the treatment strategy is mainly based on IgA nephropathy, including lifestyle improvement; for patients with urine protein>0.50 g/d, renin-angiotensin system inhibitors (RASi) are actively added for treatment. If urine protein is still>0.75-1.00 g/d after 3 months of supportive therapy, it is recommended to join clinical studies including different doses and courses of hormone therapy. If urine protein is still>1. Hauv kev tshawb fawb tam sim no, koob tshuaj pib ntawm glucocorticoids sib npaug rau 0.75-1.00 mg / kg lub cev hnyav ntawm prednisone (qhov ntau npaum li cas tsis pub tshaj 75 mg), thiab thawj koob tshuaj ntau tshaj. yog siv tsis pub dhau 2 lub hlis, thiab nws raug txo thiab tso tseg tsis pub dhau 6-8 hli. Cov lus qhia tsis niaj hnub pom zoo kom siv cov tshuaj tiv thaiv kab mob xws li cyclophosphamide (CTX). Peb tau ua raws li cov qauv kev kho mob ntawm IgA nephropathy rau tus neeg mob no, siv RASi thiab hydroxychloroquine ua cov kev kho mob yooj yim, thiab muab cov tshuaj hormones + mycophenolate mofetil kev kho mob ntawm lub hauv paus no.

Q8 Puas tuaj yeem kho tshuaj hormone ntawm extrarenal IgAV tiv thaiv nephritis?

Tsis tau. Tam sim no tsis muaj pov thawj kev kho mob raws li cov tshuaj hormones tuaj yeem tiv thaiv nephritis hauv cov neeg laus IgAV. Txawm li cas los xij, muaj ntau cov pov thawj hauv cov menyuam yaus uas qhov kev siv tshuaj tiv thaiv ntawm glucocorticoids hauv extrarenal IgAV tsis txo qhov tshwm sim ntawm lub raum kev cuam tshuam. Hauv kev sim tshuaj ntsuam xyuas uas suav nrog 352 tus menyuam yaus nrog IgAV, kev siv prednisolone thaum ntxov tsis txo qhov tshwm sim ntawm proteinuria tom qab 12 lub hlis. Qhov kev tshawb pom no tau txheeb xyuas hauv 171 cov menyuam yaus, qhia tias kev siv cov prednisolone thaum ntxov tsis tiv thaiv qhov tshwm sim ntawm nephritis [10]. Ib qho kev tshuaj ntsuam xyuas meta suav nrog 5 RCTs, ntsuas kev tiv thaiv cov nyhuv luv luv ntawm glucocorticoids (2-4 lub lis piam) ntawm nephritis ntawm 6 thiab 12 lub hlis tom qab kuaj mob hauv 789 tus menyuam [11]. Qhov xaus yog tias kev siv glucocorticoids thaum pib ntawm tus kab mob tsis tuaj yeem tiv thaiv qhov pib ntawm nephritis.

Q9 Puas tuaj yeem kho cov tshuaj hormones ntawm IgAV tiv thaiv qhov rov tshwm sim ntawm pob liab liab?

Tsis yog. A 1-xyoo randomized, kev tshuaj xyuas cov placebo hauv Canada hauv 2004 tau qhia tias tsis muaj qhov sib txawv ntawm cov menyuam yaus uas muaj pob liab liab tshwm sim ntawm pawg tiv thaiv prednisone thiab pab pawg placebo piv nrog cov placebo (2/ 21 vs. 4/19, P=0.4) [12]. Tom qab ntawd, ntau qhov chaw, yav tom ntej, randomized, ob-dig muag, placebo-tswj txoj kev tshawb fawb hauv Finland pom tias cov menyuam yaus uas muaj purpura hauv pawg tiv thaiv prednisone qis dua li hauv pawg placebo 7-10 hnub tom qab suav nrog hauv kawm [36% (27/75) vs. 56% (41/73), P=0.021], tab sis tsis muaj qhov sib txawv tseem ceeb hauv cov tsos mob ntawm daim tawv nqaij ntawm 1 lub hlis thiab qhov rov tshwm sim ntawm purpura tom qab 1 lub hli nruab nrab ntawm ob pawg [13].

Q10 Dab tsi yog qhov muaj feem cuam tshuam rau kev kuaj mob ntawm IgAV?

Qhov kev kwv yees luv luv ntawm IgAV feem ntau cuam tshuam nrog qhov hnyav ntawm kev koom tes ntawm lub plab zom mov thiab cov kabmob tseem ceeb; Lub sij hawm luv luv prognosis muaj feem xyuam rau qhov hnyav ntawm kev koom tes ntawm lub plab zom mov, xws li intussusception, plab hnyuv perforation los yog refractory gastrointestinal los ntshav, uas tej zaum yuav muaj kev phom sij rau lub neej. Qhov kev kwv yees ntev ntev yog feem ntau cuam tshuam nrog qhov hnyav ntawm lub raum kev koom tes. Shi et al. [14] ua ib qho kev tshuaj ntsuam meta ntawm 9 qhov kev tshawb fawb txog kev tswj xyuas. Lawv tau pom tias muaj hnub nyoog laus dua ntawm qhov pib, qis glomerular pom tus nqi, thawj zaug tshwm sim ntawm nephrotic syndrome, nephritis, thiab nephrotic syndrome, thiab lub raum biopsy uas qhia txog crescentic nephritis yog ib qho tseem ceeb ntawm kev pheej hmoo rau cov tsos mob tsis zoo. Tsis tas li ntawd, txawm hais tias kev koom tes ntawm cov kabmob xws li lub plawv, lub ntsws, lub hlwb, lub qhov muag, thiab cov qog nqaij hlav tsis tshua muaj, nws kuj tseem muaj feem cuam tshuam rau qhov kev tshwm sim ntawm IgAV.

