Retroperitoneal fibrosis

Retroperitoneal fibrosis is a rare thingy urologists and rheumatologists dealt with.

General

• May cause obstructive uropathy.
• One of the many IgG4-related systemic diseases.
• No standard diagnostic criteria.

Treatment:

• Immunosuppression.
• Ureteral stents - to avoid renal failure.

Microscopic

Features:

• Fibrous tissue (pink background) with inflammatory cells (plasma cells, benign lymphocytes and eosinophils).

Note:

• Entrapped adipocytes (white open spaces) are common.

Images

www:

• Retroperiteonal fibrosis (mypacs.net).

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A. KIDNEY, RIGHT, RADICAL NEPHRECTOMY:
- SEVERE PYELONEPHRITIS, CHRONIC ACTIVE.
- EXTENSIVE TOTAL SCLEROSIS OF GLOMERULI AND INTERSTITIAL FIBROSIS (END-STAGE KIDNEY).
- PERIRENAL FAT WITH FOCI OF FAT NECROSIS, FIBROSIS AND A MIXED INFLAMMATORY
  INFILTRATE WITH PLASMA CELLS.
- NEGATIVE FOR MALIGNANCY.

B. SOFT TISSUE, RETROPERITONEUM, BIOPSY:
- FIBROTIC SOFT TISSUE WITH A MINIMAL FOCAL LYMPHOCYTIC INFILTRATE.
- NEGATIVE FOR MALIGNANCY.

See also

• Riedel thyroiditis.
• IgG4-related systemic disease.