The Digestive Involvement in Systemic Autoimmune Diseases

461 Pages·2017·10.032 MB·English

by  Manuel Ramos-Casals, Munther Khamashta, Pilar Britó-Zeron, Fabiola Atzeni and Joan Rodés Teixidor (Eds.)

Handbook of Systemic Autoimmune Diseases
SeriesEditor:F.AtzeniandP.Sarzi-Puttini
Volume1 The HeartinSystemic AutoimmuneDiseases
Editedby: AndreaDoriaandPaoloPauletto
Volume2 Pulmonary InvolvementinSystemicAutoimmuneDiseases
Editedby: AtholU.WellsandChristopher P.Denton
Volume3 Neurologic InvolvementinSystemicAutoimmuneDiseases
Editedby: DorukErkan andSteven R.Levine
Volume4 ReproductiveandHormonalAspectsofSystemic AutoimmuneDiseases
Editedby: MichaelLockshinandWareBranch
Volume5 The SkininSystemicAutoimmuneDiseases
Editedby: PiercarloSarzi-Puttini,AndreaDoria,GiampieroGirolomoni
andAnnegretKuhn
Volume6 Pediatrics inSystemic AutoimmuneDiseases
Editedby: RolandoCimazandThomasLehman
Volume7 The KidneyinSystemic AutoimmuneDiseases
Editedby: JustinC.MasonandCharlesD.Pusey
Volume8 DigestiveInvolvementinSystemic AutoimmuneDiseases
Editedby: JosepFont,ManuelRamos-CasalsandJuanRode´s
Volume9 Endocrine ManifestationsofSystemic AutoimmuneDiseases
Editedby: SaraE.WalkerandLuisJ.Jara
Volume10 AntiphospholipidSyndromeinSystemicAutoimmuneDiseases
Editedby: RicardCervera,JoanCarlesReverterandMunther
Khamashta
Volume11 Pediatrics inSystemic AutoimmuneDiseases,SecondEdition
Editedby: RolandoCimazandThomasLehman
Volume12 AntiphospholipidSyndromeinSystemicAutoimmune
Diseases, SecondEdition
Editedby: RicardCervera,GerardEspinosa,andMunther Khamashta
Volume13 The DigestiveInvolvementinSystemicAutoimmuneDiseases,Second
Edition
Editedby: ManuelRamos-Casals,Munther Khamashta,Pilar Brito-
Zero´n,FabiolaAtzeni,andJoanRode´sTeixidor
Volume14 The HeartinSystemic AutoimmuneDiseases,SecondEdition
Editedby: FabiolaAtzeni,AndreaDoria,MichaelNurmohamed,and
PaoloPauletto
HandbookofSystemicAutoimmuneDiseases,Volume13
The Digestive Involvement
in Systemic Autoimmune
Diseases
SECOND EDITION
Edited by
Manuel Ramos-Casals
Sjo¨grenSyndromeResearchGroup(AGAUR),LaboratoryofAutoimmuneDiseases
JosepFont,CELLEX-IDIBAPS,DepartmentofAutoimmune Diseases,ICMiD,
UniversityofBarcelona,HospitalCl´ınic,Barcelona,Spain
Munther Khamashta
GrahamHughesLupusResearchLaboratory,DivisionofWomen’sHealth,
King’sCollegeLondon;TheRayneInstitute,StThomas’Hospital,London,
UnitedKingdom
Pilar Brito-Zero´n
AutoimmuneDiseasesUnit,DepartmentofInternalMedicine,Hospital
CIMA-Sanitas,Barcelona,andLaboratoryofAutoimmuneDiseasesJosepFont,
CELLEX-IDIBAPS,DepartmentofAutoimmuneDiseases,ICMiD,HospitalCl´ınic,
Barcelona,Spain
Fabiola Atzeni
RheumatologyUnit,L.SaccoUniversityHospital,Milan,Italy
Joan Rode´s Teixidor
LiverUnit,ICMD,Ciberehd,InstitutodeInvestigacionesBiome´dicasAugust PiI
Sunyer(IDIBAPS),HospitalClinic,Barcelona,Spain
AMSTERDAMlBOSTONlHEIDELBERGl LONDONlNEWYORKlOXFORD
PARISlSANDIEGOl SANFRANCISCOlSINGAPORElSYDNEYlTOKYO
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Dedication
The first edition of this book was one of the last projects led by Dr. Josep
Font (Barcelona, 1953e2006) before his unexpected death, and the
remainingeditorswishtodedicatetheselinesasameansofpayingadeeply
felt homage. Dr. Font devoted his professional career to the care of patients
with systemic autoimmune diseases (SAD). He trained in Internal Medicine
attheHospitalClinic ofBarcelonafrom1978 to1982 andobtainedhisPhD
in 1984 for his thesis on systemic lupus erythematosus (SLE). His post-
doctoral experience was closely linked with the Lupus Research Unit at St.