Cistanche kho mob raum li cas?

Cistancheyog ib hom tshuaj suav tshuaj ntsuab siv rau ntau pua xyoo los kho ntau yam mob, suav nrograumkab mob. Nws yog muab los ntawm qhuav stems ntawmCistanchedeserticola, ib tsob nroj nyob rau hauv cov suab puam ntawm Tuam Tshoj thiab Mongolia. Lub ntsiab active Cheebtsam ntawm cistanche yogphenylethanoidglycosides, echinacoside cov tshuaj, thiabacteoside, uas tau pom tias muaj txiaj ntsig zoo rauraumnoj qab haus huv.

Kab mob raum, tseem hu ua kab mob raum, hais txog ib yam mob uas lub raum ua haujlwm tsis zoo. Qhov no tuaj yeem ua rau muaj cov khoom pov tseg thiab cov co toxins hauv lub cev, ua rau muaj ntau yam tsos mob thiab teeb meem. Cistanche tuaj yeem pab kho mob raum ase los ntawm ntau lub tswv yim.

Ua ntej, cistanche tau pom tias muaj cov nyhuv diuretic, txhais tau tias nws tuaj yeem ua rau cov zis ntau ntxiv thiab pab tshem tawm cov khoom pov tseg ntawm lub cev. Qhov no tuaj yeem pab txo lub nra ntawm lub raum thiab tiv thaiv kev tsim cov co toxins. Los ntawm kev txhawb nqa diuresis, cistanche kuj tseem tuaj yeem pab txo qis ntshav siab, ib qho teeb meem ntawm cov kab mob raum.

Ntxiv mus, cistanche tau pom tias muaj cov teebmeem antioxidant. Kev ntxhov siab oxidative, tshwm sim los ntawm qhov tsis sib xws ntawm kev tsim cov dawb radicals thiab lub cev tiv thaiv antioxidant, ua lub luag haujlwm tseem ceeb hauv kev mob raum. ies pab neutralize dawb radicals thiab txo Oxidative kev nyuaj siab, yog li tiv thaiv lub raum los ntawm kev puas tsuaj. Cov phenylethanoid glycosides pom hauv cistanche tau tshwj xeeb hauv kev tshem tawm cov dawb radicals thiab inhibiting lipid peroxidation.

Tsis tas li ntawd, cistanche tau pom tias muaj cov nyhuv anti-inflammatory. Kev mob yog lwm yam tseem ceeb hauv kev loj hlob thiab kev loj hlob ntawm cov kab mob raum. Cistanche's anti-inflammatory zog pab txo cov zus tau tej cov pro-inflammatory cytokines thiab inhibit qhov ua kom o yuav tsum tau txoj kev, yog li alleviating o nyob rau hauv lub raum.

Tsis tas li ntawd, cistanche tau pom tias muaj cov teebmeem immunomodulatory. Hauv kab mob raum, lub cev tiv thaiv kab mob tuaj yeem ua rau tsis zoo, ua rau muaj kev mob ntau dhau thiab cov ntaub so ntswg puas. Cistanche pab tswj lub cev tiv thaiv kab mob los ntawm kev hloov kho kev tsim khoom thiab kev ua haujlwm ntawm lub cev tiv thaiv kab mob, xws li T hlwb thiab macrophages. Txoj cai tiv thaiv kab mob no pab txo qhov mob thiab tiv thaiv kev puas tsuaj ntxiv rau lub raum.

Ntxiv mus, cistanche tau pom los txhim kho lub raum kev ua haujlwm los ntawm kev txhawb nqa kev tsim kho ntawm lub raum hlab nrog cov hlwb. Lub raum tubular epithelial hlwb ua lub luag haujlwm tseem ceeb hauv kev pom thiab rov nqus cov khoom pov tseg thiab electrolytes. Hauv kab mob raum, cov hlwb no tuaj yeem raug puas tsuaj, ua rau lub raum ua haujlwm puas. Cistanche lub peev xwm los txhawb kev tsim kho ntawm cov hlwb no pab kho lub raum kom zoo thiab txhim kho lub raum tag nrho.

Ntxiv nrog rau cov kev cuam tshuam ncaj qha rau lub raum, cistanche tau pom tias muaj txiaj ntsig zoo rau lwm yam kabmob thiab lub cev hauv lub cev. Txoj hauv kev zoo rau kev noj qab haus huv no tseem ceeb tshwj xeeb hauv cov kab mob raum, vim tias tus mob feem ntau cuam tshuam rau ntau lub cev thiab lub cev. che tau pom tias muaj kev tiv thaiv rau lub siab, lub plawv, thiab cov hlab ntsha, uas feem ntau cuam tshuam los ntawm kab mob raum. Los ntawm kev txhawb nqa kev noj qab haus huv ntawm cov kabmob no, cistanche pab txhim kho lub raum tag nrho thiab tiv thaiv kev mob ntxiv.

Hauv kev xaus, cistanche yog cov tshuaj suav tshuaj ntsuab siv rau ntau pua xyoo los kho mob raum. Nws cov active Cheebtsam muaj diuretic, antioxidant, anti-inflammatory, immunomodulatory, thiab regenerative teebmeem, uas pab txhim kho lub raum ua haujlwm thiab tiv thaiv ob lub raum los ntawm kev puas tsuaj ntxiv. , cistanche muaj txiaj ntsig zoo rau lwm lub cev thiab lub cev, ua rau nws txoj hauv kev zoo rau kev kho mob raum.