- more common in males and peak in 5th or 6th decade of life
- one of the multifocal fibrosclerotic syndrome (which involves mediastinal fibrosis, sclerosing cholangitis and Riedel thyroiditis)
- presents with low back pain (commonest), malaise, anemia, uremia (ureteric obstruction), raised ESR
- 2/3 idiopathic, associated with malignancy, drug (methysergide, practolol), radiotherapy, untreated AAA, SLE, scleroderma, carcinoid syndrome
- CT is best diagnostic modality
- treatment: steroid, azathioprine
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