Download Full Article – PDF

CAS CLINIQUE / CASE REPORT
IDIOPATHIC RETROPERITONEAL FIBROSIS
PRESENTING AS RECURRENT SCROTAL EDEMA
Report of a Case and Review of the Literature
http://www.lebanesemedicaljournal.org/articles/62-1/case1.pdf
1
2
1
1,2
2
Rawan SAAB , Mansour KHOURY , Claude AFIF , Roula BOU KHALIL , Chadi BECHARA , Albert GHAOUI
2
2
2
1,2
Maroun GHABACH , Wajih RIAKOS, Sleiman OBEID , Jean OBEID , Georges AFTIMOS, Said FARHAT
2
Saab R, Khoury M, Afif C, Bou Khalil R, Bechara C, Ghaoui A,
Ghabach M, Riakos W, Obeid S, Obeid J, Aftimos G, Farhat S.
Idiopathic retroperitoneal fibrosis presenting as recurrent scrotal
edema : Report of a case and review of the literature. J Med Liban
2014 : 62 (1) : 48-53.
Saab R, Khoury M, Afif C, Bou Khalil R, Bechara C, Ghaoui A,
Ghabach M, Riakos W, Obeid S, Obeid J, Aftimos G, Farhat S.
Fibrose rétropéritonéale idiopathique manifestée par un œdème
scrotal récurrent : Cas clinique et revue de la littérature. J Med
Liban 2014 : 62 (1) : 48-53.
ABSTRACT : Primary or idiopathic retroperitoneal
fibrosis, first described by Ormond in 1948, is a rare
and elusive diagnosis, requiring a high level of suspicion. Patients usually present with entrapment of retroperitoneal organs, the most common being the
ureters, causing hydronephrosis and acute kidney
injury.
Here, we present the case of a 56-year-old male
presenting for recurrent and intermittent scrotal
edema. Upon routine laboratory workup, he was
found to have an elevated creatinine level. Imaging
showed encasement of bilateral ureters. Bilateral
ureteral stents were placed with relief of his obstructive uropathy, followed by normalization of creatinine.
The patient later underwent laparoscopic release
of retroperitoneal adhesions. Biopsies taken from the
operative site showed fibroblast proliferation and
elements of acute and chronic inflammation. With
further workup of etiologies being negative, he was
diagnosed with idiopathic retroperitoneal fibrosis.
RÉSUMÉ : La fibrose rétropéritonéale idiopathique,
d’abord décrite par Ormond en 1948, est un diagnostic rare et difficile, nécessitant un haut niveau de suspicion. Les patients se présentent habituellement avec
une compression des organes rétropéritonéaux, les
plus communs étant les uretères, résultant en une
hydronéphrose, et une insuffisance rénale aiguë.
Nous décrivons ici le cas d’un patient de 56 ans qui
s’est présenté pour un œdème scrotal récurrent et intermittent. Le bilan de routine révéla une insuffisance
rénale aiguë. Un CT scan abdominal a montré l’obstruction des deux uretères. L’uropathie obstructive
disparut après l’insertion de deux sondes double J
entraînant la normalisation de la créatinine.
Le patient a subi plus tard une urétérolyse laparoscopique. Les biopsies prélevées ont montré une
prolifération de fibroblastes et d’éléments résultant
d’une inflammation aiguë et chronique. Un bilan plus
approfondi pour exclure d’autres étiologies s’étant
révélé négatif, le diagnostic fut celui d’une fibrose
rétropéritonéale idiopathique.
Keywords : acute renal failure, scrotal edema, inflammatory
syndrome, ureterolysis
Mots-clés : insuffisance rénale aiguë, œdème scrotal,
syndrome inflammatoire, urétérolyse
INTRODUCTION
retroperitoneal fibroplasias, it affects one patient per
200,000 to 500,000 individuals, with no racial predilection, but males being twice to three times more affected
than females [2-3].
