Pericardial Effusion, a Manifestation of Systemic Lupus Erythematosus STANLEY S. BERGEN, JR. Circulation. 1960;22:144-150 doi: 10.1161/01.CIR.22.1.144 Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright © 1960 American Heart Association, Inc. All rights reserved. Print ISSN: 0009-7322. Online ISSN: 1524-4539 The online version of this article, along with updated information and services, is located on the World Wide Web at: http://circ.ahajournals.org/content/22/1/144 Permissions: Requests for permissions to reproduce figures, tables, or portions of articles originally published in Circulation can be obtained via RightsLink, a service of the Copyright Clearance Center, not the Editorial Office. Once the online version of the published article for which permission is being requested is located, click Request Permissions in the middle column of the Web page under Services. Further information about this process is available in the Permissions and Rights Question and Answer document. Reprints: Information about reprints can be found online at: http://www.lww.com/reprints Subscriptions: Information about subscribing to Circulation is online at: http://circ.ahajournals.org//subscriptions/ Downloaded from http://circ.ahajournals.org/ by guest on September 9, 2014 Pericardial Effusion, a Manifestation of Systemic Lupus Erythematosus By STANLEY S. BERGEN, JR., M.D. PERICARDIAL effusion can be a striking physical and radiologic finding, but its occurrence as a manifestation of disseminated lupus erythematosus has not been emphasized. During the last 2 years 4 patients with systemic lupus erythematosus who had an associated pericardial effusion were seen at this hospital. Two of this group were misdiagnosed and treated incorreetly, partly because of failure to consider lupus erythematosus as a cause of pericardial effusion. On physical examination he appeared well nourished and in no distress. The blood pressure was 120/80, there was no clubbing, the heart was not enlarged, the lungs were clear, and the abdomen contained no masses or enlarged organs. There was generalized lymph node enlargement and swelling of all the proximal interphalangeal joints of the hands. Laboratory data included a normnal urinalysis, hemoglobin of 10.1 Gm. per cent, white blood cell count of 9,900 per mnm.3 with a normal distribution, negative serologic test for syphilis, thymol turbidity of 9.2 units, serum albumin of 3.4 and serum globulin of 6.4 Gm. per cent, the electrophoretic pattern showing the gamima globulin to be over 50 per cent. Latex fixation test was normal; all cultures (sputunm, gastric, and urine) for tuberculosis were negative, and the bone marrow was within normal limits with negative LE preparation. A sickle-cell preparation was negative and an electrocardiogram was within normal limits. Two of LE preparations done on venous blood were positive, as was the LE precipitin test. Chest x-ray disclosed a heart of normal size, calcific left hilar lymph nodes, and linear strand densities at the right base and left mid-lung. Skin tests for Case Reports Case 1 A 39-year-old Negro cook was admitted to St. Luke's Hospital with swelling of hands and feet and left-sided chest pain for 3 months. He stated that he had been hospitalized for 6 weeks while in mnilitary service with joint pains and "glandular enlargement." He had also been hospitalized elsewhere 18 months previously following 3 months of left anterior chest pain, fever to 104 F., night sweats, joint pains, and weight loss of 15 pounds. The hospital record noted generalized lymphadenopathy, a hemoglobin of 11.2 Gin. per cent, electrocardiographic "evidence of pericarditis," and an enlarged heart compatible with pericardial effusion, and bilateral pleural thickening. Despite negative cultures of sputum and gastric