Fauzia F. Naime. (2025). O mond’s Disease: A Challenging Diagnosis. MAR Oncology and Hema ology.
(2025) 5:09
O mond’s Disease: A Challenging Diagnosis.
Mayco da Sil a Bo a o 1, Fauzia F. Naime2*
1. In e nal Medicine Residen a he Mandaqui Hospi al Complex. h ps://o cid.o g/0009-0009-6663-8772
*Co espondence o: Fauzia F. Naime, Clinical Oncologis a he Paulis a Ins i u e o Oncology and he
D . A naldo Viei a de Ca alho Cance Ins i u e (ICAVC). Medical Residency P ecep o a he Mandaqui
Hospi al Complex and ICAVC. Mas e 's deg ee in Oncology. P o esso a he School o Medicine o No e
de Julho Uni e si y. h ps://o cid.o g/0000-0002-7944-8689
Copy igh .
© 2025 Fauzia F. Naime This is an open access a icle dis ibu ed unde he C ea i e Commons A ibu ion
License, which pe mi s un es ic ed use, dis ibu ion, and ep oduc ion in any medium, p o ided he o iginal
wo k is p ope ly ci ed.
Recei ed: 09 Sep 2025
Published: 17 Sep 2025
MAR Oncology and Hema ology (2025) 5:09
Case Repo
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Fauzia F. Naime. (2025). O mond’s Disease: A Challenging Diagnosis. MAR Oncology and Hema ology.
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Abs ac
Backg ound: O mond’s disease, o e ope i oneal ib osis, is a a e in lamma o y condi ion
equen ly associa ed wi h u e e al obs uc ion and p og essi e enal dys unc ion. In i s IgG4-
ela ed o m, i may mimic malignan , in ec ious, o au oimmune p ocesses, making diagnosis
pa icula ly challenging.
Case P esen a ion: We epo he case o a 61-yea -old male pa ien wi h p og essi e weigh
loss, abdominal pain, and omi ing, who de eloped acu e kidney inju y seconda y o bila e al
u e e al obs uc ion. Imaging e ealed a e ope i oneal mass encasing he u e e s and majo
essels. Mul iple biopsies con i med ch onic in lamma ion wi h ib osis and IgG4-posi i e
plasma cell in il a ion, es ablishing he diagnosis o IgG4- ela ed e ope i oneal ib osis.
Despi e pa ial imp o emen wi h co icos e oid he apy and elie o obs uc ion h ough
neph os omy and double-J ca he e placemen , he pa ien p og essed o ch onic kidney ailu e
equi ing long- e m hemodialysis. Du ing ea men , he also de eloped se e e in ec ious
complica ions, including in ec i e endoca di is equi ing ca diac su ge y. Ri uximab he apy
was subsequen ly indica ed due o e ac o y disease.
Discussion: IgG4- ela ed e ope i oneal ib osis sha es clinical and adiological ea u es
wi h malignan and in ec ious diso de s, making his opa hology and immunohis ochemis y
essen ial o de ini i e diagnosis. Fi s -line ea men wi h glucoco icoids o en leads o
clinical imp o emen , al hough ib o ic issue is less esponsi e, highligh ing he impo ance o
ea ly diagnosis. In e ac o y cases, immunosupp essan s such as i uximab and
mycophenola e mo e il ha e shown p omising esul s.
Conclusion: This case illus a es he se e e clinical cou se o IgG4- ela ed O mond’s disease,
emphasizing he need o ea ly ecogni ion, his opa hological con i ma ion, and imely
ini ia ion o he apy. Mul idisciplina y managemen is c i ical o p e en ing i e e sible
complica ions, such as ch onic kidney ailu e, and imp o ing pa ien ou comes.
Keywo ds: Re ope i oneal ib osis, O mond’s disease, IgG4- ela ed disease, hyd oneph osis,
ch onic kidney ailu e.
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Fauzia F. Naime. (2025). O mond’s Disease: A Challenging Diagnosis. MAR Oncology and Hema ology.