Thomas’ Hospital, London. His research output was prodigious, with a total
of over 500 scientific articles published over 25 years. In addition, he
designed and coordinated many international projects including the Euro-
lupus, Europhospholipid, Catastrophic Antiphospholipid Syndrome (CAPS)
Registry, Sjo¨gren syndrome-HCV Registry, and Hispanoamerican Registry
of Extrahepatic Manifestations of HCV (HISPAMEC) and was an active
member of some of the most prestigious international research groups, such
as the Eurolupus Nephritis Trial and SLICC.
In 1995,Dr.Fontestablished theDepartment ofAutoimmune Diseases at
the Hospital Clinic, a pioneering unit in Europe specifically dedicated to the
clinical management of patients with SAD. He played a leading role in
creating a network of different specialists at the Hospital Clinic dedicated to
the care of these patients. A key characteristic of his clinical research was its
multidisciplinarydesignandtheclosecollaborationbetweendifferentmedical
specialties, of which this book is an excellent example. He was working
actively until the last days of his life, and died like a soldier “with his boots
on’.”His integrity,intelligence, andloyaltymade himaformidable physician
andawonderfulcolleagueandfriend.Hewasundoubtedlyoneoftheforemost
figuresinthefieldofautoimmunediseasesinrecentdecades,andtheimmense
human andprofessional legacythat heleft mustbemaintained and continued
by all who had the great fortune to know and work with him.
For this second edition, we would also like to honor two of the main in-
ternationalexpertsinthefieldofautoimmunityandliverdiseaseswhorecently
retired, professors Jenny Heathcote and Joan Rode´s.
In June 2013 and after authoring 350 papers, conducting 800 lectures,
mentoring 64 residents, and training 31 fellows in hepatology, Prof. Jenny
Heathcote, a world-renowned liver specialist based at Toronto Western
vi Dedication
Hospital, fully retired from her position as Senior Scientist in the Toronto
Western. In 2009 she received the Chair in Hepatology at the University and
was awarded the EASL International Recognition Award (2010) for her sus-
tained contribution to the knowledge and understanding of liver diseases. In
thelastdecade,Prof.Heathcotehasbuiltupaworldrenownedliverunitand
fostered many new initiatives. We have decided to maintain the original
chapterwrittenbyProf.Heathcoteforthefirsteditionofthisbook(includinga
brief update for the past 5 years) as a tribute from the editors to their
outstanding work in liver diseases.
Prof. Joan Rode´s, born in 1938 in Barcelona, is considered one of the
most prestigious international hepatologists and his career is also closely
linkedtotheHospitalClinicofBarcelona;hewastheMedicalDirectorofthe
Hospital Clinic between 1984 and 1986 and General Director between 2003
and2008.TherecognitionofhisresearchworkhasledProf.Rode´stooccupy
positionsininternationalorganizations(PresidentoftheEuropeanAssociation
for the Study of the Liver in 1991, President of the International Association
forthe Study ofthe Liverin1992).He has authored orcoauthoredmorethan
500papersinmajormedicaljournalsinternationally,andwasalsofounderand
Director of the Institute of Biomedical Research August Pi i Sunyer (IDI-
BAPS).TheaccumulationofmeritsanddistinctionsreceivedbyProf.Rode´sis
more than an expression of an exceptional personality; a tireless worker with
extraordinaryleadershipqualities,abletocreate,enhance,andholdtogethera
group of leading researchers and a proverbial modesty.
