CASE REPORT

Spondyloarthropathy and retroperitoneal fibrosis:a case report

Daniel Wendling*, Éric Toussirot, Fabrice MichelService de Rhumatologie, CHU Jean Minjoz, Boulevard Fleming, 25030 Besançon cedex, France

(Submitted for publication June 11, 2001; accepted in revised form September 11, 2001)

Summary – Retroperitoneal fibrosis is an uncommon inflammatory condition in which the retroperitonealtissue is transformed into a tight sheet of fibrous tissue. It can occur in association with various diseases,including rheumatic conditions. We report a new case in a 66-year-old man with spondyloarthropathy. Ninesimilar cases have been reported. Axial manifestations predominated, and half the patients carried theHLA-B27 antigen. The relation between retroperitoneal fibrosis and spondyloarthropathy remains contro-versial. Joint Bone Spine 2002 ; 69 : 334-7. © 2002 Éditions scientifiques et médicales Elsevier SAS

retroperitoneal fibrosis / spondyloarthropathy

Retroperitoneal fibrosis is an uncommon and initiallyinflammatory condition. Low back pain is among thesymptoms and can lead the patient to seek advice froma rheumatologist. Retroperitoneal fibrosis can occur inassociation with other fibrosing disorders [1] or, lessoften, with rheumatic diseases. We report a new case ofretroperitoneal fibrosis in a patient with spondyloarthr-opathy.

CASE REPORT

This patient born in 1933 had an unremarkable per-sonal and family history and was not taking medica-tions on a regular basis. He presented in 1996 with atwo-year history of intermittent oligoarthritis affectingthe hands, shoulders, and knees. The flares consistentlyresolved within a few days of initiation of a nonsteroidalantiinflammatory agent or glucocorticoid. There wereno other symptoms. Neither was there a history of

gastrointestinal manifestations, conjunctivitis or siccasyndrome, Raynaud’s phenomenon, or skin lesions.However, he reported episodes of inflammatory pain inthe low back and buttocks between 25 and 30 years ofage. Range of motion of the lumbar spine was slightlyrestricted (Schöber’s index, 10/13). Findings were nor-mal from auscultation of the heart, all the peripheralpulses were felt, and blood pressure was 140/80. Noerosions were visible on radiographs of the hands.Radiographs of the pelvis showed fusion of both sacro-iliac joints (figure 1).

Laboratory tests disclosed evidence of inflammation:the erythrocyte rate was 40 mm/h and the C-reactiveprotein level was 54 mg/L. Serum levels of creatinineand uric acid were normal. IgA levels were normal andtests for autoantibodies were negative. The HLA pat-tern was B35, B60, DR16, and DR13.

Methotrexate in a dosage of 10 mg/week provided aremission of the joint manifestations after two years.The methotrexate was stopped. One year later, inAugust 1999, he experienced an episode of pain in thelow back and abdomen. An ultrasound scan showed a

* Correspondence and reprints.E-mail address: daniel.wendling@ufc-chu.univ-fcomte.fr (D. Wendling).

Joint Bone Spine 2002 ; 69 : 334-7© 2002 Éditions scientifiques et médicales Elsevier SAS. All rights reserved

S1297319X02004049/SCO

small aneurysm of the abdominal aorta less than 25 mmin diameter and a circumferential 12-mm fibrous sheathextending along a 10-cm segment of the aorta. Com-puted tomography (figure 2) confirmed the presence ofretroperitoneal circumferential periaortic fibrosis with-out involvement of the ureters.

Investigations for a malignancy were negative.