aspirate, he was treated with Isoniazid and streptomycin with apparent improvement. He left the hospital against advice before therapy was completed. He then felt well, ;but a year before admission to St. Luke's hospital he noted intermittent swelling and pain in the proximal interphalangeal joints. Four months before hospitalization he had a 6day episode of fever, and a chest x-ray disclosed peribronchial infiltration, both of which cleared without specific therapy. Three months before admission an epitrochlear lymph node biopsy revealed "chronic inflammation." Thereafter he experienced dyspnea on effort, paroxysmal nocturnal dyspnea, ankle and hand swelling, joint pain, and "feverish- blastomyeosis, coccidioidonmycosis histoplasmosis, and lymphogranuloma venereum were negative. The Mantoux test was positive at 1 :1,000 dilution. Cardiopulmonary study revealed a defect in aeration with arterial unsaturation on exercise, reduced vital capacity and minute hyperventilation at rest, and low bicarbonate serum level. These findings were believed compatible with pulmonary fibrosis and an early diffusion block. Lymph node, skin, and mnuscle biopsies did not reveal any specific lesions, although perivascular clusters of lymphocytes and plasma cells were noted. Evidence of tuberculosis or Boeck's sareoid was not seen. Following spontaneous clearing of his chest pain, the patient was given chloroquine, 250 mg. 3 times a day, with marked improvement in his joint pain and swelling. The patient has been seen in the clinic for the last 18 months with intermittent exacerbations of joint pain. LE preparations have been repeatedly positive and he has required hospital admission on 2 occasions for prednisone therapy for relief of joint pain and febrile exacerbations. He is at present doing well on chloroquine and is believed ness." From the Department of Medicine, St. Luke's Hios- pital, New York, N.Y. 144 Circulation, Volume XXII, Downloaded from http://circ.ahajournals.org/ by guest on September 9, 2014 July 1960 t1\I1 )IA -PEIIICAI 1~'SI1 N 15 .145- Figure 1 flfjioc rd(iY) tffulaion. IM Oil C o 2. .i volou/ pcrito itdlot to hive dissemiiinalted liupus vthetaitosus witl as p)ilioofina xv fibrosis of mi deterni-ineed etier sociated ology. Case 2 A 2P-vear)-o1(l Negro mierchant seamtam,-in xvlas transferred to St. Luike's hospital forI surgery. He had s been ho ized elsewhxre 39 m-tion-itlhs previously ()pital following 6 -xveek hI;story of knee, ank1<le, at-nd hand paint.I)uriang this period the patient hiad also noted uriaarx co-ugllh produLcetive of simall frequenmey atnd Camimoun0sts of yelloxv nmucoid spuituimn. Five yea rs earlier thle p}(latient had beeni hospitallized for fex ec thiat disaIppeavred spontaineously. On physical examiiination the blood presssure Awas 1,50/90 11910m. lIg, a11d the )atitient ha-id mnla:rkedlya I)uffy- facwe \xith edemna of. thie eyelids. There was a1 1nicla1hr eruption over hlis forehead, face, and no,se th.at ha,td ai, "buitter-fly-" distribution. The heart soullids were quiet, thle healrt seemeid enliared, a-lnd were rio murmurs heard. Bilateral basilar ales were hiea-rd, and there was edema, of ank-les and wrists. Labortaitory datia ine-luded ai negaltixve serologic test for sy plhilis, tl mviol turbidity of IS units, hemnoglobin of 'I .9 (Gn. per cent, whiite blood cell counit of 4,050 per -mnn.3 with. notn-al differenftial count, serumti. allbumin of 3.2 and seruml. globulin of 5.1. Giii. prer cent. Ani eleetrocardiogra-m showed a low voltag-e aind a. chest x-rffty sblowed. a large cardiac silhouette probably duie to p)ericardial effuision, witlh t iriimalblateral pleural effusions. LB preparations wecre repeatedly positive. A diagnosis wa,is ii,i.de of systemiic lupiis ery theina tosus tmdthe adret ml ster oid tlerapy- was started. Veionius pressure at thla.t tim)e was 176 mmni. of saline nud the a ri -to-tongue circula,tion tilmie Figure 2 n te)1 o I t/ (') ), I ?'ff P,osit i ont t io) r tOt' 10 of the c /t St If rmonths ofiti pt ti(lt(f/iott usi inton c . Dveeholin) Nvas -.14 seconds. 