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In oduc ion
O mond’s disease, o e ope i oneal ib osis, is a a e condi ion i s desc ibed by John O mond in 1948.1
IgG4- ela ed diseases may be idiopa hic o seconda y o in ec ions, au oimmune diso de s, neoplasms, auma,
adio he apy, su ge ies, and he use o ce ain medica ions such as me hyse gide, b omoc ip ine, e go amine,
me hyldopa, hyd alazine, analgesics, o β-blocke s2,3. The incidence is 1.3 pe 100,000, and i usually a ec s
indi iduals be ween 40 and 60 yea s o age, wi h a sligh male p edominance.4
Idiopa hic e ope i oneal ib osis (IRF) is cu en ly classi ied wi hin a g oup o condi ions known as ch onic
pe iao i is. The h ee main componen s o ch onic pe iao i is a e IgG4- ela ed idiopa hic e ope i oneal
ib osis, IgG4- ela ed abdominal ao i is, and IgG4- ela ed pe ianeu ysmal ib osis.5
The ib osis ypically begins a he le el o he ou h lumba e eb a and hen sp eads owa d he enal hilum,
encasing he u e e s and o he abdominal o gans.6,7 In mos cases, i is de ec ed du ing he e alua ion o acu e
kidney inju y o hype ension.8 U e e al in ol emen is epo ed in 80 o 100% o cases, being he mos
common and se e e complica ion.2
Clinical mani es a ions o he disease may be nonspeci ic, such as a igue, weigh loss, low-g ade e e , and
gene al malaise, which hinde s diagnosis.1 The mos common p esen a ions include poo ly localized pain in
he back, lanks, lowe abdomen, o highs; leg edema; and hyd oneph osis due o u e e al in ol emen . The
mos equen ly a ec ed si es a e he pe iao ic egions, pe iu e e al a eas, and plaque-like masses encasing
he e ope i oneum.5
Diagnosis equi es a combina ion o his opa hology, immunohis ochemis y, and imaging. The p esence o
IgG4-posi i e plasma cells and ib o ic indings a e cha ac e is ic o he IgG4- ela ed o m.9
Compu ed omog aphy (CT) and magne ic esonance imaging (MRI) a e he imaging modali ies o choice,
capable o iden i ying ib o ic e ope i oneal masses o en loca ed an e io o he abdominal ao a and
encasing he u e e s. On CT, ib ous issue enhancemen a ies acco ding o disease s age: mo e ascula ized
wi h good con as enhancemen in ea ly s ages, and showing mild enhancemen in long-s anding ib osis.10
MRI p o ides be e de ini ion o ib o ic issue compa ed o su ounding s uc u es, especially when a -
sa u a ion sequences a e used. PET-CT may be use ul o assess in lamma o y ac i i y and moni o he apeu ic
esponse.2
F om an epidemiological pe spec i e, al hough a e, O mond’s disease is unde diagnosed due o i s
nonspeci ic p esen a ion and he lack o amilia i y o mos clinicians wi h he condi ion. In addi ion o i s
p edominance in middle-aged men, s udies sugges an associa ion wi h o he au oimmune and in lamma o y
diso de s, such as Hashimo o’s hy oidi is, asculi is, and heuma ologic diseases, ein o cing he need o
comp ehensi e clinical e alua ion.2
We p esen a case o O mond’s disease con i med by his opa hology and immunohis ochemis y, associa ed
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wi h se e e enal complica ions.
Case Repo
In ea ly No embe 2023, a 61-yea -old male pa ien was admi ed o he eme gency depa men o a public
hospi al nea his esidence wi h se e e abdominal pain, omi ing, dec eased u ine ou pu , educed appe i e,
and a weigh loss o 20 kg in one mon h. He had expe ienced mild abdominal pain and occasional omi ing
o e he pas 6 mon hs, ha ing sough ca e a he same acili y on wo p e ious occasions, whe e he was ea ed
on an ou pa ien basis wi h symp oma ic medica ion.
