Retroperitoneal fibrosis

Classification according to ICD-10
K66.2	Retroperitoneal fibrosis
ICD-10 online (WHO version 2019)

The retroperitoneal fibrosis (retroperitoneal fibrosis ), and Ormond's disease or Ormond syndrome called in the Anglo-American literature also Albarran-Ormond syndrome , "Gerota's fascitis" or "Gerota's syndrome" is a slowly increasing connective tissue between the rear peritoneum and spine with encasement of the Vessels, nerves and ureters .

history

One of the first described cases of this syndrome was the astronomer Friedrich Wilhelm Bessel . The Cuban urologist Joaquín Albarrán (1860–1912) was the first to describe retroperitoneal fibrosis (1905 ). However, the disease first became generally known with a publication by the US urologist John Kelso Ormond in 1948, who described two patients with diffuse increase in connective tissue behind the abdominal cavity (retroperitoneal) and established a clinical and pathological clinical picture.

etiology

Idiopathic form

In the case of primary (= idiopathic form = Ormond's disease or Albarran-Ormond syndrome ) retroperitoneal fibrosis, neither an underlying disease nor any other triggering event can be detected. An autoimmunological process is assumed to be the cause of the disease . Often there are elevated IgG4 serum levels, so that the suspicion arises that idiopathic retroperitoneal fibrosis is one of the IgG4-associated diseases.

Secondary form

A secondary (= Ormond syndrome) retroperitoneal fibrosis can be caused by autoimmune diseases such as Crohn's disease , primary biliary cholangitis , granulomatosis with polyangiitis , Sjogren's syndrome and Erdheim-Chester disease . Retroperitoneal fibrosis can also occur together with arteriosclerosis of the aorta or an aortic aneurysm ; In these cases, an immune reaction to atheromatous material in the arterial wall is possibly the trigger for the disease process. Furthermore, retroperitoneal fibrosis can occur after radiation , exposure to asbestos , chronic urinary congestion , inflammation or infection of the urinary tract , injuries, malignant tumors and under therapy with certain vasoactive drugs such as methysergide , pergolide or bromocriptine . A triggering event can be detected in less than 25% of the cases.

Pathogenesis of idiopathic retroperitoneal fibrosis

A specific trigger for idiopathic retroperitoneal fibrosis is not known. The tissue examination reveals fibrotic and inflammatory changes. The fibrosis is characterized by a proliferation of collagen and myofibroblast , the inflammatory components by a infiltrate , consisting predominantly of T- and B-lymphocytes is (Fig. Below). The blood test shows increased markers of inflammation such as blood sedimentation and C-reactive protein . These markers are used not only as part of the diagnosis of the disease, but also to monitor the course of inflammatory activity. A variety of other more specific inflammatory markers can be elevated such as CD20 -positive B-lymphocytes, CD4- and CD40 -positive T-lymphocytes, immunoglobulin G4 and antinuclear antibodies . An association with certain types of the HLA system has also been described.

Epidemiology

There is one case of illness out of around 200,000 Germans. In the international literature, the incidence is described as less than one event per 100,000 people, but the increasing number of case reports suggests that the disease may be more common. Men are affected relatively more often, the peak age is between 40 and 60 years.

clinic

Usually, dull, difficult to localize, non-colicky pain in the flanks, back , scrotum or lower abdomen is reported. All organ systems of the retroperitoneum can be affected. In 80–100% of cases, the ureters are walled ( ureteral stenosis ) and the urine is backed up in the kidneys, which in advanced stages can lead to hydronephrosis . However, the intestinal tract , biliary and pancreatic system , aorta and branches of large arteries , pelvic organs and peripheral nerves can also be affected. In addition, leg swelling can occur due to an obstruction of the drainage of the veins or lymph vessels . In rare cases, inflammatory or fibrotic changes of the mediastinum , pericardium , pleura , thyroid , paranasal sinuses, or eye sockets have been observed.

Diagnosis

The histological examination ( histology ) is evidence of the disease . Often times, the diagnosis is made using imaging techniques alone , as tissue removal is too great a risk. Magnetic resonance tomography and computed tomography are suitable . The ultrasound alone is not suitable.

