Retroperitoneal fibrosis or Ormond's disease is a disease featuring the proliferation of fibrous tissue in the retroperitoneum

Retroperitoneum

The retroperitoneal space is the anatomical space in the abdominal cavity behind the peritoneum. It has no specific delineating anatomical structures...

, the compartment of the body containing the kidney

Kidney

The kidneys, organs with several functions, serve essential regulatory roles in most animals, including vertebrates and some invertebrates. They are essential in the urinary system and also serve homeostatic functions such as the regulation of electrolytes, maintenance of acid–base balance, and...

s, aorta

Aorta

The aorta is the largest artery in the body, originating from the left ventricle of the heart and extending down to the abdomen, where it branches off into two smaller arteries...

, renal tract and various other structures. It may present with lower back pain, renal failure

Renal failure

Renal failure or kidney failure describes a medical condition in which the kidneys fail to adequately filter toxins and waste products from the blood...

, hypertension

Hypertension

Hypertension or high blood pressure is a cardiac chronic medical condition in which the systemic arterial blood pressure is elevated. What that means is that the heart is having to work harder than it should to pump the blood around the body. Blood pressure involves two measurements, systolic and...

, deep vein thrombosis

Deep vein thrombosis

Deep vein thrombosis is the formation of a blood clot in a deep vein. Deep vein thrombosis commonly affects the leg veins or the deep veins of the pelvis. Occasionally the veins of the arm are affected...

 and other obstructive symptoms. It is named after John Kelso Ormond

John Kelso Ormond

John Kelso Ormond was an American urologist who rediscovered retroperitoneal fibrosis in 1948.He was born in 1886 in Armstrong County, Pennsylvania and died February 25, 1978, in Ann Arbor, Michigan....

, who rediscovered the condition in 1948.

Causes

Its association with various immune-related conditions and response to immunosuppression

Immunosuppression

Immunosuppression involves an act that reduces the activation or efficacy of the immune system. Some portions of the immune system itself have immuno-suppressive effects on other parts of the immune system, and immunosuppression may occur as an adverse reaction to treatment of other...

 have led to speculations as to the autoimmune etiology of idiopathic RPF. One-third of the cases are secondary to malignancy, medication (methysergide

Methysergide

Methysergide is a prescription drug used for prophylaxis of migraine headaches and is sold under the brand names Sansert and Deseril in 2 mg dosages.-Pharmacology:Methysergide interacts with serotonin receptors...

, hydralazine

Hydralazine

Hydralazine is a direct-acting smooth muscle relaxant used to treat hypertension by acting as a vasodilator primarily in arteries and arterioles...

, beta blocker

Beta blocker

Beta blockers or beta-adrenergic blocking agents, beta-adrenergic antagonists, beta-adrenoreceptor antagonists or beta antagonists, are a class of drugs used for various indications. They are particularly for the management of cardiac arrhythmias, cardioprotection after myocardial infarction ,...

s), aortic aneurysm

Aortic aneurysm

An aortic aneurysm is a general term for any swelling of the aorta to greater than 1.5 times normal, usually representing an underlying weakness in the wall of the aorta at that location...

 or certain infections.

Diagnosis

The diagnosis of retroperitoneal fibrosis cannot be made on the basis of results of laboratory studies. CT is the best diagnostic modality: a confluent mass surrounding the aorta can be seen on a CT scan. Although biopsy is not usually recommended, it is appropriate when malignancy or infection is suspected. Biopsy should also be done if the location of fibrosis is atypical or if there is an inadequate response to initial treatment.

Treatment

In the absence of severe urinary tract obstruction (which generally requires surgery

Surgery

Surgery is an ancient medical specialty that uses operative manual and instrumental techniques on a patient to investigate and/or treat a pathological condition such as disease or injury, or to help improve bodily function or appearance.An act of performing surgery may be called a surgical...

 with omental wrapping), treatment is generally with glucocorticoid

Glucocorticoid

Glucocorticoids are a class of steroid hormones that bind to the glucocorticoid receptor , which is present in almost every vertebrate animal cell...

s initially, followed by DMARDs either as steroid-sparing agents or if refractory on steroids. The SERM tamoxifen

Tamoxifen

Tamoxifen is an antagonist of the estrogen receptor in breast tissue via its active metabolite, hydroxytamoxifen. In other tissues such as the endometrium, it behaves as an agonist, hence tamoxifen may be characterized as a mixed agonist/antagonist...

 has shown to improve the condition in various small trials, although the exact mechanism of its action remains unclear.
Associations include:

• Riedel's thyroiditis
  Riedel's thyroiditis
  Riedel's thyroiditis, also called Riedel's struma is a chronic form of thyroiditis.It is believed to be a form of autoimmune thyroiditis.-Pathophysiology:...
  
  
• previous radiotherapy
• sarcoidosis
  Sarcoidosis
  Sarcoidosis , also called sarcoid, Besnier-Boeck disease or Besnier-Boeck-Schaumann disease, is a disease in which abnormal collections of chronic inflammatory cells form as nodules in multiple organs. The cause of sarcoidosis is unknown...
  
  
• inflammatory abdominal aortic aneurysm
  Abdominal aortic aneurysm
  Abdominal aortic aneurysm is a localized dilatation of the abdominal aorta exceeding the normal diameter by more than 50 percent, and is the most common form of aortic aneurysm...
  
  
• drugs