Du ing he diagnos ic wo kup a ha hospi al in No embe 2023, he pa ien p esen ed wi h acu e kidney
inju y (AKI) and u ina y ac in ec ion. An ini ial abdominal ul asound e ealed a e ope i oneal mass in
he pe iao ic egion, causing ex insic comp ession o he u e e s. The pa ien unde wen hemodialysis (4
sessions) ollowed by placemen o a double-J ca he e in he igh u e e due o ex insic comp ession om
he mass. He showed imp o emen in enal unc ion and was also ea ed o he u ina y in ec ion. He was
discha ged om ha ins i u ion o con inue ou pa ien ea men .
In Decembe 2023, he p esen ed o ou public hospi al wi h abdominal pain and omi ing e ac o y o
medica ion. A non-con as abdominal CT scan e ealed bila e al u e e ohyd oneph osis up o he mid-u e e
and a lymph node conglome a e (Figu e 1). Labo a o y es s showed anemia (Hb 9.3 g/dL), leukocy osis
(20,450/mm³), hype kalemia (K+ 8.3 mEq/L), ele a ed u ea (204 mg/dL) and c ea inine (3.3 mg/dL), C-
eac i e p o ein (4.5 mg/dL), and u inalysis wi h >1 million leukocy es, 160,000 ed blood cells, and abundan
bac e ia (+++). The pa ien ecei ed clinical ea men o acu e kidney inju y, including ini ial managemen
o hype kalemia wi h conse a i e measu es, ollowed by hemodialysis due o e ac o iness. He was ea ed
wi h an ibio ic he apy o he u ina y ac in ec ion.
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Figu e 1. Abdominal Magne ic Resonance Imaging: I egula solid mass loca ed in he e ope i oneum,
measu ing app oxima ely 8.7 × 2.8 × 5.8 cm, in ol ing ascula s uc u es such as he abdominal ao a and
in e io ena ca a. The lesion causes ma ked bila e al hyd oneph osis. Bila e al enal cys s (Bosniak 1 and 2),
di use hickening o he bladde wall, and an enla ged p os a e a e also obse ed. Findings a e compa ible
wi h a e ope i oneal expansi e/in il a i e p ocess.
Du ing he i s 2 days, he pa ien showed clinical and labo a o y wo sening (p og ession o anemia, inc eased
leukocy osis and CRP, wo sening enal unc ion, and hype kalemia o 8.0). Placemen o a “Shilley” dialysis
ca he e and con inua ion o hemodialysis we e indica ed. Di e en ial diagnoses included lymphomas,
sa comas, and e ope i oneal me as ases om gas oin es inal ca cinomas, among o he s.
In Janua y 2024, a lapa oscopic biopsy o a mesen e ic lymph node was pe o med, and his opa hology
e ealed ch onic in lamma o y p ocess wi h ib osis, ollicula hype plasia, and ch onic eac i e
lymphadeni is, wi h no e idence o neoplasia. Immunohis ochemis y showed ch onic eac i e lymphadeni is,
pe i oneum wi hou neoplasia, and mild ch onic in lamma ion wi h ib osis. A bone ma ow biopsy was also
pe o med, e ealing lymphocy osis wi hou neoplasia.
The double-J ca he e was eplaced, and bila e al neph os omy was pe o med on 01/24/2024. The las
hemodialysis session in his phase was on 01/29/2024. Du ing hospi aliza ion, he pa ien unde wen uppe
gas oin es inal endoscopy and colonoscopy, bo h o which we e un ema kable. In Feb ua y, he pa ien los
he le neph os omy ube and emained dialysis- ee un il Ma ch 2024, when e ac o y hype kalemia equi ed
esump ion o hemodialysis.
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A new biopsy was necessa y and pe o med on 03/20/2024 o he e ope i oneal mass ia lapa oscopy.
In aope a i e ozen sec ion examina ion e ealed p oli e a ion o spindle cells wi hou a ypia, wi h
deposi ion o dense collagen pe mea ed by mild lymphomononuclea in lamma o y in il a e. The inal
immunohis ochemis y esul , ob ained only on 05/07/2024, demons a ed ch onic in lamma ion wi h ib osis,
p esence o IgG4-posi i e plasma cells (up o 14 pe HPF, app oxima ely 70% o plasma cells coun ed),
sugges ing IgG4- ela ed disease (Table 1).