2Svpi)toms and p)Ivsilal findings iinprovd duritig thle sUbsequent monthli the steroid dosag-e wasl., reduced, an.d ehiloroquine 250 mgn-. tw-iice dalilyv was added. Nexertheless the cardiacie shado-w increased in 1ag"ing Venrlous pressure levels; size, despite unchat l >V tierefore, r icardiocenit tesis a mdl pericarrabioPS w\erce reconnmended anid the patient wx as transferred to St. Luke's hospital xvhere' 1E prepairaItions of the b)l1oo were agaiin 1o)si-tive, aind an eleetroca(Irdiega reveailed inverted T1 wSxix(vs in leads i, IT,¢aYVI, and V4 6 A. Angio.ardiogra 111 confirmed the preselee of a p)e'ieardi11 effusioll (0f. 1). Ani ol)pen icaihocente^sis and pertielalrdial biopsv were performed ad30 ImL of straw colored fluid were aspirated tnd (lotted quicklv. All cuTltines a-nd LE-eell p)repara.ltions on this fluid were nieg atix e. The biops;y showed dense fibr-ous tissue and ai few scattered lymphocytes. The cliest woi-iiid dra-iinedl pericardial fluid for about 10 dayvv,s and theji elosed. The eardiae size retairned towaIrd iormnall after a few dayvs. The venouis pressure fell to 70 mm. of wtater, whereas the arm-totonlguIe eircul9ation tilme rematined at 14 seconds. Follo-win surgerv, the dosag-e of prednisone wasi graduallyv reduced, aind mi-a:iinten,maee therapPy Awithi chioroquitie xvas reinstituted. Now, 20 nioiitlis rlaterl the carm-diac exailmination chest x-ray (fig. 2), a rid lc trocarXd i og)ram aXre normal. Thei patietnt is working as a mernerchant sea rman and lie is asymptomatie except for mild joint pain tha,t is responsive to a spirin. Case 3 A- 37--vear-old Ne-gro wom.la. watris first addmitte(l to St. Luke's hospital 31/2 eairs ag-o wvithi muiiltiple ioint painls. A-t that tinie she had (liscrete a:reas Circulation, Volunme XXII, July 1600 Downloaded from http://circ.ahajournals.org/ by guest on September 9, 2014 N 146 VR(IEIUEN 4 Figure 3 (fase 3. lRo i/gy a)oqramr of the 7hes t, shlowrilj }aobnd able phleurr l perik rrbifl cff nion)Q.s. of hIyporpig-neatatiorl oxlo tlle tyIrunk alnd extromities, gy?nerirlized lx rrlradeririinrtlr.x arild %spleeomegaIlv. Pertinent laibora"Itory da.1tai rexel,,ed dopressed blood platelet onnits-, rlxcrsal of the niorrualT album1-iiin 'lobuvlinratio, positrive MTazzini and KNahn tests, a-ga1tive VDR-rL, .anld positive LE-cell pr-epatrationi,s;. Oni aspirlin a,ind predriisone all Symptom-s eletared and the blood Plteltet counts re- turned to norm"aL. Approximaitely 6 months; laiter sh-ie wras re-admitted to the h10spitall bec.aIuse of s':'evere joint pain. aqnemiaqn aind thromboeNtoaperria. She improved on pr-ednisone tand, as the dosagi-e wa:s reduced cliloroquiine was bepinr wvithl illailrtenlaIree of imiprovemrerit. Dur ing this exacerbation a left pleural effusion nid a splenic infartet oecurred, both of -wlhieh improved by timne of diselha rge. A thirid admirssion 1 year Iater was prompted Jbv ataxia, and eon fusion thait el,-ared -when ehloroqutine wais stopped. Three wxeeslaster she suffered sevexoe rilit upper abdominal paiin. The temperatin1e was 1O0.S F., the heart seemed enulared, a prade IT apieal sy stolic iiiilrnnr'mr ivras heard,-and a tender liver edge was felt below the ri-Igt Costal imargin. LaJ;bor'a-1to-i'v- studies; showed 4+4 albumrirnuria, hm(lo')'O-bin of 9.6 C0i a. per cent, wbite blood cell eounit of 7, 00 per} 111r113., seri.,um albuinin of 2.7 and serumur globulin of 4.2 Cn. per eemnt. An electrocardiogram reve; led low v oltage anid inverted T in le.aids IT II, aVe, and V1 6' A chest x-ray wrascorISistelrt with peticar;dia.