w w w . r e u m a t o l o g i a . c o m . b r
REVISTA
BRASILEIRA
DE
REUMATOLOGIA
Review
article
Scientific
people
named
in
the
classification
of
vasculitis
Gokhan
Sargin
∗,
Taskin
Senturk
AdnanMenderesUniversityMedicalFaculty,DepartmentofRheumatology,Aydin,Turkey
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Articlehistory:
Received11September2015 Accepted6May2016
Availableonline25August2016
Keywords:
Vasculitis Scientificpeople Eponym
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ThefirstInternationalChapelHillConsensusConferencewasheldin1994.Therehavebeen suggestionsaboutthenomenclatureofsystemicvasculitis.Importantcategorieswereadded totheclassificationofvasculitis,andmanychangesweremadefordiseasenamesinthe secondChapelHillConsensusConference2012,whichwerenotincludedintheChapel HillConsensusConference1994.Thenewnomenclaturewasintroducedinsteadofbeing referredtobymanynamessuchasChurg-StraussandWegener”s.Newcategoriessuchas Behc¸et”sandCoganetc.werealsoadded.Thesepeoplearehonoredbytheclassification. Theycontributetosciencethroughtheircasestudies,scientificarticles,andobservations. Thisarticlereviewsonlyeponymspresentinthecurrentclassificationofvasculitis.The aimofthispaperistogiveinformationaboutscientistsmentionedintheclassificationof vasculitis.
©2016ElsevierEditoraLtda.ThisisanopenaccessarticleundertheCCBY-NC-ND license(http://creativecommons.org/licenses/by-nc-nd/4.0/).
Nomes
de
cientistas
usados
na
classificac¸ão
das
vasculites
Palavras-chave:
Vasculites Cientistas Epônimo
r
e
s
u
m
o
AprimeiraInternationalChapelHillConsensusConference(CHHC)ocorreuem1994. Fizeram-se sugestõessobreanomenclatura das vasculitessistêmicas. Nasegunda CHHC,2012, adicionaram-se importantes categoriasà classificac¸ãoda vasculite e fizeram-se várias mudanc¸asemnomesdedoenc¸asquenãoestavamincluídasnaCHCC1994. Introduziu-seumanovanomenclaturaemvezdeseusaremnomescomoChurg-StrausseWegener. Tambémforamadicionadasnovascategorias,comodeBehc¸eteCoganetc.Essaspessoas sãohomenageadaspelaclassificac¸ão.Elascontribuíramparaaciênciacomseusestudos decaso,artigoscientíficoseobservac¸ões.Esteartigoanalisaapenasepônimospresentes naclassificac¸ãoatualdasvasculites.Oobjetivoéprestarinformac¸õessobreoscientistas mencionadosnaclassificac¸ãodasvasculites.
©2016ElsevierEditoraLtda.Este ´eumartigoOpenAccesssobumalicenc¸aCC BY-NC-ND(http://creativecommons.org/licenses/by-nc-nd/4.0/).
∗ Correspondingauthor.
E-mail:[email protected](G.Sargin).
http://dx.doi.org/10.1016/j.rbre.2016.06.002
2255-5021/©2016ElsevierEditoraLtda.ThisisanopenaccessarticleundertheCCBY-NC-NDlicense(http://creativecommons.org/
Introduction
Systemicvasculitisisaninflammatorycondition.Theprimary inflammatory process involves the vessel wall ofdifferent organsandsystems,affectingbloodvesselsofdifferenttypes andsizes. Thenomenclatureand classificationofsystemic vasculitishasbeenaproblemforresearchersandclinicians formanyyears.1Therearedifferentwaysofclassifying
vas-culitides that include the size of predominantly affected vessels,typeofinflammatoryinfiltrate(e.g.neutrophilic, lym-phocytic), etiological agent(primary or secondary), disease extension(single-organvasculitisorsystemicvasculitis)and the pathophysiological mechanism involved (e.g. immune complexdeposits,ANCA).ThefirstInternationalChapelHill ConsensusConference(CHHC)washeldin1994.2Therewas
an attempt to replace eponyms by noneponymous terms whichwouldrepresentthepathophysiologicprocess. Impor-tantcategorieswereaddedtotheclassificationofvasculitis, and many names of diseases were changed atthe second CHHC2012,whichwerenotincludedintheCHCC1994.2,3The
newnomenclaturewasintroducedinsteadofbeingreferred to by many names such as Churg-Strauss and Wegener’s. The new categories were also added to the classification system,suchasvariable vesselvasculitis.Thesepeopleare honored by the classification. They contribute to science through their casestudies,scientific articles, and observa-tions.Thearticlereviewsonlyeponymspresentinthecurrent classificationofvasculitis.Theaimofthispaperis togive informationaboutscientistsmentionedintheclassificationof vasculitis.
