Kansas Journal of Medicine 2013

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1 : A Rre Entity in the Post Dilysis Er Hroon Khlid, M.D. 1, Christopher Fox, M.D. 2, Isc Opole, M.D., Ph.D., F.A.C.P. 1 University of Knss Medicl Center, Knss City, KS 1 Deprtment of Internl Medicine 2 Deprtment of Pthology nd Lortory Medicine Introduction Uremic pneumonitis, known s uremic lung, is compliction of end stge renl disese. It rrely is seen these dys in developed countries due to ccess to hemodilysis in ptients with dvnced renl filure. Uremic pneumonitis is clinicl entity tht ws descried s erly s Its pthophysiology is sed on uremi-induced incresed permeility of pulmonry lveolo-cpillry interfces, leding to interstitil nd intr-lveolr edem, telectsis, lveolr hemorrhge, nd pulmonry hyline memrne formtion. 2 These chnges re compounded y leeding dithesis secondry to pltelet dysfunction in dvnced renl disese. 3 The pulmonry symptoms nd rdiogrphic findings re reversile with hemodilysis. We descrie the clinicl presenttion nd mngement of ptient without prior history of kidney disese presenting with uremic pneumonitis. Cse Report A 23-yer-old Hispnic mle with n uneventful pst medicl history presented with complints of coughing up lood nd epistxis. Symptoms strted two weeks prior with chest pin which worsened with rething, lying on his ck, nd coughing. It progressed to productive cough with hemoptysis of pproximtely one spoonful of right red lood dily fter out week. He lso reported infrequent self-limiting episodes of epistxis. The ptient felt feverish. He hd chills nd swets for one week. Other symptoms included shortness of reth on exertion, loss of ppetite, ftigue, nuse, nd metllic tste in his mouth. He denied rsh or rthrlgis nd reported norml urinry output. He hd moved to the United Sttes from Mexico out four yers prior nd served s delivery mn for grocery stores for one nd hlf yers, then s construction worker for one yer, nd most recently hd een mowing lwns for out yer. He previously smoked cigrettes nd drnk out six cns of eer dily until pproximtely two yers prior to presenttion. He denied use of illicit drugs. Fmily history ws significnt for his mother hving hypertension. On physicl exmintion, the ptient ws ferile. He hd tchycrdi, respirtory rte of 24 pm, n oxygen sturtion of 97% on four liters y nsl cnnul, nd lood pressure of 154/90 mmhg. He hd mrked pllor, ws in mild respirtory distress with hyperdynmic precordium, nd corse rles ilterlly on posterior chest usculttion. There ws no jugulr venous distension, pericrdil friction ru, stupor, sterixis, or peripherl edem. At presenttion, his lood ure/nitrogen ws mrkedly elevted t 218 mg/dl with serum cretinine of 41.5 mg/dl. His hemtocrit ws low with hemogloin of 4.6 g/dl, nd urinlysis showed 4+ proteins, 1+ 71

2 lood, 2-10 white lood cells, 2-10 red lood cells, nd urine protein/cretinine rtio of There were no csts noted. An utoimmune work up ws inconclusive. Blood counts nd serum chemistry re listed in Tles 1 nd 2. Tle 1. Serum chemistry t presenttion nd dys 5 nd 10 fter initition of dilysis. Vrile Reference Rnge On Evlution Dy 5 * Dy 10 * Sodium mmol/l Potssium mmol/l Chloride mmol/l CO mmol/l Blood Ure Nitrogen 8-20 mg/dl Cretinine mg/dl Anion Gp egfr > 60 ml/min/1.73 m Mgnesium mg/dl Phosphorus mg/dl * After ptient underwent hemodilysis. Tle 2. Bsic hemtologicl profile t presenttion. Vrile Reference Rnge On Evlution Hemtocrit 40-50% 13.4 Hemogloin g/dl 4.6 MCV FL 83 WBC K/UL 14.9 Pltelet K/UL 206 PTT secs 31.9 INR An initil chest X-ry showed symmetric ilterl ir spce opcities primrily involving the lower lung zones (Figure 1). Non-contrst computed tomogrphy (CT) of the chest showed diffuse nd somewht symmetric irspce opcities throughout the lungs ilterlly with predominnce in the lower loes nd reltive spring of the upper loes nd long the peripherl mrgins of the lungs (Figure 1). Upon dmission, this ptient received pcked red lood cell trnsfusions nd hemodilysis ws initited. The initil working dignoses were pulmonry renl syndrome nd lupus, ut comprehensive utoimmune work up ws inconclusive. Renl ultrsound showed trophic ilterl kidneys. An echocrdiogrm ws norml. Blood, sputum, nd urine cultures remined negtive for ny growth. A renl iopsy showed chnges suggestive of end stge renl disese nd primrily n immunologiclly medited glomerulonephritis producing mesngio-prolifertive nd foclly crescentic type of injury (Figure 2). The ptient s pulmonry symptoms nd epistxis resolved with repeted dilysis, s did rdiogrphic evidence of lveolr infiltrtes (Figure 3). This ws more consistent with uremic pneumonitis rther thn the pulmonry renl syndrome. 72