Computed tomography and magnetic resonance tomography often show an increase in the connective tissue surrounding the aorta (= periaortal), which encapsulates the distal aorta below the branches of the renal arteries and adjacent anatomical structures. The tissue has a texture that suggests dense fibrosis. The aorta can appear both narrowed and expanded. In the case of atypical features such as lymph node enlargement, displacement symptoms or atypical localization, tissue removal is recommended in order to rule out a malignant or granulomatous process.

therapy

Due to the rarity of the disease, there are no controlled studies on the treatment of retroperitoneal fibrosis. The therapy recommendations are therefore based on case reports (case reports) and smaller case series . In individual cases, spontaneous healing (spontaneous remissions) has been described, but these courses are the exception.

Operative therapy

If there is a urinary tract disorder, the drainage must be restored. This is usually achieved with an internal splint with a catheter. Otherwise, the ureters are surgically exposed and relocated intraperitoneally . In exceptional cases, e.g. B. In the case of a chronic infection, the removal of a kidney (nephrectomy) may be necessary.

Medical therapy

Due to their relative rarity, there is no uniform drug therapy. There are good results with immunosuppressants (such as corticosteroids or azathioprine ) and with tamoxifen . Also, cyclophosphamide , methotrexate , mycophenolate mofetil , cyclosporin A and colchicine have been used successfully. It is true that there is no laboratory parameter that is specific for retroperitoneal fibrosis; however, the response to drug therapy can be monitored by determining the C-reactive protein, a non-specific inflammatory marker.

Treatments for complications

Complications can make special therapeutic measures necessary. Due to a narrowing of the intestine , an intestinal obstruction can occur which must be treated surgically. A urinary outflow disorder can lead to urinary tract infections up to and including inflammation of the kidneys , which must be treated with antibiotics. A narrowing of the large veins can lead to disturbances of the blood flow and thrombosis , which may require treatment with anticoagulant substances .

Course and prognosis

Usually there is a good response to immunosuppressive therapy. However, there is a risk of relapse, especially if the duration of treatment is too short. It is generally treated between 12 and 24 months. Occasionally, permanent kidney damage occurs due to a long-standing urinary obstruction. In early case series, mortality was given as 10 to 20%, in current reports the mortality is well below 10%, the incidence of progressive chronic kidney damage in treated patients is less than 5% of the cases.

literature

• EF van Bommel: Retroperitoneal fibrosis. In: Neth J Med. , 2002 Jul, 60 (6), pp. 231-242. Review: PMID 12365466
• J. Albarran: Retention rénale par periurétérité. Liberation external de l'uretère. Association française d'urologie, 1905, 9, p. 511.
• E Neumann-Redlin von Meding .: 150 years ago: the description of retroperitoneal fibrosis, the "Ormond's disease", using the clinical picture of FWBessels (1784–1846). In: Der Urologe (B) , 1996, 36, pp. 378-382
• JK Ormond: Bilateral ureteral obstruction due to envelopment and compression by an inflammatory retroperitoneal process. In: The Journal of Urology , 1948, 59, pp. 1072-1079.
• JK Ormond: Idiopathic retroperitoneal fibrosis: a discussion of the etiology. In: The Journal of Urology , 1965, 94, pp. 385-390.

Web links

• Retroperitoneal fibrosis, diagnostic imaging (English)

Individual evidence

1. ↑ Alphabetical directory for the ICD-10-WHO version 2019, volume 3. German Institute for Medical Documentation and Information (DIMDI), Cologne, 2019, p. 753
2. ^ Pschyrembel clinical dictionary . 261st edition. Walter de Gruyter, Berlin / New York 2007, ISBN 978-3-11-018534-8 .
3. ↑ Fig .: Histology of retroperitoneal fibrosis from D Corradi, R Maestri, A Palmisano, S Bosio, P Greco, L Manenti, S Ferretti, R Cobelli, G Moroni, AP Dei Tos, C Buzio, A Vaglio: Idiopathic retroperitoneal fibrosis: clinicopathologic features and differential diagnosis . In: Kidney International . 72, No. 6, September 2007, ISSN  0085-2538 , pp. 742-753. doi : 10.1038 / sj.ki.5002427 . PMID 17622270 .
4. ↑ ^{a b c d e f g} Richard D Swartz: Idiopathic retroperitoneal fibrosis: a review of the pathogenesis and approaches to treatment . In: American Journal of Kidney Diseases . 54, No. 3, September 2009, ISSN  1523-6838 , pp. 546-553. doi : 10.1053 / j.ajkd.2009.04.019 . PMID 19515472 .

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