Large
vessel
vasculitis
Takayasuarteritis
Takayasuarteritis(TA)isachronic,idiopathic,granulomatous arteritisoftheaortaanditsbranches.Itisaformoflargevessel vasculitis,andusuallyaffectsyoungerpatients(<50years).2,3
Thediseaseisalsoknownaspulselessdisease.Here,thename comesfromMikitoTakayasu.HewasaJapanese ophthalmolo-gist,bornin1860.TakayasugraduatedfromtheTokyoImperial Universityin1887.Hereportedacaseatthe12thAnnual Meet-ingoftheJapanOphthalmologySociety.4,5 Thepatienthad
noabnormalityinhermedicalexamination,exceptfor pecu-liarchangesoftheretinalcentralvesselswithaortitis.Inthe patient’s history, therewere visual disturbances,and com-plete lossofvisual acuitywithretinal abnormalities.After presentationofthiscase,itwaspublishedintheActaofthe OphthalmicSocietyofJapanin1908.6Similarcaseswerereported
consequently.Itwasreportedthattheterm‘Takayasu arteri-tis’ was first used by YasuzoShinmi, and officially named as ‘Takayasu arteritis’ by the researchers committee of the DepartmentofHealthandWelfareofJapanin1975.4,7
Mik-itoTakayasu died inNovember 1938.4,5 Although,there are
othersynonyms,thediseasewascalledasTakayasuarteritis inthenomenclatureofvasculitidesatthe2012international CHCC.3
Medium
vessel
vasculitis
Kawasakidisease
Kawasakidiseaseisamedium-sizedvesselvasculitis(visceral arteries,itsbranches,andespeciallycoronaryarteries),and usuallyoccurs inyoung children.Thedisease is character-ized byfever,erythematousrash,conjunctivitis,strawberry tongue, lymphadenopathy, and specific desquamations.2,3
ThediseasenamecomesfromTomisakuKawasaki.Hewas aJapanesepediatrician,borninTokyoin1925.Kawasaki grad-uatedfromtheSchoolofMedicine,ChibaUniversityin1948.8
Hedescribedaboyaged4yearsand3monthswithhighfever, mucocutaneous features, and cervical adenopathy in1961, andpresentedsevencasesentitle‘Non-scarletfever desquama-tionsyndrome’in1962attheChibaPrefecturePediatricMeeting, and20casesentitle‘Twentycasesofocular-mucocutaneous syn-drome’in1964atthemeetingofthe15thEasternandCentral JapanPediatricMeetinginMatsumoto.9–11Hepublisheda
clin-icalobservationof50patientsin1967underthetitle“Acute febrilemucocutaneoussyndrome”.12Inthisarticle,patientshad
lymphoidinvolvementwithspecificdesquamationofthe fin-gers and toes. Later, Kawasaki et al. reported 50 cases in September1974atPediatricsentitled‘Anewinfantileacutefebrile mucocutaneous lymph nodesyndrome prevailingin Japan’.13 He
retired from the pediatric department of Japan Red Cross Hospital.8
Small
vessel
vasculitis
Granulomatosiswithpolyangiitis(Wegener’s)
Granulomatosis with polyangiitis (Wegener’s) is an ANCA-associated multifocal necrotizing granulomatous vasculitis thataffectssmalltomedium-sizevesselsofthekidney,lower and upper respiratory tract.2,3 The disease is named after
FriedrichWegener,aGermanpathologist,bornin1907inVarel, Germany.14–16 Hecompletedhismedicaleducationin1932,
studied at the pathology department of Kiel University.16
Hewas reportedtobeamember oftheNaziparty,aswas halfofGermandoctorsduringWorldWarII.17–19 InKiel,he
described acasewith generalizedangiitis, and necrotizing granulomaofthe upperand lower respiratorysystem, kid-ney and spleen.14 Althoughhe workedas apathologist in
Lodz(alocalizedJewishghetto),therewereconflictingreports aboutwhereheworkedinthehealthoffice.16,19 Despitethe
suspicion, Wegener was released due to lack of evidence asawar criminal.16Itisreportedthat hewas silentabout