Table 1 - Immunohis ochemis y
An ico pos
Clone
Resul ados
CK AE1/AE2
AE1/AE2
Nega i e
Desmina
D33
Nega i e
Ac ina 1A4
1A4
Posi i e
P o .S100
Policlonal
Nega i e
CD34
QBEND10
Vessels
Be a-Ca enin
BETA-CATENIN-1
Nega i e
Ki-67
MIB-1
5%
CD20
L26
Ra e cells
CD3
Policlonal
Nume osos lin óci os
PAX-5
DAK-PAX-5
Ra e cells
Caldesmon
H-CD
Nega i e
Calponina
CALP
Ra e cells
CD68
KP1
Focal
Fac o XIIIa
EP3372
Posi i e
ALK-1(CD246)
ALK-1
Nega i e
CD138
MI15
Up o 20% o plasma cells pe HPF
AA- e A P O I O
-
IgG4 in 14 plasma cells pe HPF
A e discha ge om ou ins i u ion, he pa ien was e e ed o ano he hospi al wi h special ies in
oncology/hema ology, heuma ology and neph ology. He con inues o unde go ou pa ien hemodialysis h ee
imes a week and has been main ained on p ednisone 20 mg/day o o e one yea , wi h signi ican educ ion
o e ope i oneal ib osis (Figu es 2 (be o e) and Figu e 3 (a e )
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Figu e 2. To al abdominal compu ed omog aphy: Examina ion pe o med a he onse o he condi ion,
p io o ea men , showing bila e al hyd oneph osis and a la ge e ope i oneal mass.
Figu e 3. To al abdominal compu ed omog aphy: Image ob ained a e ini ia ion o co icos e oid he apy,
showing imp o emen o hyd oneph osis and signi ican educ ion in he size o he e ope i oneal mass.
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Discussion
IgG4- ela ed diseases mimic many malignan , in ec ious, and in lamma o y condi ions. His opa hology is
essen ial o diagnosis. The pa hological hallma ks o he disease a e lymphoplasmacy ic in il a ion, s o i o m
ib osis, and obli e a i e phlebi is.
Cu en ly, O mond’s disease, o e ope i oneal ib osis, is classi ied as an IgG4- ela ed disease, which may
be idiopa hic ( wo- hi ds o cases) o seconda y o in ec ions, neoplasms, o au oimmune diseases. I is a
ch onic in lamma o y condi ion cha ac e ized by issue in il a ion wi h IgG4-posi i e plasma cells, ib osis,
and o en ele a ed se um IgG4 le els. The in lamma o y p ocess a ec s he in a enal po ion o he abdominal
ao a and he iliac a e ies, and in il a es in ol ing he u e e s and he in e io ena ca a a e also commonly
obse ed.1
The h ee cen al pa hological ea u es a e lymphoplasmacy ic in il a ion, obli e a i e phlebi is, and s o i o m
ib osis. Lymphocy es and plasma cells a e polyclonal, eosinophils a e o en p esen , and neu ophilic
in il a ion is a e.11 Fib osis is a his opa hological condi ion equi ed o diagnosis, p esen ing wi h a ypically
i egula dis ibu ion and s o i o m pa e n, cha ac e ized by collagen ibe s a anged adially and in e laced
wi hin he issue — a unique pa e n associa ed wi h IgG4- ela ed disease.9
In he idiopa hic o m, he pa hophysiology is no ye ully unde s ood, al hough an au oimmune esponse is
belie ed o be in ol ed. Lymphocy e and plasma cell in il a ion accompanied by a cy okine-media ed
in lamma o y eac ion appea s o be he cen al mechanism o pa hogenesis. Tissue in il a ion by IgG4-
posi i e plasma cells, as well as ele a ed se um le els o his immunoglobulin, may be obse ed in some cases;
howe e , IgG4 p esence is no exclusi e o he disease.1 Se um IgG4 le els a e no su icien ly sensi i e o
speci ic. Plasma IgG4 concen a ions may a y om no mal o ele a ed depending on he si e o in ol emen .
