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Retroperitoneal fibrosisAn extraarticular manifestation of ankylosing spondylitis.

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Arthritis & Rheumatism (Arthritis Care & Research)
Vol. 47, No. 2, April 15, 2002, pp 210 –214
DOI 10.1002/art1.10267
© 2002, American College of Rheumatology
CASE REPORT
Retroperitoneal Fibrosis: An Extraarticular
Manifestation of Ankylosing Spondylitis
CLAIRE M. A. LEBLANC,1 ROBERT D. INMAN,2 PETER DENT,3 CHARLES SMITH,1 PAUL BABYN,1
RONALD M. LAXER1
Introduction
Idiopathic retroperitoneal fibrosis (RPF) was first described as a clinical entity in 1948 (1). Most patients with
RPF are 30 – 60 years old, and very few pediatric cases
have been reported. Although in most cases the RPF is
idiopathic, it may also be secondary to underlying infection, malignancy, drugs, radiation, trauma, or surgery. In
most patients, fibrosis is confined to the retroperitoneal
region of the lower lumbar spine; however 15% have fibrosis in other organs (2). Spondylarthritis, which may
also be complicated by extraarticular fibrosis, has been
reported in patients with RPF. We present a case of a
7-year-old boy who originally presented with RPF and was
later diagnosed as having spondylarthritis, and discuss the
relevance of this association.
Case report
A 19-month-old boy presented with a 6-week history of
severe nocturnal leg pain, reduced ambulation, and morning stiffness of 2 hours duration. On examination, there
was limited flexion, stress pain of both hips, and tight
tendo Achillis, but joint effusions were not detected. Laboratory tests showed hemoglobin (Hgb) 97 gm/dl, Westergren erythrocyte sedimentation rate (ESR) 29 mm/hour,
and negative antinuclear antibody (ANA) and rheumatoid
factor (RF). Chest, abdominal, and hip radiographs and
abdominal ultrasound were normal. The patient was
treated with 70 mg/kg/day of aspirin for possible juvenile
rheumatoid arthritis, which only improved the stiffness.
1
Claire M. A. LeBlanc, MD, FRCPC, Charles Smith, MD,
FRCPC, Paul Babyn, MD, FRCPC, Ronald M. Laxer, MD,
FRCPC: University of Toronto, and The Hospital for Sick
Children, Toronto, Ontario, Canada; 2Robert D. Inman, MD,
FRCPC: University Health Network, University of Toronto,
Ontario, Canada; 3Peter Dent, MD, FRCPC: McMaster University Medical Centre, Hamilton, Ontario, Canada.
Address correspondence to Claire M. A. LeBlanc, MD,
FRCPC, Division of Rheumatology, Room 8253 Elm Wing,
The Hospital for Sick Children, 555 University Avenue,
Toronto, Ontario M5G 1X8, Canada. E-mail: claire.leblanc@
sickkids.on.ca.
Submitted for publication March 18, 2001; accepted in
revised form October 24, 2001.
210
AND
At 5 years of age, he developed worsening hip pain and
stiffness. Examination showed hip and knee flexion contractures, reduced back flexion, and warm, non-tender ankles, but no effused joints. The Hgb was 85 gm/dl, platelet
count 613,000/mm3, and ESR 42 mm/hour. The results of
iron studies, albumin, creatinine kinase, urinalysis, and
HLA–B27 were either normal or negative. Radiographs of
the lumbosacral spine, sacroiliac joints, and hips were
reported as normal. Treatment consisted of an injection of
triamcinolone hexacetonide in both hips that initiated a
dramatic, but short-lived improvement. Subsequently,
naproxen (18 mg/kg/day) and oral prednisone (0.6 mg/kg
every other day) were initiated.