Retroperitoneal fibrosis may be primary (Ormond’s
disease) or secondary to malignancy [4-5], medications
[3] or contiguity to inflammation (Table I). It has been
described in 5% of abdominal aortic aneurysm cases as
well, and called “inflammatory aortic aneurysm” [6].
The etiology of the disease is unknown, though it has
been suggested to be immunologically-mediated, due to
association with autoimmune diseases, and the response
to corticosteroids as well as immunosuppressive therapy
[7]. In addition, up to 15% of patients have extra-retroperitoneal fibrotic processes including sclerosing cholangitis, mediastinal fibrosis, fibrosing thyroiditis (Riedl’s
thyroiditis), and fibrotic orbital pseudotumors [8-9].
Here we describe a case of a patient that presented for
Retroperitoneal fibrosis, also known as Ormond’s disease,
was described as a clinical entity in 1948 [1]. It is a rare
collagen vascular disease, characterized by the formation
of plaque-like fibrosis in the retroperitoneal tissue, mainly on the anterior surface of the fourth and fifth lumbar
vertebrae. As a result, the structures are entrapped retroperitoneally, namely the ureters; other involved organs
may be compromised, such as the small bowel, colon and
spinal cord.
Also known as retroperitoneal fasciitis or chronic
1
Saint George University Medical Hospital, Balamand University, Beirut, Lebanon.
2
Rosary Sisters Hospital, Ashrafieh, Beirut, Lebanon.
Corresponding author: Said Farhat, MD. Saint George
University Medical Hospital. Ashrafieh-Beirut. Lebanon.
e-mail: [email protected]
48 Lebanese Medical Journal 2014 • Volume 62 (1)
TABLE I
ACQUIRED ETIOLOGIES OF RETROPERITONEAL FIBROSIS
ACQUIRED ETIOLOGIES
Malignancy
Irradiation
Trauma
Inflammation
Medication
NOTES
Metastatic colon and breast cancer, lymphomas, sarcomas, carcinomas of the lung, cervix, prostate and bladder.
Malignant retroperitoneal fibrosis is distinguished radiologically from retroperitoneal lymph nodes metastasis by
medial displacement of the ureters.
Treatment of pelvic tumors
Peritoneal injury
Chronic retroperitoneal inflammation, mainly due to aortitis (a result of severe atherosclerosis and plaque necrosis)
Beta-blockers, methylsergide and methyldopa
recurrent scrotal edema that was misdiagnosed as inguinal
hernia and was found to have retroperitoneal fibrosis.
CASE REPORT
A 56-year-old male patient presented to our hospital for
a hernia repair.
He had been complaining of intermittent scrotal
edema, without erythema or pain. He presented to his
general practitioner who found no palpable masses upon
examination, and was diagnosed with an inguinal hernia,
for which a non steroidal anti-inflammatory drug was
prescribed, without improvement. He presented for a preoperative examination. Upon evaluation, a hernia could
not be found. A general workup was ordered.
Laboratory results showed renal failure with creatinine
of 3.11 mg/dl (0.50-1.20), urea: 61 mg/dl (10-50), phophorus: 5.9 mg/dl (2.6-5), magnesium: 2.8 (1.6-2.6), hemoglobin: 10.8 g/dl (13-18), hematocrit: 33.5% (38-50%)
and C-reastive protein: 77 mg/l (negative at < 5 mg/l).
An abdomen-pelvis ultrasound was done, showing
bilateral increase in kidney size of 13 cm on the right,
and 13.4 cm on the left with no parenchymal disease;
a
bilateral pelvicalyceal dilatation was noted, without
nephrolithiasis. Doppler ultrasound of the renal vessels
was normal.
A CT scan followed (Fig. 1a), revealing the same
bilateral moderate hydronephrosis, with diffuse infiltration of the retroperitoneal fat surrounding the aorta and
inferior vena cava. There was thickening of the peritoneal
reflections at the level of the renal arteries, reaching the
bifurcation; this infiltration could be responsible for the
bilateral renal dilatation. There were no perivascular adenopathies.