DISCUSSION

Our patient was diagnosed with spondyloarthropathymeeting Amor’s and ESSG criteria. Subsequently, anultrasound scan done to investigate pain in the low backand abdomen showed retroperitoneal fibrosis [2]. Ret-roperitoneal fibrosis, also called Ormond syndrome, israre, with a prevalence of less than 1/10 000 [1]. It ischaracterized by gradual transformation of the retro-

peritoneal tissue into a sheet of tight fibrous tissue (withthree successive stages, inflammatory, fibroblastic, andfibrous). The typical patient is a man (male-to-femaleratio>2) 40 to 60 years of age. At the initial inflamma-tory stage, the main symptoms are low back pain withor without limitation of lumbar spine motion, abdomi-nal pain, gastrointestinal complaints, and constitutionalsymptoms. Subsequently, the manifestations vary withthe extent of the fibrosis, which can involve the retro-peritoneal organs in the abdomen, pelvis, and chest.Compression of the urinary tract can lead to anuria andhypertension. Laboratory tests show evidence of inflam-mation in over two-thirds of patients and of renalfailure in patients with chronic extensive disease. Mul-tifocal forms have been described with, for instance,concomitant involvement of the mediastinum orarthralgia related to periarticular fibrosis [3]. The intro-duction of ultrasonography, magnetic resonance imag-ing (MRI) and, above all, computed tomography (CT)has reduced the need for intravenous urography andangiography. These modern imaging techniques canprovide the diagnosis [2] and supply information onthe extent of the fibrosis and on its course under treat-ment.

Retroperitoneal fibrosis can be idiopathic or second-ary. Causes include malignancies (breast, stomach, pros-tate, lymphoma, urinary tract), drugs (methysergideabove all, but also ergotamine and its derivatives (bro-mocriptine), amphetamines, pergolide, beta-blockers,haloperidol, and analgesics); vascular disease (with afibrous sheath surrounding the aorta or an inflamma-tory aneurysm of the aorta), and locoregional abnor-malities (infection, bleeding, inflammation, iatrogeniclesions). The pathophysiology of idiopathic periaorticretroperitoneal fibrosis may involve an immunoallergicreaction against atheromatous plaque or one of itscomponents resulting in local or diffuse vasculitis [1].

Retroperitoneal fibrosis often occurs in patients withother conditions, particularly fibrosing disorders(Riedel’s disease, orbital pseudotumors, sclerosing cho-langitis, pancreatic pseudotumors, pulmonary fibro-sis...). Anecdotal case reports have been reported inwhich retroperitoneal fibrosis occurred in patients withrheumatic diseases including necrotizing angeitis (e.g.,periarteritis nodosum, giant cell arteritis, and Takayasudisease), granulomatous diseases (e.g., primary biliarycirrhosis and sarcoidosis); systemic diseases (e.g., poly-chondritis, scleroderma [4, 5], systemic lupus erythe-matosus [6] and Sjögren’s syndrome [7]) andpanniculitis [8]. We found nine other reports of retro-

Figure 1. Radiograph of the pelvis, anteroposterior view: completeobliteration of both sacroiliac joints.

Figure 2. Abdominal computed tomography scan: retroperitonealfibrosis surrounding the aorta.

Spondyloarthropathy and retroperitoneal fibrosis 335

peritoneal fibrosis in patients with a definitive diagnosisof spondyloarthropathy [9, 10, 11, 12, 13, 14, 15, 16].Physicians should be aware of the risk of overdiagnos-ing spondyloarthropathy in patients with low back painrelated to retroperitoneal fibrosis [17-22]. Retroperito-neal fibrosis has been reported in three patients withSAPHO syndrome [23, 24] and one with uveitis [25]and has also occurred in HLAB27-positive patientswithout spondyloarthropathy [5, 26, 27, 28].

