Title:Unknown Extra-Articular Manifestation of Spondyloarthritis: What
About Retroperitoneal Fibrosis? A Case Based Literature Review
Volume: 18
Issue: 4
Author(s): Soumaya Boussaid*, Marouene Ben Majdouba, Sonia Rekik, Samia Jammali, Elhem Cheour, Hela Sahli and Mohamed Elleuch
Affiliation:
- Rheumatology Department, Rabta Hospital, Tunis, Tunisia
- Faculty of Medicine of Tunis, University Tunis el Manar, Tunis, Tunisia
Keywords:
Retroperitoneal fibrosis, spondyloarthritis, ankylosing spondylitis, fibroinflammatory tissue, retroperitoneum, acute abdominal pain.
Abstract:
Introduction: Retroperitoneal fibrosis (RPF) is a rare disease characterized by fibroinflammatory
tissue proliferation in the retroperitoneum. It results in a chronic inflammatory and
fibrosis condition, possibly leading to compression of the retroperitoneal structures, especially to
encasement of the ureters and the inferior vena cava. It may have an idiopathic or a secondary
origin. Spondyloarthritis (SpA) is one of the rare conditions described among the secondary forms.
Case Presentation: Herein, we report a new case of RPF in a patient with AS presented with acute
abdominal pain radiating to the lumbar region and the left testicle. On clinical examination, we
found a mild stiffness of the lumbar spine and a decrease in chest expansion. Sacroiliac joint pain
was also found. The rest of the physical examination was normal. Laboratory tests showed inflammation
with increased C-reactive protein (130 mg/l) and creatinine (112 micromol/l) levels. The
computed tomography scan revealed a soft tissue density mass located around the sub-renal aorta.
Diagnosis of idiopathic RPF associated with AS was retained. The patient was treated with a daily
dosage of 1 mg/kg of oral glucocorticoid with a good outcome.
Conclusion: RPF is a rare condition that can be either idiopathic or secondary. Its association with
spondyloarthritis, mainly in its ankylosing spondylitis form, seems to be more than anectodal.
Treatment may involve medical therapy and/or surgical management.
Key Message: In the presence of back pain, fatigue, weight loss, and low grade fever in spondyloarthritis
patients, physicians should screen for retroperitoneal fibrosis as it could be a possible cause.