the events until his death.20 Wegener reported a peculiar
rhinogenicgranulomatosiswithmarkedinvolvementofthe arterial system and kidney,and published articles in1936 and 1939.21,22 In these articles, the disease’s clinical and
pathological features were defined. The term of‘Wegener’s granulomatosis’wasfirstusedbyGodmanandChurg.23Falk
etal.recommendedanalternativenameforWegener’s gran-ulomatosis: Granulomatosiswithpolyangiitis (Wegener’s).18
2012.3 It was identified as Wegener’s in brackets to avoid
confusioninliterature.Hereceivedamasterclinicianprize from the American College of Chest Physicians in 1989.24
Rosen reportedatthe CHEST in2007that the board voted almostunanimouslytorescindtheaward.25Hediedin1990,
inLübeck.14–16
Eosinophilic
granulomatosis
with
polyangiitis
(Churg-Strauss)
Eosinophilicgranulomatosiswithpolyangiitis(Churg-Strauss) isANCA-associatednecrotizinggranulomatousvasculitisthat affects predominantly small to medium-size vessels. It is generallyassociated withasthma, paranasalsinusitis, pul-monaryinfiltrates,neuropathy,andeosinophiliaofperipheral blood or tissue.2,3 Thedisease is named afterJacob Churg
and Lotte Strauss. Jacob Churg was a pathologist, born in 1910,Dolhinow.26,27Hewasinterestedinmedicinesincethe
ageofeight,workedinthedepartmentofinternalmedicine, butwas reportedassaying‘I wasnotvery successfulwith patients’.27 He then workedas an assistantin the
pathol-ogydepartmentofVilnaUniversity.Hedescribed apatient withasthma,lymphadenopathy,andeosinophilia,whodied ofcranialhemorrhage.27Thebiopsyspecimensoflymphnode
showedeosinophilic infiltration,early granulomas, vasculi-tisincranialarteries,andgranulomasinvarioustissues.Dr. Straussalsohadsimilarcases.26LotteStrausswasa
patholo-gist,bornin1913inNuremberg,Germany.26 Shespecialized
in pediatric and perinatal pathology. They, both reviewed cases with asthma, fever, and hypereosinophilia. Most of them had characteristic, specific anatomical lesions with histopathologicentitytermed‘allergicgranuloma’.Theirarticle waspublishedin1951entitle‘AllergicGranulomatosis,Allergic Angiitis,andPeriarteritisNodosa’.28ChurgandStraussdescribed
theentityand calleditthe Churg-Strausssyndrome.When searchingtheterm‘Churg-Strausssyndrome’onPubMed,we foundanearlyarticlereportedbyAbul-HajSkandFlanaganP, in1961entitled‘Asthmaassociatedwithdisseminatednecrotizing granulomatousvasculitis,theChurg-Strausssyndrome.Reportofa case’.29JacobChurgdiedin2005,andLotteStraussin1985.26
IgA
vasculitis
(Henoch-Schönlein)
IgA vasculitis (Henoch-Schönlein) generally affects small vessels with immunoglobulin A deposits and granulocytes in the vessels. The clinical features include palpable pur-pura,arthritis,abdominalpain,gastrointestinalbleeding,and glomerulonephritis.2,3 The disease is named after Eduard
HeinrichHenochandJohannLukasSchönlein.Eduard Hein-richHenoch was aGerman pediatrician, bornin Berlinon July16,1820,anddiedinDresdenonAugust28,1910.30,31He
completedhismedicaleducationin1843,andstudiedwith MoritzRombergandJohannLukasSchönlein.Heworkedas headofthepediatricclinicoftheRoyalCharitéHospital.He publishedhis famoustextbook called‘Lectures onChildren’s Diseases’.32 On the other hand, he reported a
fifteen-year-old-malepatientwithabdominalpain,arthralgia,purpura;a seven-year-oldmale withpurpura,nephritis,and apatient
withhematuriaand purpura.Thisarticlewaspublishedin 1868inthe‘KlinWschr’.33Thesefindingswerealsodescribedby
