Robbins Essential Pathology PDF - Kidney Chapter

CHAPTER 11 Kidney 194.e1 A B...

CHAPTER 11 Kidney 194.e1 A B Supplemental eFig. 11.2 IgA nephropathy. (A) Light microscopy showing mesangial proliferation and matrix increase. (B) Characteristic deposition of IgA, principally in mesangial regions, detected by immunofluorescence.  CHAPTER 11 Kidney 195 A B D C Fig. 11.8 Lupus nephritis. (A) Diffuse proliferative glomerulonephritis. Note the marked increase in cellularity throughout the glomerulus (H&E stain). (B) Membranous pattern of nephritis showing a glomerulus with sev- eral “wire loop” lesions representing extensive deposits of immune complexes (periodic acid–Schiff stain). (C) Deposition of IgG antibody in a granular pattern, detected by immunofluorescence. (D) Electron micrograph showing subendothelial deposits (arrowheads) in the GBM. The arrow indicates the basement membrane. (A and B, Courtesy Dr. Helmut Rennke, Department of Pathology, Brigham and Women’s Hospital, Boston. C, Courtesy Dr. Jean Olson, Department of Pathology, University of California San Francisco. D, Courtesy Dr. Edwin Eigenbrodt, Department of Pathology, University of Texas, Southwestern Medical School, Dallas.) suscepbe an maes. Normay, anmcroba properes o e bad- DISEASES OF TUBULES AND INTERSTITIUM der wa and perodc empyng o e badder keep e urne sere. he mos requen causes o dsorders o ese srucures are nlamma- Oulow obsrucon, suc as a caused by an enarged prosae or ory and resu n a group o condons coecvey caed tubuonterstta uerne proapse, causes sass o urne and predsposon o ower ur- neprts; and oxc or scemc damage o e ubuar epeum, caed nar y rac necon and ereore pyeoneprs. Vescoureera relux, acute tubuar njury. Wen e nlammaon s e resu o an necon,  somemes due o a congena deec n e ureerovescuar vave, s usuay nvoves e rena pevs (par o e coecng sysem n e urne anoer predsposng acor because conamnaed urne rom e bad- oulow rac) and s ereore caed pyeoneprts;  may be acue or der can eak back no e ureers. Insrumenaon o e urnar y rac cronc. Cystc dseases o e kdney are generay consdered separaey can aso aow upward movemen o bacera. Muc ess commony, rom dseases o e ubues and nersum, bu we w dscuss em a acue pyeoneprs resus rom emaogenous spread rom dsan e end o s secon because e cyss arse rom ubuar epeum. necon, parcuary n ndvduas w some mmune abnormay, as n e seng o mupe myeoma (see Caper 9). Acute Pyelonephritis This inammation of the renal pelvis and kidney is caused by bac Morphology. One or bo kdneys sow parencyma abscesses a teria that usually ascend from the lower urinary tract. may coaesce o orm arge areas o queacon and puruen nlam- maon (Fg. 11.10). Coecons o neurops  e ubues and can Pathogeness. he causave organsms are predomnany gram-neg- exend o nvove e nersa space. Gomeru are ypcay spared. ave bac, mos oten Escerca co, wc spread rom e gas- Dabec paens w pyeoneprs are prone o deveop necross o ronesna rac. he organsms rs nec e nearby urera e ps o papae (papary necross), key because o e underyng uroeum and en ascend roug e badder and e ureer o e mcrovascuar dsease and resung scema (Suppemena eFg. 11.3). kdney. Because o e sorer eng o e urera, emaes are more 196 CHAPTER 11 Kidney A B C Fig. 11.9 Rapidly progressive (crescentic) glomerulonephritis. (A) Note the collapsed glomerular tufts and the cres- cent-shaped mass of proliferating parietal epithelial cells and leukocytes inside the Bowman capsule (arrows) (PAS stain). (B) Immunofluorescence showing linear deposit of anti-GBM antibody characteristic of Goodpasture syn- drome, one form of RPGN. (C) Electron micrograph showing characteristic wrinkling of GBM with focal disruptions (arrows). (A, Courtesy Dr. M. A. Venkatachalam, University of Texas Health Sciences Center, San Antonio, Texas.) C ln cal Feature s. Te ds e as e  ypc a  y pres e n s w   su dde n ons e  Morphology. One or bo kdneys sow uneven scarrng nvovng e o p an n  e cosover ebr a  ang e ove ry  ng one o  e k  d ne y s and pevs and cayces, wc may be markedy deormed (Fg. 11.11). In sysemc sg ns o n e c   on. I s usu a  y un   ae ra  (un  ke g omer u- conras, n cronc dseases o e gomeruus or bood vesses, bo  ar ds ord ers). Te ur  ne con a ns neu rop s , s ome  mes a ace d kdneys are dfusey and amos equay afeced. In cronc pyeone- o proenace