-,dl effusion and a sma111l righlt pleural<i1 effusion (fig. 3). Transiently on the thir d hospifital (day pleural aindpler'icardi:il frictioni rrmlbs wo-rvle hear d. The corigestix failurle uniprioved folloixvir, digitaliis and diuretie thlerapyv. An increase in predrris:one dosagqe led to imiproveiment Figure l?ocrlryer oryaqiant of hte of intensive crles 4 ini exsr- ,? rrftter 4 weeks tlrthcrap joinit p1)irrs, skSin raish, and(I graqdualI resolitiorn of the ffirsinras orveir a 4--week period (fig. 4). The pulse pres,sur.e did not f.ll below 20 na-in. of p,1- of ior did t(ile venouspl);l'(ssiire exeeed 170 inm. of ater4. TThe paitient has not been seen since discha-rge. Case 4 A 133-yea-old school girl was a-dmitted to St. TLuke-'s lospitall -with joilit paiins, fever. a,nd a diffmrse maculopaplar scalin skin raIsh for 1 week foIOillo'f a s;ore tlr'at. Phxsical examin"ation revea1led tavelveairdifr of 120, ai irade TT, aipieal systolie hiea1r4t n11rnrinr11q, ar1d mlltiple wTarm, tender, swx ollen. slimlrtlvcl thematous ioints. Xn electroeai'1dio-r'ar1r1 disclosed tIle Wernekeha,eh phenomenon. The x'lirito blood eell ,ount wars 5,400 per nim.3, tie herrroblobii wVas 9 0 GI. per cenlt, and chest x- "v- reTveanled aii enl'rm'ed nlobul:ar heart eornn'rtqlno w ithl perieaq rld,ial effusion (firr 5). Tire fatient wu-as believed to have aecute rheinarritic fever wvithi mvoe:arditis, periearditis. and peric'rm'dirrl effusion. She waqs griven 40 imr. of redlnisone a daT aiid as she iniproved and her eleetroeardlionraureinverted toevard norma1l. the doso was r1m.aduallav redueed. Beeause of the skin ra,sh and hemnrrtoloic fnding'_s, LE; preparations Were r positive on many oceasions. rirde. whliel were Prednllisoel0 teatri11rent wats eonltinued, hiut the pa tient Tims never' been completelv free of fever and -joint pairri. Despite intensive theraipy she has exper1ienced 3 ex-aceerbalti.ons of her disease. The periea,-1rdial effusion iezidu,911y resolved over a 3-month pertiod (fig. 6). Att preselit sIre is fairly well eortralled on moetlh> 1-prednisolone and, ehloroquine buLt retainis ani e xt\tre1ie "Cushiin-oid" appearance. Circulation, Volume XXII. July 1960 Downloaded from http://circ.ahajournals.org/ by guest on September 9, 2014 IPERLCA1I1)1A.I EFFUSIUON 147 LE il :_AE A'8 Figure 5 Posterooertrior root/Ify/i ooroln o(f the chest in ctO-Ise i, s/tol-ing Ce liircjnirnf Of it toe ubhoorfte. Figure 6 JPosteront eriot lroci o idgi -nor l l Of] thte iles t iii OSf? 4 offter, re(is sio o! f/IC lap plis tJffbCiflto sus. Discussion Ful pia1tiii1ts with cl Jei(ecatitdlil effu)sion, as a major. Illtlnifestation oftluips elmx-theiiatosins (lisseliiiatiis w elre I>11'0enotnnleed.-I. II 2 inistaiees slig te S ifi-i ea11iee of the efflsiIIo was 1 IIIisiIIter. yrete(l 8111(1 ill 1 8|1 ilC(Ol'e11ei0diaoiliosis waiS made. 1lii 1he laltter iistiice, )1i)tpl thlelrcapyv w7a1s (lelaved f-or 18 iioi-itlis. Thflt all 4 pat ients w eive Ne,irroes is Hotlwortly, s1ilnee less thani 3(0 per eeiit of tlei genier:al eline p)p)a1t ioI of St. 111ke s liosp|li tal is -Negro. That 2 ol' the paltieiits ale 11ei is also strikingy. 'r51wo of the (ri oup) .1W doiltig welI Cati1d the thir(d hias beeni reporited to he flee of conimplaiitis witlimut iiiediceitiiiis. ()ily the Y iiiest i)atieit seeustis to he doilomg. poorly; ill lher (c/se sile effects oh ftieil)py uel povingI as (lifficult .1s Ile (1 iswase. Mlany of the e inle(al and laboratorl inani festatiois of 1lipIs; ervthiemlfat)sus wer-e seen iin these ptatieits. No eorielatioii of svmi])tolis, plhysieal fitlitnins, otr al boratotrv tests won (1l predict the (,leYel)lileiit of p)(eiea1 dial effasioil. The usual phisieal fiiiiliuigs i1l(1 electr-o cardiograplie evidleiiee of periiear dial disease Were p)reselit hut wele sinilal to filnilidins