Two years later the boy developed lower abdominal
pain, nausea, vomiting, and anorexia. Hip pain and stiffness recurred despite treatment with prednisone 0.25
mg/kg every 48 hours. Examination revealed a distended
abdomen and palpable left kidney. Laboratory evaluation
revealed the following findings: Hgb 91 gm/dl (normal
120 –160), ESR 45 mm/hour (normal 1–10), urea 7.6 mmol/
liter (normal 2.9 –7.1), creatinine 94 mol/liter (normal 10 –
80), and normal urinalysis. Abdominal ultrasound and
intravenous pyelogram showed severe bilateral hydronephrosis, with minimal vesicoureteral reflux on voiding
cystourethrogram. Extensive fibrotic tissue extending from
the pelvic brim to the lower pole of kidneys was seen on
abdominal computerized tomography (CT) and at laparotomy (Figure 1A). Subsequently, a right ureterolysis with
omental wrap and left ureter bypass surgery using the
Boari flap procedure were performed. Microscopic examination revealed dense collagenized fibrosis with chronic
inflammatory cells, compatible with retroperitoneal fibrosis (Figure 1B).
At 8 years of age the patient developed low back pain
and stiffness. Marked hip and knee flexion contractures,
and reduced hip and back range of motion were noted, as
well as bilaterally tight tendo Achillis requiring heel cord
and plantar fascia releases. The neurologic examination
was normal. Abdominal CT showed improvement of hydronephrosis and resolution of RPF; the hips appeared
normal. Magnetic resonance imaging (MRI) of the spine
and spinal cord was normal. Treatment with oral prednisone (15 mg/day) was required to control symptoms and
Retroperitoneal Fibrosis and Spondylitis
211
Figure 1. A, Computerized tomography scan at the level of the lower kidneys shows asymmetric ureteral wall thickening, and the start of
a retroperitoneal mass (arrow) on the left. B, The ureteric lumen (U) is lined by urothelium, supported by a wall of smooth muscle (S).
Increased amounts of dense collagen are seen in the subepithelial region (C1) as well as in the interstitial regions external to the muscle
layer (C2) (Masson’s trichrome stain).
any reduction in dosage caused recurrence of leg and back
pains.
At 12 years of age, azathioprine was added to the treatment regimen to control persistent symptoms, and growth
hormone injections for short stature were started. Six
months later, he developed severe nocturnal leg, hip, and
flank pain, ascending low back pain, and morning stiffness
of 4 hours duration. Height and weight were charted at less
than the third percentile. A Schöber test of 12.5 cm, and
reduced range of motion of wrists, ankles, knees, hips, and
back were noted. No joint effusions were found.
Two years subsequently the patient exhibited reduced
range of motion in the wrists, hips, and left elbow, and
both ankles were effused. The pelvic radiographs revealed
bilateral widening and sclerosis of sacroiliac (SI) joints
(Figure 2). MRI demonstrated erosive disease of SI joints.
Treatment with indomethacin was reinitiated, and in followup assessments symptomatic improvement was noted;
however, the patient continues to have back, leg, and ankle
pain.
Literature review
We performed a MEDLINE search of the English and
French language literature from 1966 –2000 (3–16) and
found 15 reported cases of RPF and spondylarthritis (Table
1). Males were more commonly affected (2:1 ratio). The
mean age at diagnosis of RPF or spondylarthritis was 43
years; in all but one case spondylarthritis preceded or
occurred simultaneously with RPF. In patients in whom it
was reported, axial arthritis occurred in 10 of 14 patients,
and peripheral arthritis in 2 of 9 patients. HLA–B27 was
present in 7 of 12 patients tested; 60% of those who were
HLA–B27–negative were female. Treatment of the RPF
was surgical in 3 of 15 (20%), involved systemic corticosteroids or irradiation in 7 of 15 (47%), and both surgical
and medical in 4 of 15 (27%). Outcome was significantly
improved in 10 of 15 patients, 2 had persistent arthralgia,
2 had recurrent RPF, and 1 died of acute renal failure
secondary to hypersensitivity vasculitis. The longest followup was 5 years.
Discussion
Figure 2. Coronal view of the sacroiliac joints with extensive iliac
greater than sacral sclerosis and sacroiliac irregularities bilaterally.
In this report, we describe a 12-year-old boy diagnosed as
having ankylosing spondylitis (AS) and RPF. Extraarticular fibrotic manifestations are prominent in patients with
ankylosing spondylitis. Aortic insufficiency occurs in approximately 5% of affected individuals and is secondary
to inflammation and fibrosis of the vasa vasorum (17).
Pulmonary fibrosis occurs in about 1% of patients, especially those with long-standing disease. We propose that
RPF should be considered as another extraarticular fibrotic
manifestation of AS.