Urgent bilateral double J ureteral stents were inserted
with subsequent normalization of the creatinine within six
days.
Retroperitoneal fibrosis was suspected. A tuberculin
skin test was positive at 15 mm. HIV, hepatitis B and
hepatitis C studies were negative. ANA profile was
found negative, with C3 and C4 levels being normal.
RA test for rheumatoid arthritis was also done, with negative results (< 8 IU/ml).
Erythroid sedimentation rate (ESR) was elevated, at
117 mm (1-13), in addition to a B2 microglobulin level
elevated at 3.96 mg/l (0.8-1.2). Protein electrophoresis
b
FIGURE 1. Abdomen and pelvis CT scan. (a) Patient scan upon presentation, with diffuse infiltration of the retroperitoneal fat
surrounding the aorta and inferior vena cava. (b) CT scan six months later, following steroid therapy.
R. SAAB et al. – Idiopathic retroperitoneal fibrosis
Lebanese Medical Journal 2014 • Volume 62 (1) 49
FIGURE 2
Histopathology of
retroperitoneal fibrosis biopsy
Focal adipose tissue invasion
by dense hyaline fibrosis
and immune-electrophoresis followed, showing absence of
monoclonality. Further workup with urinary 5-hydroxyindoleacetic acid was also negative. Upper endoscopy followed, showing mild active gastritis, and biopsy revealing
the presence of Helicobater pylori. Colonoscopy revealed
no abnormalities.
Following improvement in clinical and laboratory
findings, a laparoscopic bilateral release of retroperitoneal adhesions was performed, with biopsy of the retroperitoneal adipose tissue, which was sent to pathology.
A Mycobacterium tuberculosis polymerase chain reaction (PCR) was done, with negative results.
Histology revealed focal adipose tissue invasion by
dense hyaline fibrosis, with inflammatory exudates composed of plasma cells and few neutrophils (Fig. 2). The
conclusion was compatible with idiopathic retroperitoneal fibrosis.
The patient was treated with steroids (1 mg/kg) for six
months.
A repeat abdomen-pelvis CT scan done six months later
showed significant improvements, with decreased retroperitoneal fat infiltration, and decreased peritoneal thickening (Fig. 1b). Three months later, the double JJ stents
were successfully removed, with creatinine remaining persistently normal for up to four months post removal. A
repeat CT scan was normal as well. The scrotal edema progressively resolved over a period of two months after initiation of steroid therapy. He was kept on low dose steroids
for two years with a maintained response.
PATHOLOGY
Examination reveals dense retroperitoneal plaques, on
the anterior aspect, beginning at the aortic bifurcation,
extending caudally to envelop the aorta and inferior vena
cava, as well as the ureters, bilaterally in 2/3 of cases [10].
The aortic branches may also be involved, namely the
gonadal, renal, celiac and superior mesenteric arteries.
Association with other vasculitic and connective tissue disease has been described, such as ankylosing
spondylitis, polyarteritis nodosa, systemic lupus erythe50 Lebanese Medical Journal 2014 • Volume 62 (1)
matosus and others [9-11].
In the early disease stages, the plaque consists of a
highly vascularized tissue, with collagen deposition and
leukocyte infiltrates (macrophages, polyclonal B cells,
CD4 + T cells). Rarely, vasculitic lesions may be found
in that inflammatory magma [9, 12].
Gradually, the tissue is replaced by fibrosis. The
periaortic infiltrate in Ormond’s disease is comparable to
that of inflammatory abdominal aortic aneurysm.
Severe aortic atherosclerosis and damage to the aortic
media is usually, however not uniformly, present.
The breaching of the media has raised the suggestion
that retroperitoneal fibrosis develops as an autoimmune
response to antigens within the severely atherosclerotic
aorta [13]. In some cases, the immune reaction is triggered by an external agent, and some drugs are implicated, namely methlysergide, methyldopa and ß-blockers
[3]. These agents act as haptens, triggering a hypersensitivity or autoimmune reaction. Methylsergide is a strong
serotonin antagonist, causing a rebound serotonin release following prolonged intake. In patients with carcinoid tumors, again, serotonin or its metabolites has been
found to trigger the immune response.