ill i etiologyv. periCarditis and efflIsioi I of am (l-rist'Iaii rejoil edi a syii(dltoIIie thcat ineluded inflaiillamatimii ol'ser1ons iebtiiibres, and the samlle year Beli. }h lKleimpei er, and Schifri1:i noted the association of a diffuse vcaseular disease; Awith lupu 1 s eryithelliatosus. Jarehol reit erat ed that lnlns eryItelicatosus Wa s a g'eliiel'a lied vasculfar disease aid r-e l)orted 1 (ease of loeulatedi periearlial effius-ioiil aid another cease Awith pericardial adhiesioiis. lin 1938 acutte (issenlnli-ated lupus etheniat.osuis Aas replorted in 2 piatients Awho bad livdopeieadnu. Contratto and Levine in 19389, reported a ease ol' aeute 1u1us erv- Review of Literature lfii 1924 1ih)mairi and Sacks1 described the \valvular and eidocaidial iuaiiifestai ionls of IllpilScse rthlemiatoslis. El('e've1 yea ils I a tI e theniatosus with a piericardial fr-iction rub and i}erieardial flnidt. Jhe\ iioted tlhe sinilaritv of their case to eases oiluaculte rhennatlie e'ver, iiue1](-]udligr aprolto-ed l1P-4 interval. Thl associatioii of -ver-rueoius eiudoe,ar(Iiti.s aid I)ericar(Ill ilnid iii ae ]merytheniatosus was By lount ami-l 11carrett in 1.945. deseribed bNKlelJerer, PO11l(a,l;c aI(n htai1t reviewed 35 eases of dissemintted hi pus eryilieniat osns aild eoneluded tialt the (diffulse (eoniiee(tive tissine lesion and its progri essive degenerative iatnre wvere iiidieadive of a sensitivity ieaetioii. TIis idea reeeirxed su)pp)ort fromi a repIort xy Fox'1 of a patient wiho received ut tetaiilus seruiii a1m1(1 developed 1111)1,; erytheiiai osiIs. l otll(her5] ' asSociatiol reports111 wtis iiotedl (tallerg-ieo recaetiols ;iiid perioardia)l ablioiial- (ircufatior, Voiunuc XXJI, July 1960 Downloaded from http://circ.ahajournals.org/ by guest on September 9, 2014 148 BERGEN ities, both clinically11 12 and experimentally.13 This hypothesis has been further developed by Dameshek,14 who referred to systemic lupus erythematosus as "a complex auto-immune disorder. " Kleniperer et al.9 noted the frequent occurrenee of fibrinous pericarditis in lupus erythematosus, and a few years later Aegerter and Longl' called attention to the multiple mesenehymal lesioiis and serous effusions of the collagen diseases. In 1949 Curtis and Horne6 described what they believed were the first reported cases of lupus erythematosus with pericardial effusion as the main manifestation. One of their 2 patients underwent repeated pericardiocentesis for relief. Recently there have been many reports of pericarditis as a manifestation of lupus erythematosus,17-24 with the incidence varyinig from 1823 to 50 per cent.24 23 Acute pericardial effusion has been noted less frequently, 18 20, 22, 23, 23-27 with an incidence of 826 to 18 per cent.23 Most of the effusions were small, but occasionally were massive.20 22 23, 25, 28, 29 Pericarditis has also been noted in discoid lupus.30 Harvey et al.28 emphasized that pericardial involvement usually occurs during an acute exacerbation of the illness. The most common manifestation of pericardial involvement seems to be pericardial rub7 22, 23, 25-28, 31, 32 with chest pain,22, 25, 28, 32 gallop,28' electrocardiographic changes,7 8, 16, 22, 23, 25, 28, 32 failure,28' cardiomegaly,16' 25, 33 cardiae and arrhythmias28 also being noted. In most cases of lupus erythematosus adhesive fibrinous pericarditis is found 31, 33 at autopsy7' 8, 9, 16, 17, 19, 23, 27, 29, 34, 35 although Harvey et al.28 reported 2 cases of purulent pericarditis. No inistance of constrictive pericarditis has been reported thus far 25, 28 but tamponade has been noted.'6 22 23. 