ous maer  a  ( or m ng w  e ce  c ass ) ; b a c er  a c an be prs, e scars may exend roug e corex o e kdney surace. c u ure d  rom ur ne s amp es. An b o  c  re a men  ypc a  y e ads o he soogc pcure s ypca o cronc nlammaon, w ners- res ou on, bu  may re c ur n p a  en s w   pre d sp os  ng ac ors , a bross and nraes o mononucear ces (ympocyes, pasma and  e prog noss s p o orer n ndv  du a s w o d e veop p ap   ar y ces, and macropages). Tubues may be daed or srunken, and oten ne cross. conan cass o proen rom wc e lud as been resorbed (caed cood cass, reerrng o e omogeneous proen-rc maera, sm- ar o e cood seen n e yrod gand). he scarrng may ead o Chronic Pyelonephritis narrowng or oberaon o bood vesses, dmnsed rena peruson, Chronic infection and the resultant scarring of the kidney may acvaon o e renn–angoensn sysem, and sysemc yperenson. develop in patients with urinary reux or obstruction. Pathogeness. As dscussed prevousy, relux o urne rom e bad- der o e ureers due o congena vescoureera relux or acqured Clncal Features. Cronc pyeoneprs s an mporan cause o cronc obsrucon o e low o urne (caused, or nsance, by rena cacu) kdney dsease. Paens come o medca aenon because o e gradua can ead o repeaed bacera necon. he resu s cronc nlam- onse o rena aure (azoema) or yperenson, or because kdney ds- maon, ssue oss, and bross. Oten, e acue pase s sube and ease s ound upon aboraory esng, eer roune or or oer suspeced may no even be noced by afeced ndvduas, wo presen w e condons. Imagng sudes are usuay dagnosc; by e me sympoms nsdous deveopmen o cronc rena aure. appear, bacera are rarey deeced n e urne.  CHAPTER 11 Kidney 196.e1 A B Supplemental eFig. 11.3 Papillary necrosis. (A) Areas of hemorrhagic necrosis involve the papillae (arrows). (B) The tissue is necrotic and seen as fragmented debris. (Courtesy Dr. A. Renshaw, Baptist Hospital, Miami.)  CHAPTER 11 Kidney 197 eevaon o e serum creanne occurs n abou 50% o cases. Cnca Drug-Induced Tubulointerstitial Nephritis recognon o drug-nduced kdney njur y s mperave, because w- Renal injury can be caused by diverse therapeutic agents, most drawa o e ofendng drug s oowed by recover y, aoug  may commonly by immune mechanisms. ake severa mons or rena uncon o reurn o norma. Pathogenes s. he ong s o drugs nked o kdney njur y ncudes anbocs, durecs, and nonseroda annlammaor y drugs, Acute Tubular Injury among oers. Drugs may bnd o and mod y se proens, creang Severe injury to tubular epithelial cells is caused by ischemia or “neoangens” a ec an IgE response (ype I ypersensvy) or a exposure to toxins and results in an acute decline in renal function. T-ce response (ype IV ypersensvy) (see Caper 4). he rena Cncans oten use e erm acute tubuar necross (ATN), bu paoog y s no dose dependen,  occurs ater a ag oowng drug rank necross s rare; ereore, acue ubuar njur y (ATI) s preerred exposure, and  recurs upon exposure o e same drug or cemcay he resuan cnca syndrome s aso caed acute kdney njury (AKI), reaed drugs; a o ese eaures are conssen w an underyng prevousy reerred o as acue rena aure. mmune mecansm. Pathogeness. Iscemc ATI resus rom reduced bood low, usuay due o ypoensve sock caused by bood oss, sepss, or e sysemc Morphology. he nersum s edemaous and conans an n- nlammaor y response syndrome (SIRS), wc s naed by severe rae o mononucear ces (ympocyes and macropages), some- ssue njures (see Caper 3). Acue emoyss, as n a ransuson mes w abundan eosnops and neurops (Fg. 11.12). reacon, can aso ead o ATI. Dverse oxns, suc as eavy meas Drugs a evoke a T-ce response may ec e ormaon o non- (e.g., mercur y) and sovens (e.g., carbon eracorde), drugs suc caseang granuomas. as genamcn, and radograpc conras agens can cause a nepro- oxc orm o ATI. Because o er g meaboc demand, ubuar Clncal Features. Ras, ever, and eosnopa, usuay wn wo epea ces are parcuary vunerabe o scema and oxns (Sup- weeks ater drug exposure, are eary sysemc manesaons o a pemena eFg. 11.4). In addon, because ubues absorb waer, e drug reacon. Rena manesaons ncude emaura and, n some concenraon o oxns ncreases n e umen. Necroc epea ces cases, eosnops n e urne. Acue kdney njur y w ogura and may be sed no e umen, causng oulow obsrucon. Bo o ese A B Fig. 11.10 Acute pyelonephritis. (A) Cortical surface shows pale areas of inflammation and abscess forma- tion. (B) Neutrophilic infiltrates in the tubules and interstitium. A B Fig. 11.11 Chronic pyelonephritis. (A) Shrunken kidney with irregular, coarse scars. (B) Tubular atrophy and interstitial fibrosis with foci of chronic inflammation. (Courtesy ExpertPath, copyright Elsevier.) 