Retroperitoneal fibrosis is a rare disorder in which fibrous tissue is deposited in the lower lumbar retroperitoneal region. It commonly involves the ureters, aorta, vena
cava, and common iliac vessels. Presenting features may
include back pain, and constitutional symptoms such as
fatigue, weight loss, and low grade fever. If allowed to
progress, compression of the ureters, lymphatics, and
3
1961
4
1965
5
1969
6
1977
7
1981
8
1981
9
1982
10
1983
10
1983
11
1985
12
1988
13
1989
14
1992
15
1993
16
1999
NA ⫽ not available; SP ⫽ spondylarthritis; yrs ⫽ years; RPF ⫽ retroperitoneal fibrosis; SI ⫽ sacroiliac; L ⫽ left; R ⫽ right; B ⫽ bilateral; RF ⫽ rheumatoid factor; ANA ⫽ antinuclear antibodies; NSAID ⫽
nonsteroidal antiinflammatory drug; ASA ⫽ aspirin; U ⫽ ureterolysis; S ⫽ corticosteroids; I ⫽ irradiation.
Sex
F
M
M
M
M
M
M
F
F
M
F
F
M
M
M
Race
NA
White White
NA
White
NA
NA
White
White
White
NA
NA
NA
NA
NA
Age at SP
43
51
64
54
25
32
60
28
34
31
72
34
28
36
55
diagnosis
(yrs)
Age at RPF
43
51
64
54
24
32
60
28
34
51
72
64
63
52
54
diagnosis
(yrs)
X-ray SI
L
NA
B
B
R
B
L
B
B
NA
B
B
B
B
Normal
X-ray spine
NA
pos
neg
pos
neg
pos
neg
neg
pos
NA
neg
pos
pos
pos
neg
Arthritis
NA
pos
neg
pos
neg
neg
pos
pos
pos
pos
neg
pos
pos
pos
pos
(axial)
Arthritis
NA
neg
neg
neg
pos
neg
neg
neg
neg
NA
pos
NA
NA
NA
NA
(peripheral)
HLA–B27
NA
NA
NA
pos
pos
neg
pos
neg
neg
pos
neg
pos
neg
pos
pos
RF/ANA
NA
NA
NA
NA
NA/neg NA/neg NA/neg
neg/neg
neg/NA
NA/NA
NA/NA NA/NA neg/NA
NA/pos
NA
Treatment
—
—
—
—
—
NA
—
NSAID
ASA
Indocid
—
I
NA
Indocid Phenylbuta(SP)
zone
Treatment
U
—
U
S
I&S
S
U&S
S
S
U
U&S
S
U&S
S
I
(RPF)
Outcome
Improved Died Improved Improved Improved Relapse Improved Arthralgia Arthralgia Improved Improved Relapse Improved Improved Improved
⫻3
⫻ 3 yrs
⫻ 1 yr
⫻ 2 yrs at 5 yrs ⫻ 6 weeks
⫻ 6 month ⫻ 4 yrs
⫻ 2 yrs ⫻ 8 month ⫻ 12 month
month
Reference
Table 1. Reported patients with retroperitoneal fibrosis and spondylitis
212
LeBlanc et al
Retroperitoneal Fibrosis and Spondylitis
blood vessels can result in renal failure, edema, ischemia,
and venous thrombosis, respectively. Up to 15% of patients with RPF have additional fibrosis outside the retroperitoneum including sclerosing cholangitis, constrictive
pericarditis, superior vena cava syndrome, pulmonary fibrosis, fibrosing thyroiditis, orbital/sinus pseudotumors,
and possibly Dupuytren’s contracture (2). Although RPF
has been precipitated by medications, trauma, infection,
and malignancy, 70% of cases are idiopathic. Some adult
patients with RPF have atherosclerotic disease with
perivascular fibrosis (18). Others may have concurrent inflammatory or autoimmune diseases such as systemic lupus erythematosus, scleroderma, glomerulonephritis, panniculitis, and AS. With appropriate early treatment the
prognosis for patients with RPF is generally excellent (2).