25 The fluid does not have any special characteristic22' 25 and has been variously described as turbid,5 34 serosanguineous,28' 29 clear brown,7 and clear, pale yellow.'6 The amount varies from a few cubic centimeters3' 36 to 3 liters,24 with some cases requiring pericardiocentesis for relief.'16 23, 24, 32 In at least 3 cases typical LE cells have been demonstrated in the pericardial fluid.22' 32, 37 Radiologic reviews of the x-ray manifestations of lupus erythematosus usually mentionl pericardial effusions.20 38 The differentiation between acute cardiac dilatation and pericardial effusion is often difficult and is frequently 0 resolved only by angiocardiography.20 Pericardial biopsy may also be helpful.41-44 It is well known that lupus erythematosus is rare in men,'4' 28 and at one time it was felt to be rare in non-Caucasian races.28 An incidence in Negroes in larger series26 belies this impression aind is close to the incidence of Negroes in the gemmeral population.45 Of the 5 case reports of lupus in the male Negro4 27, 34, 36 (as is true of the 2 Negro men in this report) pericardial involvement with effusions of various quantities was present in all. The occurrence of false-positive serologic tests for syphilis, as noted in case 3 of this group, has been well doeunlented in from 1526 to 44 per cent45 of patients with lupus erythematosus. The treponerna pallidum immobilization test can be used for differentiation from actual syphilis if necessary.46 Since 3 of the 4 cases in this report were initially misdiagnosed and 2 were incorrectly treated, a greater awareness of pericardial effusion as a possible manifestation of lupus erythematosus may be needed. McKusick and Harvey25 and McCuiston and Moser22 have suggested that many so-called idiopathic or nonspecific cases of pericarditis are actually cases of lupus erythematosus. In many reports idiopathic pericarditis is considered a sensitivitv47' 48 or immune reaction.49 If this possibility is kept in mind, a greater effort to secure tissue or bacteriologic proof is iieeded before pericarditis is arbitrarily considered to be idiopathic, viral, or tuberculous in origin. A recent review22 reveals the similarities and dissimilarities between idiopathic pericarditis amid the pericarditis of lupus erythematosus. Awareness of urinary, hematologic, skin, and sernum protein changes will help in the differentiation of these 2 types of pericarditis. Angiocardiography, pericardiocentesis, and periCirculation, Volume XXII, July 1960 Downloaded from http://circ.ahajournals.org/ by guest on September 9, 2014 PERICARDIAL EFFUSION cardial biopsy may aid in making the correct diagnosis. Medically, the treatment of choice is administration of the adrenal steroid hormones'8, 31, 32 with whatever maintenance dosage is needed to avoid recurrence. Surgicallv, pericardiocentesis may prevent tamponade, and in refractory cases, such as case 1, actual open drainage or pericardiopexy may be useful.3 Summary Four cases of systemic lupus erythematosuis with pericardial effusion as a prominent maniifestation are reported. Lack of awareness of this finding as a not infrequent part of the disease entity led to misdiagnosis in 2 instances and incorrect treatment in 1. The literature on the subject is reviewed and suggestions are offered for diagnosis. Summario in Interlingua Es reportate quatro casos de systemic lupus erythematose con effusion pericardial como manifestation prominente. Non-recognition del facto que effusion pericardial es un elemento non infrequente in le tableau clinic de systemic lupus erythematose esseva le causa de un misdiagnose in duo del casos e de unI incorrecte forma de tractamento in un. Le litteratura concernite con iste thema es revistate. Suggestiones pro le diagnose es offerite. References 1. LIBMAN, E., AND SACKS, B.: A hitherto undescribed form of valvular and mural endocarditis. Arch. Int. Med. 33: 701, 1924. 