Finally, we wish to thank Linda Versteeg-Buschman, Halima Williams
and their staff at Elsevier for their hard work. It has been a great pleasure
working together on this second edition.
The Editors
List of Contributors
R.Abdalian,UniversityHealth Network,Toronto, ON,Canada
N.Acar-Denizli,MimarSinanFineArtsUniversity,Istanbul,Turkey
G.S. Alarco´n, The University of Alabama at Birmingham, Birmingham, AL, United
States
J.AlSaleh,DubaiHospital,Dubai,UnitedArabEmirates
F.Atzeni,IRCCS Galeazzi OrthopedicInstitute,Milan,Italy
C.Ayuso,HospitalClinic, UniversityofBarcelona,Spain
K. Bari,UniversityofCincinnatiMedical Center,Cincinnati,OH,UnitedStates
R.P. Baughman, University of Cincinnati Medical Center, Cincinnati, OH, United
States
D.Ben-AmiShor,Tel-Aviv University,Israel
X.Bosch,HospitalCl´ınic,Barcelona,Spain
P.Brito-Zero´n,HospitalCIMA-Sanitas,Barcelona,Spain;LaboratoryofAutoimmune
Diseases Josep Font, CELLEX-IDIBAPS, Department of Autoimmune Diseases,
ICMiD,Hospital Cl´ınic,Barcelona, Spain
P.Cacoub,SorbonneUniversite´s,UPMCUnivParis06,UMR7211,andInflammation-
Immunopathology-Biotherapy Department (DHU i2B), Paris, France; INSERM,
UMR_S 959, Paris, France; CNRS, FRE3632, Paris, France; AP-HP, Groupe
Hospitalier Pitie´-Salpeˆtrie`re,Paris,France
R.Cervera,HospitalCl´ınic,Barcelona, Spain
R.W. Chapman, University of Oxford, Oxford, United Kingdom; John Radcliffe
Hospital,Oxford,UnitedKingdom
C. Commarmond, Sorbonne Universite´s, UPMC Univ Paris 06, UMR 7211, and
Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Paris,
France; INSERM, UMR_S 959, Paris, France; CNRS, FRE3632, Paris, France;
AP-HP,GroupeHospitalierPitie´-Salpeˆtrie`re,Paris,France
A.J.Czaja,MayoClinicCollegeofMedicine, Rochester,MN,UnitedStates
A.C.Desbois,SorbonneUniversite´s,UPMCUnivParis06,UMR7211,andInflammation-
Immunopathology-Biotherapy Department (DHU i2B), Paris, France; INSERM,
UMR_S 959, Paris, France; CNRS, FRE3632, Paris, France; AP-HP, Groupe Hos-
pitalierPitie´-Salpeˆtrie`re,Paris,France
S.DeVita,UniversityofUdine,Udine,Italy
xix
xx ListofContributors
H.Direskeneli,MarmaraUniversity,Istanbul,Turkey
F.Domont,SorbonneUniversite´s,UPMCUnivParis06,UMR7211,andInflammation-
Immunopathology-BiotherapyDepartment(DHUi2B),Paris,France;AP-HP,Groupe
HospitalierPitie´-Salpeˆtrie`re,Paris,France
L.Donoso,HospitalClinic,UniversityofBarcelona, Spain
G.Espinosa,HospitalCl´ınic,Barcelona,Spain
J.J.Ferna´ndez-Mart´ın,HospitalA´lvaroCunqueiro,Vigo,Spain
R.A. Ferrandiz, Hospital Nacional Cayetano Heredia and Universidad Peruana
CayetanoHeredia,Lima,Peru´
X. Forns, Instituto de Investigaciones Biome´dicas August Pi I Sunyer (IDIBAPS),
HospitalClinic,Barcelona, Spain
M.Gand´ıa,HospitalPuntaEuropa,Algeciras(Ca´diz),Spain;HospitalJerezPuertadel
Sur-ASISA,JerezdelaFrontera(Ca´diz),Spain
L.Guillevin,Universite´ Paris5 eRene´ Descartes,Paris,France
R.Hamad,Dubai Hospital,Dubai,UnitedArabEmirates
J.Heathcote,UniversityHealth Network,Toronto,ON,Canada
I.R.L.Kean,UniversityofWisconsineMadison,Madison,WI, UnitedStates
W.F.Kean,McMasterUniversityFacultyofHealthSciences, Hamilton, ON,Canada
M. Khamashta, Dubai Hospital, Dubai, United Arab Emirates; King’s College
London,London,UnitedKingdom
D.Khanna,UniversityofMichiganMedicalSchool,AnnArbor,MI,UnitedStates