Although the clinical report cited above suggests a relationship between RPF and AS, limitations in our current
knowledge of the pathogenesis of each condition restrict
our ability to transfer mechanistic models to the alternative disease. In both cases, there is a paucity of information
on the target tissues, reflecting the relative inaccessibility
of these sites. Hughes and Buckley reported that the cellular infiltrate in RPF was predominantly a macrophagelike cell that expressed CD4, CD11, CD25, CD45, CD68,
and HLA–DR (19). The cellular infiltrate in SI joint inflammation is a mixed population, but certainly there are cells
which similarly express CD4 and CD45 (20). Ramshaw et
al (21) reported on the cytokine gene expression of adventitial inflammation in a precursor of RPF. They observed
abundant interferon-␥, interleukin-(IL)1␣, IL-2, IL-2 receptor, and IL-4 polymerase chain reaction products, whereas
little tumor necrosis factor (TNF)␣ was detected. Braun et
al examined cytokine expression in SI joint biopsies in
patients with AS (20) and found TNF-␣ and transforming
growth factor ␤ expressed at sites of inflammation. It is
difficult to resolve whether there is sufficient information
on cytokines to develop treatment strategies at this point.
Certainly the results to date with anti-TNF therapy in AS
have been encouraging (22). Although cyclosporin (23),
mycophenolate mofetil (24), and tamoxifen (25) have been
used in RPF, there is certainly a need for innovative approaches to treatment, and the clinical association with AS
may alone warrant a trial of anti-TNF therapy in this
disease.
The role that HLA–B27 may play in the pathogenesis of
RPF may have to await the clarification of B27 in the
pathogenesis of AS, for which there is no consensus at the
present time. The canonical role for HLA–B27 is a restricting element in mounting a CD8 –positive CTL response to
self or foreign antigens. The argument from the cellular
immunology perspective comes down to characterizing
the “arthritogenic” peptide, which initiates and perpetuates the B27-orchestrated immune response. The identity
of such peptides has proved elusive, at least in terms of
establishing pathogenicity. The link between B27 and infectious triggers has also invoked molecular mimicry in a
pathogenic role, suggesting that shared homology between
B27 and certain pathogens may lead to an autoimmune
response in the course of host immune response to such
cross-reactive microbial antigens. This theory, however,
has not been rigorously proven (26). Any or all of the
213
putative roles for B27 in AS could have relevance for the
pathogenesis of RPF, but further studies on the immunology of the latter will be needed to answer this question.
RPF has rarely been reported in childhood. Hip or gluteal pain is more common in this age group than adults
(27). As in adults, RPF is idiopathic in most cases although
associations with lupus, Henoch-Schönlein purpura, relapsing polychondritis, osteomyelitis, and juvenile
chronic arthritis have been documented (28). To our
knowledge, this is the first reported pediatric case of RPF
with associated sacroiliitis. Rheumatologists should be
aware of the association of RPF and spondylarthropathies
in order to make an accurate diagnosis and institute early
treatment.
REFERENCES
1. Ormond JK. Bilateral ureteral obstruction due to envelopment
and compression by an inflammatory retroperitoneal process.
J Urol 1948;59:1072–9.
2. Amis ES. Retroperitoneal fibrosis. AJR Am J Roentgenol 1991;
157:321–9.
3. Symmons RE, Dahlin DC, Engel S. Idiopathic retroperitoneal
fibrosis. Obstet Gynecol 1961;18:591– 6.
4. Reidbord HE, Hawk WA. Idiopathic retroperitoneal fibrosis
and necrotising vasculitis. Cleve Clin Q 1965;32:19 –27.
5. Hissong SL, Freimanis AK. Retroperitoneal fibrosis: extraperitoneal lesions (report of 3 unusual areas of involvement). AJR
Am J Roentgenol 1969;107:776 – 84.
6. De Meulder A, Dutrieux JL. Rapport d’un cas de fibrose retroperitoneal associee a une spondylarthrite ankylosante. Louvain Med 1977;96:387–94.
7. Littlejohn GO, Keystone EC. The association of retroperitoneal fibrosis with systemic vasculitis and HLA–B27: a case
report and review of the literature. J Rheumatol 1981;8:665–9.
8. Vital-Durand D, Rouhier D, Brette R. Association fibrose retroperitoneale-spondylarthrite ankylosante: une nouvelle observation. Ann Med Interne (Paris) 1981;132:200 –1.