The association of the disease with familial, as well as
connective tissue diseases suggests a role for genetic factors [14]. As an example, the human leukocyte antigen
(HLA) B27, present in ankylosing spondilitis, has also
been demonstrated in several patients with retro-peritoneal fibrosis [15].
Further investigation also showed an association with
the abundant presence of plasma cells. These cells are
rich in immunoglobulins, specifically the IgG class 4
(IgG4). The findings led investigators to implement the
term “hyperimmunoglobulin G4 syndrome” [16].
PATHOGENESIS
Sixty to 70% of retroperitoneal fibrosis cases are idiopathic [4, 17-18]. Associated etiologic factors comprise
the rest of the conditions leading to secondary disease,
Table I.
R. SAAB et al. – Idiopathic retroperitoneal fibrosis
Some connective tissue diseases have been associated
with retroperitoneal fibrosis [9, 11, 13, 19] (Table II).
CLINICAL FEATURES
Symptoms are vague and nonspecific, leading to a late
diagnosis (6-12 months following the onset of physical
complaints) [9-10].
The peak incidence in adults is around 40-60 years of
age [6]. Presentation in childhood is extremely rare, with
around 33 cases in patients younger than 18 years of age
being reported [19-20].
In around 90% of the cases, the most common symptoms are colicky poorly localized pains in the lower
abdomen, flanks, back or scrotum [21]. Fever, thrombophlebitis and lower limb edema are also reported.
Uncommon nonspecific symptoms include anorexia,
nausea, vomiting and weight loss.
Review of the literature reveals reports of Raynaud’s
phenomenon, limb claudication and urinary symptoms,
such ureteral colics, hematuria and frequency. Another
rare and confusing manifestation of idiopathic retroperitoneal fibrosis is scrotal involvement and edema, resulting from the extrinsic compression of the gonadal
venous and lymphatic systems [22-23], making the presentation of our patient unique.
Patients with autoimmune conditions such as inflammatory bowel disease and sclerosing cholangitis may
have associated retroperitoneal fibrosis.
As such, diagnosis requires a high degree of suspicion.
The disease progresses through two clinical stages: an
early stage, related to the onset of the inflammatory process, and a late stage, due to compression and entrapment of the retroperitoneal organs [21]. Symptoms can
be grouped as early and late, as in table III.
DIAGNOSIS
As previously stated, diagnosis of retroperitoneal fibrosis requires a high index of suspicion.
Confirmatory laboratory results include: elevated
inflammatory markers (such as ESR, CRP and alkaline
TABLE III
EARLY AND LATE SYMPTOMS
Early Symptoms
Dull pain (poorly localized,
back, flank, scrotum
or lower abdomen)
Ureteral obstruction,
hydronephrosis &
renal insufficiency
Venous thromboembolism
(up to 25%)
Lower extremity edema
Late Symptoms
Nausea, vomiting,
anorexia, malaise
Hypertension
Weight loss
Small bowel obstruction /
Pseudo-obstruction
R. SAAB et al. – Idiopathic retroperitoneal fibrosis
TABLE II
CONNECTIVE TISSUE DISEASES AND VASCULITIDES
Associated Connective Tissue Diseases
Ankylosing spondylitis
Scleroderma
Systemic vasculitides
(Wegener’s granulomatosis, polyarteritis nodosa)
Systemic lupus erythematosus
Rheumatoid arthritis
Riedl’s thyroiditis
phosphatase), normocytic normochromic anemia (compatible with chronic inflammatory disease), raised urea
and creatinine levels (in 50 to 75% of cases due to ureteral entrapment), and polyclonal hypergammaglobulinemia (and hyper IgG4 [16]).