B. Kostov, Institut d’Investigacions Biome`diques August Pi i Sunyer, Barcelona and
UniversityofBarcelona
M.P.Manns,HannoverMedicalSchool,Hannover,Germany
I.F.Masala,SantissimaTrinita` Hospital,Cagliari,Italy
G. Mieli-Vergani, King’s College London Faculty of Life Sciences & Medicine at
King’sCollegeHospital,London,UnitedKingdom
H.Mix,HannoverMedical School,Hannover,Germany
C.Morcillo,HospitalCIMA-Sanitas,Barcelona
J.Ordi-Ros,VallD’HebronGeneral Hospital,Barcelona, Spain
M.Page´s,HospitalClinic, UniversityofBarcelona,Spain
C.Pagnoux,UniversityofToronto,Toronto,ON,Canada
L.Pallare´s-Ferreres,HospitaldeSonEspases,PalmadeMallorca,Spain
N.P.Papageorgiou,AmericanMedical Center,Nicosia,Cyprus
R.Pe´rez-A´lvarez,HospitalA´lvaroCunqueiro,Vigo,Spain
M.Pe´rez-de-Lis Novo,Hospital A´lvaroCunqueiro,Vigo,Spain
L.Quartuccio,UniversityofUdine,Udine,Italy
ListofContributors xxi
K.D.Rainsford,SheffieldHallamUniversity,Sheffield, England,UnitedKingdom
M. Ramos-Casals, Sjo¨gren Syndrome Research Group (AGAUR), Laboratory of
AutoimmuneDiseasesJosepFont,CELLEX-IDIBAPS,DepartmentofAutoimmune
Diseases,ICMiD,UniversityofBarcelona,HospitalCl´ınic,Barcelona,Spain
S. Retamozo, Hospital Privado Universitario de Co´rdoba, Institute University of
Biomedical Sciences,UniversityofCo´rdoba(IUCBC),Co´rdoba,Argentina
I.Rodr´ıguez-Pinto´,HospitalCl´ınic,Barcelona, Spain
J.Romero-D´ıaz,InstitutoNacionaldeCienciasMe´dicasyNutricio´nSalvadorZubira´n,
Me´xicoCity,Me´xico
D.Saadoun,SorbonneUniversite´s,UPMCUnivParis06,UMR7211,andInflammation-
Immunopathology-Biotherapy Department (DHU i2B), Paris, France; INSERM,
UMR_S959,Paris,France;CNRS,FRE3632,Paris,France;AP-HP, GroupeHos-
pitalierPitie´-Salpeˆtrie`re,Paris,France
J. Sa´nchez-Guerrero, Instituto Nacional de Ciencias Me´dicas y Nutricio´n Salvador
Zubira´n,Me´xicoCity,Me´xico
J.-M. Sanchez-Tapias, Instituto de Investigaciones Biome´dicas August Pi I Sunyer
(IDIBAPS),Hospital Clinic,Barcelona, Spain
P.Sarzi-Puttini,UniversityHospitalL.Sacco, Milan,Italy
L.Savey,SorbonneUniversite´s,UPMCUnivParis06,UMR7211,andInflammation-
Immunopathology-Biotherapy Department (DHU i2B), Paris, France; AP-HP,
GroupeHospitalierPitie´-Salpeˆtrie`re, Paris,France
A. Selva-O’Callaghan, Universitat Auto`noma de Barcelona, Barcelona, Spain; Vall
D’HebronGeneral Hospital,Barcelona,Spain
Y.Shoenfeld,Tel-AvivUniversity,Israel
A.B.Shreiner,UniversityofMichiganMedicalSchool,AnnArbor,MI,UnitedStates
M.-J.SotoCa´rdenas,UniversityofCa´diz,HospitalPuerta delMar,Ca´diz,Spain
J.H.Stone,MassachusettsGeneralHospital,Boston,MA02114,UnitedStates
R.Talotta,UniversityHospitalL.Sacco,Milan,Italy
J.R.Teixidor,InstitutodeInvestigacionesBiome´dicasAugustPiISunyer(IDIBAPS),
Hospital Clinic,Barcelona, Spain
M.Vauthier,AP-HP,GroupeHospitalierPitie´-Salpeˆtrie`re,Paris,France
D. Vergani, King’s College London Faculty of Life Sciences & Medicine at King’s
CollegeHospital,London,UnitedKingdom
M.Vilardell-Tarre´s,VallD’HebronGeneral Hospital,Barcelona, Spain
K.D. Williamson, University of Oxford, Oxford, United Kingdom; John Radcliffe
Hospital,Oxford,UnitedKingdom
Preface
ThesecondeditionofTheDigestiveSysteminSystemicAutoimmuneDiseases
represents the state of the art in the field of digestive disorders for the most
common systemic and organ-specific autoimmune diseases.