Moni o ing se um IgG4 le els may be use ul o assessing disease ac i i y bu should no be used in isola ion
o he apeu ic decisions.12 Sys ema ic e iews, such as ha o Umeha a e al. (2012)13, emphasize he
impo ance o bioma ke s such as se um IgG4 in helping o di e en ia e his condi ion om o he causes o
e ope i oneal ib osis.
Tissue biopsy is he gold s anda d o diagnosis. Imaging exams such as con as -enhanced compu ed
omog aphy, magne ic esonance imaging, and PET-CT a e impo an o he diagnos ic app oach ac oss
mul iple o gans, and di e en ia ing IgG4- ela ed disease om malignan umo s is essen ial.
The main di e en ial diagnoses in his case include lymphomas, sa comas, sa coidosis, his iocy osis, d ug-
induced ubuloin e s i ial neph i is, idiopa hic memb anous glome uloneph i is, among o he s.
Fi s -line s anda d ea men is glucoco icoids, aimed a a enua ing o hal ing he ch onic in lamma o y
p ocess. The ini ial dose o p ednisolone is gene ally 0.6–1.0 mg/kg pe day.14,15 A e 2 o 4 weeks, he dose
is educed by 5 mg e e y 1 o 2 weeks, acco ding o clinical esponse. A longe cou se o glucoco icoid
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he apy may be necessa y. Clinical mani es a ions gene ally espond apidly, wi h signi ican imp o emen in
in lamma o y lesions and eco e y o a ec ed o gan unc ion. Howe e , e ope i oneal ib osis is less
esponsi e o glucoco icoid he apy, which unde sco es he impo ance o ea ly diagnosis and ea men .16
I pa ien s do no espond o co icos e oids, li e a u e ecommends he use o an ies ogenic agen s such as
amoxi en o immunosupp essan s such as aza hiop ine and cyclophosphamide.17 The ex en o ib osis is a
de e mining ac o in ea men esponse; howe e , in e ac o y cases o in hose showing li le imp o emen
wi h co icos e oids, immunosupp essan s such as i uximab and mycophenola e mo e il a e used as
al e na i es. Zhang e al. (2020)18 epo ed success ul ou comes wi h i uximab in pa ien s wi h IgG4- ela ed
e ope i oneal ib osis.
This case illus a es he ypical p og ession o IgG4- ela ed O mond’s disease, om bila e al u ina y
obs uc ion o ch onic enal ailu e. In pa ien s wi h enal ailu e due o obs uc i e u opa hy, elie ing he
obs uc ion is essen ial o p ese ing enal unc ion. His opa hology and immunohis ochemis y con i med he
diagnosis, unde sco ing he ele ance o de ailed diagnos ic in es iga ion. Al hough ini ial managemen
p o ided pa ial elie o obs uc ion, he pa ien ’s cou se was ma ked by complica ions such as e ac o y
hype kalemia and ecu en in ec ions. The p esen ed case showed a mo e se e e p og ession, wi h es ablished
ch onic enal ailu e, due o delayed diagnosis and ini ia ion o speci ic ea men .
Re ope i oneal ib osis should be conside ed as a mani es a ion o sys emic disease because o i s po en ial
o a ec mul iple o gans. S udies sugges ha ea ly diagnosis and agg essi e managemen can p e en
i e e sible complica ions.19
Conclusion
O mond’s disease, pa icula ly in i s IgG4- ela ed o m, emains a a e and challenging condi ion ha can lead
o se e e complica ions such as ch onic kidney ailu e. Gi en i s abili y o mimic malignan , in ec ious, and
in lamma o y diso de s, i should always be included in he di e en ial diagnosis o e ope i oneal masses o
unce ain o igin. Ea ly ecogni ion and p omp ini ia ion o ea men a e c ucial o p e en i e e sible o gan
damage and imp o e pa ien ou comes.
Re e ences
1. P ůcha M, Kolombo I, Š ádle P. O mond's Disease--IgG4- ela ed Disease. P ague Med Rep.
2015;116(3):181-92. doi: 10.14712/23362936.2015.57
2. Vaglio A, Sal a ani C, Buzio C. Re ope i oneal ib osis. Lance 2006;
367: 241–251.