Imaging may include intravenous pyelography (IVP),
showing medially deviated and narrowed ureters, with
proximal dilatation and various degrees of hydronephrosis. The middle third portion is the most involved. These
findings, however, are not specific for retroperitoneal
fibrosis [10]. Ultrasound is used as well, revealing clearly marginated and markedly hypoechoic retroperitoneal
infiltrative masses. As for the CT scan, it shows a sharply marginated homogeneous soft tissue mass encasing
the ureters and abdominal aorta, enhancing with contrast. The inferior vena cava may be encased between the
renal hila and the sacral promontory [10]: Magnetic resonance imaging (MRI) is the diagnostic method of
choice, revealing the chronic retroperitoneal mass with
intermediate T1 signal intensity, and low T2 intensity.
High T2 signal intensity may be due to more acute
active disease or secondary retroperitoneal fibrosis due
to malignancy.
Despite the advances in imaging techniques, a biopsy
is required for histological confirmation, and differentiation of idiopathic retroperitoneal fibrosis from secondary retroperitoneal fibrosis due to other causes.
MANAGEMENT
Goals of management are to preserve renal function,
prevent organ involvement and relieve symptoms, which
require integration of both surgical and nonsurgical
therapies.
Empirical treatment regimen includes corticosteroids
and immunosuppressive therapies.
Corticosteroids offer beneficial effects through antiinflammatory actions, and inhibition of fibrotic tissue
maturation. In 2002, Van Bommel [24] reported a satisfactory outcome with corticosteroids, however, despite
their successes, the use of these agents as first-line therapy is still controversial. A standard protocol is prednisolone 40-60 mg/day, tapered to 10 mg/day within two
to three months, and discontinued within one to two years.
Combining corticosteroids with azathioprine or peniLebanese Medical Journal 2014 • Volume 62 (1) 51
cilliamine may be useful in patients displaying signs of
inflammation, as witness by raised levels of ESR and
CRP, positive ANA results and positive positron emission
tomography (PET) findings [25].
Combination with ureteral surgery showed decrease
in restenosis, however the response may vary, and very
high levels of corticosteroids may be required, and side
effects outweigh the benefits of treatment [26].
Tamoxifen, a nonsteroidal antiestrogen, was used in
1991 by Clarke et al. [27] in the treatment of retroperitoneal fibrosis. Though the mechanism is not entirely
clear, tamoxifen increases the synthesis of the inhibitory
growth factor transforming growth factor-ß (TGF-ß),
which may decrease the size of the fibrotic mass [28]. In
a randomized, open-label, controlled trial, Vaglio et al.
[29] compared the efficacy of prednisone with that of
tamoxifen with a variable protocol.
Compared with steroids, tamoxifen has lower adverse
effects, however, the increased risk of thromboembolism
and ovarian cancer should be carefully considered [30].
Mycophenolate mofetil was also studied, acting by
blocking the proliferation of T and B lymphocytes. In
2008, Swartz et al. [31] conducted a trial using highdose prednisone and mycophenolate mofetil (1000 mg
bid) as steroid-sparing therapy, discontinued within 6 to
12 months. Recent studies show radiologic regression of
the mass, with improvement in outcome.
When steroid therapy has failed to be tapered, azathioprine has been used as a steroid-sparing treatment. Cogan
and Fastrez [32] used 150 mg/day of azathioprine for six
weeks in a patient who recurred following discontinuation
of steroid therapy, with a significant response.
Experimental therapies with immunosuppressive
agents such as methotrexate, cyclosporine and cyclophosphamide have showed promising results. Future
steroid-sparing options could include biologic disease
modifying agents such as tumor necrosis alpha (TNF α)
inhibitors, and anti-CD20 drugs [33].
Surgical management confirms the diagnosis. It is
required in severe organ encasement, mainly in ureteral
involvement. Release of retroperitoneal adhesions is the
procedure of choice, but is reserved for moderate to
severe manifestations, due to the occurrence of post surgical adhesions [34-37].