This volume consists of an introductory section including chapters
focusing on etiopathogenic aspects along with a specific chapter on imaging
techniques in digestive diseases, followed by two chapters on organ-specific
autoimmune diseases and autoimmune manifestations of viral hepatitis
(including a new chapter on new anti-HCVagents).
The final section deals with the digestive manifestations of patients with
systemic and rheumatic autoimmune diseases, with the aim of being a prac-
tical guide to the identification, diagnosis, and treatment of digestive
involvementinthesepatientsthatwillbeusefulforallmedicalspecialties.For
this second edition, we have also included new chapters (spondyloar-
thropathies, Behc¸et disease, sarcoidosis and IgG4-related disease), expanding
to other rheumatic diseases and rare systemic diseases.
The final chapter is devoted to update the gastrointestinal and liver com-
plications of the rheumatologic medications used in daily practice.
We hope you will enjoy and learn from the second edition of this book.
The Editors
xxiii
Chapter 1
Digestive System and
Autoimmunity
H. Mix and M.P. Manns
HannoverMedicalSchool,Hannover,Germany
1. THE DIGESTIVE SYSTEM AND GUT-ASSOCIATED
IMMUNITY
Mammalsdependonfeedinganddigestion.Whilesingle-celledorganismscan
directly take in nutrients from their outside environment, multicellular or-
ganisms,withmostoftheircellsremovedfromdirectcontactwiththeoutside
environment,havedevelopedspecializedstructuresforobtainingandbreaking
down their food. Large, complex molecules must be broken down into
monomersthatcanthenbedistributedthroughoutthebodytoeverycell.This
vital function is accomplished by a series of specialized organs that comprise
the digestive system. The human digestive system is a coiled, muscular tube
about 6e9min length when fully extended, stretching fromthe mouth tothe
anus. Several specialized compartments occur along this length: the mouth,
pharynx, esophagus, stomach, small intestine, large intestine, and anus.
Accessory digestive organs are connected to the main system by a series of
ducts, including salivary glands, the pancreas, and the liver with the biliary
system. Like the skin, the digestive tract is situated at the interface between
externalandinternalmilieus.Tomaintainhomeostasis,physicalandchemical
mechanisms as elements of the innate immune response are used to protect
against exogenous, potentially noxious agents. The membranes of the diges-
tive tract provide a physical barrier against invading pathogens. A huge
number of chemical factors, including low pH in the stomach, pepsin, lyso-
zyme, antimicrobial substances such as cryptidins and defensins, limit the
growth and invasion of microorganisms [1] (Table 1.1).
Inadditiontoinnatedefensemechanisms,thedigestivesystemislinedby
mucosal lymphatic tissues [2]. It consists of diffuse lymphocytic infiltrates
throughout the epithelium and lamina propria of the mucosa or nonencapsu-
TheDigestiveInvolvementinSystemicAutoimmuneDiseases.http://dx.doi.org/10.1016/B978-0-444-63707-9.00001-5
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