In most patients with a definitive diagnosis of spondy-loarthropathy, the diagnosis was ankylosing spondyli-tis. Two-thirds of patients were males, and age atdiagnosis ranged from 27 to 72 years. Most patientshad axial manifestations, and few reports mention thepresence of peripheral arthritis [13]. The inflammationas assessed by laboratory tests was unusually marked.Only half the patients were positive for the HLAB27antigen. The relation between retroperitoneal fibrosisand spondyloarthropathy remains controversial. Thespondyloarthropathy usually antedates the retroperito-neal fibrosis, suggesting that it may promote the occur-rence of retroperitoneal fibrosis. Another hypothesis isthat the HLAB27 antigen may be associated with sus-ceptibility to retroperitoneal fibrosis and that this lastcondition may be most likely to occur in patients whoare B27-positive but do not have spondyloarthropathy[5, 26, 27, 28]. However, half the patients with bothconditions reported in the literature were negative forHLAB27 and the frequency of HLAB27 in large seriesof patients with retroperitoneal fibrosis is unknown.Furthermore, in a study of eight patients with retroperi-toneal fibrosis whose Class I HLA type was determined,B27 was consistently absent [29]. It has been suggestedthat retroperitoneal fibrosis may cause changes in thesacroiliac joints [27] but the longitudinal data thatwould be needed to confirm this hypothesis are lacking.Finally, the small number of reported cases and theoccurrence of many other conditions in patients withretroperitoneal fibrosis leave open the possibility thatchance alone may explain the combination. If this werethe case, however, one would expect a greater numberof reports of retroperitoneal fibrosis in patients withrheumatoid arthritis. There has been a single report ofjuvenile chronic arthritis and retroperitoneal fibrosis, ina 13-year-old girl [30]. Whether this low frequency ofretroperitoneal fibrosis is related to the more commonuse of glucocorticoids and immunomodulating agentsin rheumatoid arthritis than in spondyloarthropathiesremains unknown.

CONCLUSION

Rheumatologists should be aware of the symptoms ofretroperitoneal fibrosis. This condition mainly affectsmen and can cause inflammatory back pain. It canoccur in combination with various rheumatic condi-tions including spondyloarthropathy.

REFERENCES

1 Chapelon-Abric C, Godeau P, Piette JC. Fibroses systémiquesidiopathiques. In: Kahn MF, Peltier AP, Meyer O, Piette JC,Eds. Maladies et syndromes systémiques. Paris: Flammarion;2000. p. 1169-88.

2 Poitras JM, Dennis GJ. Clinical images: diagnosis and treat-ment of retroperitoneal fibrosis. Arthritis Rheum 2000 ; 43 :943.

3 Ewald EA, Gikas PW, William Castor C. Periarticular fibrosisassociated with idiopathic retroperitoneal fibrosis. J Rheumatol1988 ; 15 : 1443-6.

4 Dellambre V, Bataille D, Robert Y, et al. Fibrose rétropéri-tonérale et sclérodermie. Association fortuite ou mécanismecommun? Rev Med Int 1991 ; 12 : S129.

5 Mansell MA, Watts RWE. Retroperitoneal fibrosis and sclero-derma. Postgrad Med J 1980 ; 56 : 730-3.

6 Lichon FS, Sequeira W, Pilloff A, Skoskey JL. Retroperitonealfibrosis associated with systemic lupus erythematosus: a casereport and brief review. J Rheumatol 1984 ; 11 : 373-4.

7 Gentric A, Herve JP, Cledes J. Fibrose rétropéritonéale idio-pathique et syndrome de Gougerot-Sjögren. Presse Med 1987 ;34 : 1702.

8 Bonnet C, Arnaud M, Bertin P, Treves R, Desproges-Got-teron R. Idiopathic retroperitoneal fibrosis with systemic mani-festations. J Rheumatol 1994 ; 21 : 360-2.

9 Azais I, Roblot P, Barrier J, Debiais F, Becq-Giraudon B,Bontoux D. Fibrose rétropéritonéale idiopathique et spondylar-thrite ankylosante. Rev Rhum 1993 ; 60 : 367-70.

10 De Meulder A, Durieux JL. Rapport d’un cas de fibroserétropéritonéale associée à une spondylarthrite ankylosante.Louvain Med 1977 ; 96 : 387-94.

11 Vital-Durand D, Rouhier D, Brette R. Association fibroserétropéritonéale-spondylarthrite ankylosante: une nouvelleobservation. Ann Med Int 1981 ; 132 : 200-1.