CONCLUSION
Retroperitoneal fibrosis is a rare disease, occurring as
primary Ormond’s disease, or secondary to many etiological factors. Diagnosis is based on a high index of
suspicion, as symptoms are vague, and the disease is
insidious. Treatment is a combination of medical and
surgical therapy, and future immune modulators and
disease-modifying agents are promising. Because of
asymptomatic recurrences of retroperitoneal fibrosis,
patients require close follow-up.
SOURCES OF SUPPORT (Funds, grants, drugs): none.
52 Lebanese Medical Journal 2014 • Volume 62 (1)
REFERENCES
1. Ormond JK. Bilateral ureteral obstruction due to envelopment and compression by an inflammatory retroperitoneal process. J Urol 1948; 59: 1072-9.
2. Kottra JJ, Dunnick NR. Retroperitoneal fibrosis. Radiol
Clin North Am Nov 1996; 34 (6): 1259-75.
3. Higgins PM, Aber GM. Idiopathic retroperitoneal fibrosis – an update. Dig Dis 1990; 8 (4): 206-22.
4. Lepor H, Walsh PC. Idiopathic retroperitoneal fibrosis.
J Urol Jul 1979; 122 (1): 1-6.
5. Thomas MH, Chisholm GD. Retroperitoneal fibrosis
associated with malignant disease. Br J Cancer Nov
1973; 28 (5): 453-8.
6. Von Fritschen U, Maltzfeld E, Clasen A, Kortmann H.
Inflammatory abdominal aortic aneurysm: a postoperative course of retroperitoneal fibrosis. J Vasc Surg 1999;
30: 1090-8.
7. Carton RW, Wong R. Multifocal fibrosclerosis manifested
by vena caval obstructions and associated with vasculitis.
Ann Intern Med Jan 1969; 70 (1): 81-6.
8. Izzedine H, Servais A, Launay-Vacher V, Deray G.
Retroperitoneal fibrosis due to Wegener’s granulomatosis: a misdiagnosis as tuberculosis. Am J Med Aug 1
2002; 113 (2): 164-6.
9. Zabetakis PM, Novich RK, Matarese RA, Michelis MF.
Idiopathic retroperitoneal fibrosis: a systemic connective
tissue disease? J Urol Jul 1979; 122 (1): 100-2.
10. Monev S. Idiopathic retroperitoneal fibrosis: Prompt diagnosis preserves organ function. Cleveland Clinic
Journal of Medicine 2002; 69 (2): 160-6.
11. Littlejohn JO, 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.
12. Parums DV, Choudhury RP, Shields SA, Davies AH.
Characterisation of inflammatory cells associated with
“idiopathic retroperitoneal fibrosis”. Br J Urol Jun 1991;
67 (6): 564-8.
13. Ramshaw AL, Roskell DE, Parums DV. Cytokine expression in aortic adventitial inflammation associated with
advanced atherosclerosis (chronic periaortitis). J Clin
Pathol 1994; 47: 721-7.
14. Hatsiopoulou O, Irving S, Sharma SD. Retroperitoneal
fibrosis in 2 brothers. J Urol Jan 2001; 165 (1): 182.
15. Astudillo L, Alric L, Jamard B, Laroche M. [Retroperitoneal fibrosis in an HLA-B27-positive patient]. Rev
Med Interne Dec 1999; 20 (12): 1149-50.
16. Neild GH, Rodriguez-Justo M, Wall C, Connolly JO.
Hyper-IgG4 disease: report and characterisation of a new
disease. BMC Med Oct 6 2006; 4: 23.
17. van Bommel EF, van Spengler J, van der Hoven B,
Kramer P. Retroperitoneal fibrosis: report of 12 cases
and a review of the literature. Neth J Med Dec 1991; 39
(5-6): 338-45.
18. Buff DD, Bogin MB, Faltz LL. Retroperitoneal fibrosis.
A report of selected cases and a review of the literature.
N Y State J Med Sep 1989; 89 (9): 511-16.