2. CHRISTIAN, H. A.: Long continued fever with inflammatory changes in serous and synovial membranes with essential glomerulonephritis: A clinical syndrome of unknown etiology. M. Clin. North America 18: 1023, 1935. 3. BAEHR, G., KLEMPERER, P., AND SCHIPRIN, A.: A diffuse disease of the peripheral circulation (usually associated with lupus erythematosus and endocarditis). Tr. A. Am. Physicians 50: 139, 1935. 4. JARCHO, S.: Lupus erythematosus associated with visceral vascular lesions. Bull. Johns Hopkins Hosp. 59: 262, 1936. 5. Case Records of the Massachusetts General Hospital: Case # 24201. Acute disseminated lupus erythematosus; hydropericardium. New England J. Med. 218: 838, 1938. 6. Case Records of the Massachusetts General Hospital: Case # 24341. Acute disseminated lupus Circulation, Volume XXII, July 1960 149 7. 8. 9. 10. 11. erythematosus; hydropericardium. New England J. Med. 219: 273, 1938. CONTRATTO, A. W., AND LEVINE, S. A.: Acute lupus erythematosus disseminatus report of a case. New England J. Med. 221: 602, 1939. BLOUNT, S. G., JR., AND BARRETT, J. T.: Acute lupus erythematosus disseminatus: A report of a case in a male with associated atypical verrucous endocarditis (Libman-Sacks). Ann. Int. Med. 23: 251, 1945. KLEMPERER, P., POLLACK, A. D., AND BAEHR, G.: Pathology of disseminated lupus erythematosus. Arch. Path. 32: 569, 1941. Fox, R. A.: Disseminated lupus erythematosusan allergic disease. Arch. Path. 36: 311, 1943. CLARK, E., AND KAPLAN, B. I.: Endocardial, arterial and other mesenchymal alterations associated with serum diseases in man. Arch. Path. 24: 458, 1937. 12. HARKAVY, J.: Vascular allergy. Arch. Int. Med. 67: 709, 1941. 13. WILCOX, H. B., AND ANDRUS, E. C.: Anaphylaxis in the isolated heart. J. Exper. Med. 67: 169, 1938. 14. DAMESHEK, W.: Systemic lupus erythematosus. Ann. Int. Med. 48: 707, 1958. 15. AEGERTER, E., AND LONG, J. H.: The collagen diseases. Am. J. M. Sc. 218: 324, 1949. 16. CURTIS, A. C., AND HORNE, S. F.: Disseminated lupus erythematosus with pericardial effusion. Ann. Int. Med. 30: 209, 1949. 17. BAGGENSTOSS, A. H.: Visceral lesions in disseminated lupus erythematosus. Proc. Staff Meet., Mlayo Clin. 27: 412, 1952. 18. COHEN, H., AND CADMAN, E. F. B.: The natural history of lupus erythematosus and its modification by cortisone and corticotrophin (ACTH). Lancet 2: 305, 1953. 19. GOLD, S. C., AND GowING, N. F. C.: Systemic lupus erythematosus, a clinical and pathological study. Quart. J. Med. 22: 457, 1953. 20. GOULD, D. M.., AND DAVES, M. L.: A review of roentgen findings in systemic lupus erythematosus (SLE). Am. J. M. Sc. 235: 596, 1958. 21. MEACHAM, G. C., AND WEISBERGER, A. S.: Unusual manifestations of disseminated lupus erythematosus. Ann. Int. Med. 43: 143, 1955. 22. MCCUISTON, C. F., AND MOSER, K. M.: Studies in pericarditis. I. Differentiation of the acute idiopathic form from that occurring in disseminated lupus. Am. J. Cardiology 4: 42, 1959. 23. SHEARN, M. A., AND PIROFSKY, B.: Disseminated lupus erythematosus. Arch. Int. Med. 90: 791, 1952. 24. TUMULTY, P. A.: The clinical course of systemic lupus erythematosus. J.A.M.A. 156: 947, 1954. 25. McKUSICK, V. A., AND HARVEY, A. McG.: Diseases of the pericardium. Advances Int. Med. 7: 157, 1955. Downloaded from http://circ.ahajournals.org/ by guest on September 9, 2014 BERGEN 150 26. JESSAR, R. A., LAMONT-HAVERS, R. W., AND RAGAN, C.: Natural history of lupus erythematosus disseminatus. Ann. Int. Med. 38: 717, 1953. 27. LINDAU, W.: Subacute disseminated lupus erythematosus in the Negro male. Southern M. J. 46: 1099, 1953. 28. HARvEY, A. McG., SHULMAN, L. E., TUMULTY, P. A., CONLEY, C. L., AND SCHOENRICH, E. H.: Systemic lupus erythematosus. Reviews of the literature and clinical analysis of 138 cases. Medicine 33: 291, 1954. 29. HILL, L. C.: Systemie lupus erythematosus. Brit. M. J. 2: 655, 1957. 30. DUBOIS, E. 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