9. Thorel JB, Toulet R, Lenormand JP, Schmitt B. Fibrose retroperitoneale idiopathique chez un sujet porteur d’un antigene
HLA–B27. Revue du Rheumatisme 1982;49:891–5.
10. Goldbach P, Mohsenifar Z, Salick AI. Familial mediastinal
fibrosis associated with seronegative spondylarthropathy. Arthritis Rheum 1983;26:221–5.
11. Solomon SD, Maurer KH. The association of retroperitoneal
fibrosis and ankylosing spondylitis. J Rheumatol 1985;12:818.
12. Wicks IA, Robertson MR, Murnahan GF, Bertouch JV. Idiopathic retroperitoneal fibrosis presenting with back pain.
J Rheumatol 1988;15:1572– 4.
13. Reilly PA, Moran CJ. Retroperitoneal fibrosis in radiotherapy
treated ankylosing spondylitis [letter]. Br J Rheumatol 1989;
28:273– 4.
14. De la Iglesia Martinez F, Grana Gil J, Gomez Veiga F, Rodiguez
Garcia J, Gomez Rodriguez N, Atanes Sandoval A. The association of idiopathic retroperitoneal fibrosis and ankylosing
spondylitis. J Rheumatol 1992;19:1147–9.
15. Azias I, Roblot P, Barrier J, Debiasis F, Becq-Giraudon B,
Bontoux D. Fibrose retroperitoneale idiopathique et spondylarthrite ankylosante. Rev Rheum (Engl Ed) 1993;321– 4.
16. Astudillo L, Alric L, Jamard B, Laroche M. Fibrose retroperitoneale chez un patient HLA–B27 positif. Rev Med Interne
1999;20:1149 –50.
17. Bullock N. Idiopathic retroperitoneal fibrosis [editorial]. BMJ
1988;297:240 –1.
18. Eulderink F. Pathology of ankylosing spondylitis. In: Rahn
MA, editor. Ankylosing spondylitis and related spondylarthropathies. 1990;4:497– 684.
19. Hughes D, Buckley PJ. Idiopathic retroperitoneal fibrosis is a
macrophage-rich process: implications for its pathogenesis
and treatment. Am J Surg Pathol 1993;17:482–90.
214
20. Braun J, Bollow M, Neure L, Seipelt E, Seyrekbasan F, Herbst
H, et al. Use of immunohistologic and in situ hybridization
techniques in the examination of sacroiliac joint biopsy specimens from patients with ankylosing spondylitis. Arthritis
Rheum 1995;38:499 –505.
21. Ramshaw AL, Roskell DE, Parums DV. Cytokine gene expression in aortic adventitial inflammation associated with
chronic periaortitis (advanced atherosclerosis). J Clin Pathol
1994;47:721–7.
22. Stone M, Salonen D, Lax M, Payne U, Lapp V, Inman RD.
Clinical and imaging correlates of response to treatment with
infliximab in patients with ankylosing spondylitis. J Rheumatol 2001;28:1605–14.
23. Marzano A, Trapani A, Leone N, Acts GC, Rizzetto M. Treat-
LeBlanc et al
24.
25.
26.
27.
28.
ment of idiopathic retroperitoneal fibrosis using cyclosporin.
Ann Rheum Dis 2001;60:427– 8.
Grotz W, von Zedtwitz I, Andre M, Shollmeyer P. Treatment
of retroperitoneal fibrosis by mycophenolate mofetil and corticosteroids. Lancet 1998;352:1195.
Dedeoglu F, Rose CD, Athreya BH, Conard K, Grissom L,
Magnusson M. Successful treatment of retroperitoneal fibrosis
with tamoxifen in a child. J Rheumatol 2001;28:1693–5.
Albert LJ, Inman RD. Molecular mimicry and autoimmunity.
N Engl J Med 1999;341:2068 –74.
Snow BW, Garrett RA. Retroperitoneal fibrosis in children
(eosinophilic and idiopathic). Urology 1984;23:569.
Sherman C, Winchester P, Brill PW, Mininberg D. Childhood
retroperitoneal fibrosis. Pediatr Radiol 1988;18:245–7.
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