19. Gilkeson GS, Allen NB. Retroperitoneal fibrosis. A true
connective tissue disease. Rheum Dis Clin North Am Feb
1996; 22 (1): 23-38.
20. Buff DD, Bogin MB, Faltz LL. Retroperitoneal fibrosis.
A report of selected cases and a review of the literature.
N Y State J Med Sep 1989; 89 (9): 511-16.
R. SAAB et al. – Idiopathic retroperitoneal fibrosis
21.
22.
23.
24.
25.
26.
27.
28.
29.
Koep L, Zuidema GD. The clinical significance of retroperitoneal fibrosis. Surgery Mar 1977; 81 (3): 250-7.
Ward CS, Dundas DD, Dow J, Dormandy JA, Shearer RJ.
Scrotal swelling due to perianeurysmal fibrosis. Br J
Urol 1988 Jun; 61 (6): 536-8.
Schulte-Baukloh H, Knispel HH, Dallenbach F, Miller K.
Scrotal involvement with idiopathic retroperitoneal
fibrosis. J Urol 1999 Nov; 162 (5): 1692-3.
van Bommel EF. Retroperitoneal fibrosis. Neth J Med Jul
2002; 60 (6): 231-42.
Harreby M, Bilde T, Helin P. Retroperitoneal fibrosis
treated with methylprednisolone pulse and disease-modifying antirheumatic drugs. Scand J Urol Nephrol Sep
1994; 28 (3): 237-42.
Wagenknecht LV, Hardy JC. Value of various treatments
for retroperitoneal fibrosis. Eur Urol 1981; 7 (4): 193200.
Clark CP, Vanderpool D, Preskitt JT. The response of retroperitoneal fibrosis to tamoxifen. Surgery. Apr 1991;
109 (4): 502-6.
Frankart L, Lorge F, Donkier J. Tamoxifen for retroperitoneal fibrosis. Postgrad Med J 1997; 73: 653-4.
Vaglio A, Palmisano A, Alberici F et al. Prednisone versus tamoxifen in patients with idiopathic retroperitoneal
fibrosis: an open-label randomised controlled trial.
Lancet Jul 23 2011; 378 (9788): 338-46.
R. SAAB et al. – Idiopathic retroperitoneal fibrosis
30.
31.
32.
33.
34.
35.
36.
37.
Monev S, Remer E, Calabrese L. Effect of tamoxifen in
patients with idiopathic retroperitoneal fibrosis. Arthritis
Rheum 1998; 41 (suppl): 419.
Swartz RD, Lake AM, Roberts WW, Faerber GJ, Wolf JS
Jr. Idiopathic retroperitoneal fibrosis: a role for mycophenolate mofetil. Clin Nephrol Apr 2008; 69 (4):
260-8.
Cogan E, Fastrez R. Azathioprine. An alternative treatment for recurrent idiopathic retroperitoneal fibrosis.
Arch Intern Med Apr 1985; 145 (4): 753-5.
McDougal WS, MacDonell RC. Treatment of idiopathic
retroperitoneal fibrosis by immunosuppression. J Urol
1991; 145: 112-14.
Kerr WS, Suby HI, Vickery A, Fraley E. Idiopathic retroperitoneal fibrosis: clinical experiences with 15 cases,
1956-1967. J Urol May 1968; 99 (5): 575-84.
Kavoussi LR, Clayman RV, Brunt LM, Soper NJ. Laparoscopic ureterolysis. J Urol Feb 1992; 147 (2): 426-9.
Fugita OE, Jarrett TW, Kavoussi P, Kavoussi LR. Laparoscopic treatment of retroperitoneal fibrosis. J Endourol
Oct 2002; 16 (8): 571-4.
Styn NR, Frauman S, Faerber GJ, Wolf JS Jr. University
of Michigan surgical experience with ureterolysis for
retroperitoneal fibrosis: a comparison of laparoscopic
and open surgical approaches. Urology Feb 2011; 77 (2):
339-43.
Lebanese Medical Journal 2014 • Volume 62 (1) 53