Endgames Case Review

A non-cardiac cause of bilateral leg swelling

BMJ 2017; 359 doi: https://doi.org/10.1136/bmj.j5306 (Published 30 November 2017) Cite this as: BMJ 2017;359:j5306
  1. Daniel R Stevenson, clinical fellow,
  2. Hasnain Hashim, rheumatology SpR,
  3. Mohammed Salman, GPVTS,
  4. Maria Mouyis, rheumatology consultant
  1. Northwick Park Hospital, Harrow, UK
  1. Correspondence to Daniel Stevenson danny.r.stevenson{at}gmail.com

A 65 year old man described swelling in both legs, which had worsened over six weeks. He also reported vague abdominal discomfort and tiredness. Examination of the lower limbs revealed pitting oedema bilaterally up to the groin, but no erythema, tenderness, or warmth. The patient was otherwise systemically well with normal heart sounds and a clear chest examination. There were no palpable masses in the abdomen or lymphadenopathy. Blood tests were unremarkable other than a normocytic anaemia (haemoglobin 95 g/L; normal range 130-180), C reactive protein of 33.0 mg/L (normal range 0-5.0) and a raised IgG4 level (3.55 g/L; normal range 0.08-1.4). No deep vein thrombosis was found on Doppler ultrasound. The patient had computed tomography of his abdomen and pelvis, which showed retroperitoneal fibrosis (fig 1). A unifying diagnosis was made of retroperitoneal fibrosis secondary to IgG4 related disease.

Fig 1

Cross sectional and coronal views of computed tomography of the abdomen and pelvis. The arrowhead shows a mass encasing the aorta, suggesting a diagnosis of retroperitoneal fibrosis

Questions

  • 1. What typical features should lead to consideration of retroperitoneal fibrosis as a diagnosis?

  • 2. What are the underlying causes of retroperitoneal fibrosis?

  • 3. How is retroperitoneal fibrosis and IgG4 related disease diagnosed and treated?

Answers

1. What typical features should lead to consideration of retroperitoneal fibrosis as a diagnosis?

Short answer

The onset of retroperitoneal fibrosis is insidious and most commonly presents with poorly localised abdominal and lumbar pain, fatigue, and weight loss. Routine investigations find increased acute phase reactants, and acute kidney injury if ureteral obstruction is present.

Discussion

Retroperitoneal fibrosis is a rare condition of inflammation and fibrosis in the infrarenal periaortic and iliac vessel.1 It is difficult to diagnose because it is rare …

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