198 CHAPTER 11 Kidney aeraons conrbue o a decrease n e GFR, wc exacerbaes e o afeced ndvduas, cyss deveop n ony some ubues, mos key ubuar scema, seng up an nexorabe cyce o njur y. owng o a sporadc somac muaon n e second norma PKD aee. Morphology. Tubuar epea ces sow e amarks o ce Morphology. he kdneys are progressvey repaced by lud-ed njur y (see Caper 1), ncudng e oss o brus borders, vacu- cyss and may aan enormous szes (Fg. 11.14). he ner venng ozaon, deacmen, coaguave necross, and sougng (Fg. rena parencyma undergoes scemc aropy because o e pres- 11.13). hese are oten accompaned by rupures n e ubuar sure o e expandng cyss. Evdence o supermposed yperen- basemen membrane (ubuorrexs). he nersum s edema- son or necon s common. Asympomac ver cyss are presen ous. Durng recover y, e epea ces may sow sgns o regen- n abou a rd o paens, and cerebra aneur ysms n e crce o eraon, ncudng a ow cuboda morpoog y and moses. Ws are ound n 10% o 30%. Clncal Features. ATI presens w e abrup onse o ogura and Clncal Features. Cnca manesaons reaed o kdney dsease yp- azoema, wc may rapdy progress o urema. Recover y s possbe cay appear around e our decade o e. hese ncude oca pan, w reamen o e underyng condon and approprae supporve emaura, yperenson, and a eavy, draggng sensaon n e abdo- care (dayss, careu managemen o luds and eecroyes). men. Inermen gross emaura commony occurs. he mos mpor- an compcaons, because o er deeerous efec on aready margna Cystic Diseases rena uncon, are yperenson, due o acvaon o e renn-angoen- Renal cystic diseases range from focal incidental ndings of no sn sysem n e seng o dmnsed bood low, and urnary necon. clinical signicance to bilateral lesions that destroy both kidneys. Progresson s sow ; umaey, end-sage rena dsease occurs, bu e Smpe cysts are e mos common orm o cysc dsease and are me course s gy varabe. Paens are aso a g rsk o subarac- deeced a posmorem examnaon or durng radoogc sudes; ey nod emorrage because o e assocaon w cerebra aneurysms. ave no cnca sgncance. In conras, autosoma domnant poycys- tc kdney dsease, wc accouns or amos 10% o cronc kdney Autosomal Recessive Polycystic Kidney Disease dseases, causes subsana morbdy. he rare auosoma recessve poycysc dsease occurs n cdood. I s caused by muaons n e PKHD1 gene, wc encodes a pua- Autosomal Dominant Polycystic Kidney Disease ve membrane recepor proen caed brocysn. Lke poycysn, As e name mpes, s dsease s nered n an auosoma dom- brocysn s ound n ca n ubuar epea ces, bu s unc- nan ason; owever, n as many as 25% o paens ere s no amy on remans unknown. Grossy, numerous sma cyss n e corex sor y, eer because oer afeced members o e amy ave ded and medua gve e kdney a sponge-ke appearance. he dsease s or ave a md orm o e dsease a s asympomac, or because e nvaraby baera. In amos a cases, mupe epeum-ned ver dsease s caused by a new muaon. cyss and a proeraon o pora be ducs aso are presen. Pathogeness. he muaed gene s PKD1 n 85% o 95% o paens and Other Cystic Kidney Diseases PKD2 n e remander. he encoded proens, poycysn-1 and poy- cysn-2, respecvey, eerodmerze and co-ocaze n ubuar epe- Numerous oer ama and sporadc cysc dseases are known. a ces o nonmoe prmary ca, wc sense lud low and reguae Fama juvene nepronoptss, wc s caracerzed by a varabe numerous ceuar uncons. Abnormaes o poycysn-1 or poy- number o meduar y cyss, s an auosoma recessve dsease a s e cysn-2 are beeved o ead o deecve cary uncon and ncreased mos common genec cause o end-sage rena dsease n cdren and secreon o lud rom epea ces, evenuay resung n cys or- young adus. As w oer ypes o poycysc dsease, e majory o maon. hus, s dsease s an exampe o a copaty. Aoug ger- e genes mpcaed n nepronopss encode componens o ep- mne muaons o e PKD genes are presen n a rena ubuar ces ea ca. Grossy, e kdneys are sma, ave conraced granuar A B Fig. 11.12 Drug-induced tubulointerstitial nephritis. (A) Chronic inflammatory infiltrate in the interstitium with tubular injury. (B) Prominent eosinophilic infiltrate. (Courtesy ExpertPath, copyright Elsevier.)