3 Figure 1. Initil chest x-ry () nd non-contrst chest CT () imges t presenttion. There ws extensive diffuse symmetric ilterl ir spce opcities primrily involving the lower lung zones chrcteristic of diffuse pulmonry hemorrhge. Figure 2. ) Renl iopsy showed single glomerulus exhiiting firoepithelil crescent (periodic cid-schiff-methenmine silver stin, 400x). ) Immunofluorescence stin demonstrted irregulr deposits of C1q long the sement memrne of cpillry loops nd within mesngil res (FITC nti-c1q, 400x). Figure 3. Follow-up chest x-ry () nd non-contrst chest CT imges () showed ilterl ir spce opcities lrgely resolved following hemodilysis. 73

4 Discussion Uremic pneumonitis, common in the pre-dilysis er with frequent references in medicl literture into the 1940s-1960s, is now rre in the developed world with improved recognition nd cre of renl disese nd ccess to dilysis. Ptients typiclly presented with dyspne nd chrcteristic nd sometimes reversile rdiologicl utterfly densities or tswing shdows. 4,5 This syndrome uniting pulmonry rdiologicl fetures nd uremi previously hd een referred to y vrious nmes, including pulmonry zotemi, 6 pulmonry hyperemi with cidosis, 7 uremic edem, 8 or fluid lung. 9 It hs een descried in ssocition with uremi secondry to severe glomerulonephritis nd hemolytic-uremic syndrome, lthough it is elieved to occur secondry to severe uremi from ny etiology. Hughes 5 reported on series of seven cses with chrcteristic rdiogrphic chnges severl of which mirror the findings in our ptient, including the resolution of opcities following dilysis, coupled with hemorrhgic chnges in the lung, nd focl hemorrhges nd lveolr luminous nd firinous edem. Our ptient neither hd prior dignosis of renl dysfunction nor long-stnding uremic symptoms. However, imging studies nd iopsy results indicted chronic References 1 Hopps HC, Wissler RW. Uremic pneumonitis. Am J Pthol 1955; 31(2): PMID: Bleyl U, Snder E, Schindler T. The pthology nd iology of uremic pneumonitis. Intensive Cre Med 1981; 7(4): PMID: Kw D, Mlhotr D. Pltelet dysfunction nd end-stge renl disese. Semin Dil 2006; 19(4): PMID: kidney dmge with evidence of glomerulonephritis nd glomerulosclerosis, suggesting prior untreted or unrecognized renl disese. In cse series of six ptients presenting with pulmonry-renl syndrome without concomitnt destructive pulmonry disese, Hermn et l. 10 reported two ptients with evidence of nti-glomerulr sement memrne disese consistent with the Goodpsture Syndrome. Two hd idiopthic rpidly progressive glomerulonephritis. One hd immune complex deposition consistent with Systemic Lupus Erythemtosus nd one hd vsculitic nd immunologic chnges consistent with Wegener s grnulomtosis. Our ptient hd mesngio-prolifertive nd focl crescentic glomerulonephritis which likely produced end-stge kidney disese with severe uremi leding to pneumonitis. Severe complictions from uremi re more likely to e encountered in ptients with limited ccess to helthcre. Uremic pneumonitis cn e proten in presenttion nd could e confused for other disese entities such s cute pulmonry edem, pneumoni, utoimmune, fungl or metsttic disese. 11 It is importnt for clinicins to consider this rre condition s prt of their differentil dignosis for utterfly lung or ts-wing shdows in the setting of hemoptysis nd renl filure. 4 Hodson CJ. Pulmonry edem nd the "tswing" shdow. J Fc Rdiol (London) 1950; 1: Hughes RT. The pthology of utterfly densities in uremi. Thorx 1967; 22(2): PMID: Rendich RA, Levy AH, Conve AM. Pulmonry mnifesttions of zotemi. Am J Roentgenol 1941; 46:

5 7 Drinker CK. Pulmonry Edem nd Inflmmtion. Cmridge, MA: Hvrd University Press, Donich L. Uremic edem of the lungs. Am J Roentgenol Rdium Ther 1947; 58(5): PMID: Alwll N, Lunderquist A, Olsson O. Studies on electrolyte-fluid retention. I. Uremic lung, fluid lung? On pthogenesis nd therpy; preliminry report. Act Med Scnd 1953; 146(3): PMID: Hermn PG, Blikin JP, Seltzer SE, Ehrie M. The pulmonry-renl syndrome. AJR Am J Roentgenol 1978; 130(6): PMID: Kohen JA, Opshl JA, Kjellstrnd CM. Deceptive ptterns of uremic pulmonry edem. Am J Kidney Dis 1986; 7(6): PMID: Keywords: uremi, lung, pneumonitis, kidney diseses 75

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