JohannLukasSchönleinasanentity.SchönleinwasaGerman ProfessorofInternalMedicine,borninBamberg,Germanyon 30November1793.34,35HeworkedinWürzburg,Zürich,and
Berlin.Schönleinwrotefewpapers,anddescribedrheumatica purpurarubrawithcutaneous,arthriticsymptomsandrenal lesions.36,37 He alsodescribed typhoid crystals in patients’
stools,andthecausativeagentoffavus.JohannLukas Schön-leinretiredin1859anddiedinBamberginJanuary1864.34–36
Variable
vessel
vasculitis
Cogan’ssyndrome
Cogan’s syndrome is a rare chronic inflammatory dis-ease characterizedbylargetosmall-sized vesselvasculitis, nonsyphiliticinterstitialkeratitisandvestibulo-auditory dys-function such as tinnitus, hearing loss, and vertigo.3 The
disease is named afterDavid Glendenning Cogan. He was an American ophthalmologist, born in Massachusetts in 1908.38HewasgraduatedfromtheDartmouthCollege,
Har-vardUniversity,andcontinuedhiscareeratHarvardMedical School,ChicagoUniversityClinics,andMassachusettsEyeand Ear Infirmary. Cogan was certified by the American Board of Ophthalmology in 1937, and became a member of the editorial board at ArchivesofOphthalmology, Investigative Ophthalmology,AlbrechtvonGraefesArchivfurKlinischeund ExperiementelleOphthalmologie,andJournalofNeurological Science.38In1945,hereportedfivepatientswithnonsyphilitic
interstitial keratitis and vestibulo-auditory symptoms as a syndromeattheArchivesofOphthalmology.39Hence,this
clini-calentityhascometobecalledasCogan’ssyndrome.Hewas interestedinneuro-ophthalmology,andophthalmic manifes-tationsofsystemicvasculardisease,andhepublishedmany books on these topics.40,41 He died inWayne aftera heart
attackonSeptember9,1993.42
Behc¸et’sdisease
Behc¸et’sdiseaseisachronic,inflammatory,multisystem vas-culitisand ischaracterizedbyrecurrentoralulcers,genital ulcers,anduveitis.3Thediseasegenerallyaffectethnicgroups
suchMediterraneanandEastAsianalongtheSilkRoad.Hulusi Behc¸etwasaTurkishdermatologist,thefirstprofessorinthe Turkishacademiclife,borninIstanbul,TurkeyonFebruary20, 1889.43,44Hecompletedhismedicaleducation,andgraduated
fromMilitaryMedicalSchoolin1910,thenworkedasan assis-tantintheGulhanedermatologyclinicuntil1914.InJuly1914, hewasappointedasChiefAssistantattheKırklareliMilitary Hospital,andworkedasadermatologistattheEdirneMilitary Hospital.HealsoworkedatCharitéHospital,Hasköy Derma-tologyandVenerealDiseasesHospital,andGurabaHospital.45
Mischner in 1947at the International Medical Congress of Geneva.45ThediseaseisencodedasBehc¸et’sdisease(M35.2),
atICD-10Version:2015.47HewashonoredwiththeTÜB˙ITAK
ScienceAwardin1975,andEczacıbas¸ıScienceAwardin1982.45
CoinswerealsoproducedforhistributeduringtheNational DermatologyCongressin1996.HulusiBehc¸etdiedinMarch8, 1948.43–45
Conclusion
Althoughthenamedvasculitisinthisarticlehadbeen previ-ouslydescribedbyothervaluablescientists,theseeponyms arecommonlyusedinthenomenclatureofclassificationof vasculitis after scientists have honored them. The Ameri-canCollegeofRheumatology,the EuropeanLeagueAgainst Rheumatism,andthe AmericanSociety ofNephrology rec-ommend diagnostic terms instead of eponyms such as Churg-Strauss, Wegener’s etc. Many new nomenclatures, anddiagnostictermsexplainclinico-pathologicalinformation aboutthedisease.Asaresult,thesepeoplewhohave con-tributedtoscienceemphasizeaboutthesignificanceofclinical observation,approachandresearch.
Conflicts
of
interest
Theauthorsdeclarenoconflictsofinterest.
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