12 Goldbach P, Mohsenifar Z, Salik AI. Familial mediastinalfibrosis associated with seronegative spondylarthropathy. Arthri-tis Rheum 1983 ; 26 : 221-5.

13 Solomon SD, Maurer KH. The association of retroperitonealfibrosis and ankylosing spondylitis. J Rheumatol 1985 ; 12 :818.

14 Wicks IA, Robertson MR, Murnaghan GF, Bertouch JV. Idio-pathic retroperitoneal fibrosis presenting with back pain. JRheumatol 1988 ; 15 : 1572-4.

15 Reilly PA, Moran CJ. Retroperitoneal fibrosis in radiotherapy-treated ankylosing spondylitis. Br J Rheumatol 1989 ; 28 :273-4.

16 De La Iglesia Martinez F, Grana GILJ, Gomez Veiga F, Rod-riguez Garcia J, Gomez Rodriguez N, Atanes Sandoval A. Theassociation of idiopathic retroperitoneal fibrosis and ankylosingspondylitis. J Rheumatol 1992 ; 13 : 1147-9.

17 Symmonds RE, Dahlin DC, Engel S. Idiopathic retroperitonealfibrosis. Obstet Gynecol 1961 ; 18 : 591-6.

18 Hissong SL, Freimanis AK. Retroperitoneal fibrosis. Am JRoentgenol 1969 ; 107 : 776-84.

336 D. Wendling et al.

19 Littlejohn GO, Keystone EC. The association of retroperito-neal fibrosis with systemic vasculitis and HLA B27: a case reportand review of the literature. J Rheumatol 1981 ; 8 : 665-9.

20 Ruivard M, Souweine B, Aumaitre O, Marcheix JC, Philippe P.Fibrose rétropéritonéale associée à une spondylarthropathie.Rev Med Int 1991 ; 12 : S420.

21 Takahashi M, Ischikawa A, Kondo H. A case of seronegativespondylarthropathy with iritis and retroperitoneal fibrosis. Ryu-machi 1999 ; 39 : 17-21.

22 Astudillo L, Alric L, Jamard B, Laroche M. Fibrose rétropéri-tonéale chez un patient HLA-B27 positif. Rev Med Int 1999 ;20 : 1149-50.

23 Schilling F, Schweden F. Lumbo-sacroiliac hyperostosis withretroperitoneal fibrosis in spondarthritis hyperostotical pustulo-psoriatica with stenocostoclavicular hyperostosis and superiorand inferior venous obstruction syndrome: pathogenetichypothesis of fibro-osteopathia psoriatica. Z Rheumatol 1996 ;55 : 331-47.

24 Boonen A, Verwilghen J, Dequeker J, van der Linden S,

Westhovens R. Is SAPHO a spondylarthropathy? A vasculopa-thy? Report of a case. Rev Rhum (Engl Ed) 1997 ; 64 : 424-7.

25 Doi M, Uji Y. A case of uveitis associated with idiopathicretroperitoneal fibrosis. Am J Ophtalmol 1994 ; 117 : 358-62.

26 Fernandez-Herlihy L. Idiopathic retroperitoneal fibrosis andHLA B27. J Urol 1980 ; 124 : 752.

27 Thorel JB, Toulet R, Lenormand JP, Schmitt B. Fibroserétropéritonéale idiopathique chez un sujet porteur d’un anti-gène HLA B27. Rev Rhum 1982 ; 49 : 891-5.

28 Willscher MK, Novicki DE, Cwazka WF. Association of HLAB27 antigen with retroperitoneal fibrosis. J Urol 1978 ; 120 :631-3.

29 Chevet D, Rivalan J, Rebibou JM, Michel F, Guignier F.Idiopathic retroperitoneal fibrosis and HLA antigens. Nephron1996 ; 73 : 495.

30 Tsai TC, Chang PY, Chen BF, Huang FY, Shih SL. Retroperi-toneal fibrosis and juvenile rheumatoid arthritis. Pediatr Neph-rol 1996 ; 10 : 208-9.

Spondyloarthropathy and retroperitoneal fibrosis 337