Sclerosing cholangitis associated with multifocal

The association of sclerosing cholangitis, retroperitoneal fibrosis, and Riedel's thyroiditis has been reported twice before,'2 and on both occasions the authors successfully used steroids to control the fibrotic process. A further case of fibrosis with this triad of organ involvement in which a combination of surgery and steroid treatment has arrested disease progression is described. This suggests an inappropriate immune response in this type of fibrotic overlap syndrome. (Gut 1992; 33: 1430-1432) Liver Unit and Departments of Pathology and Radiology, Queen Elizabeth Medical Centre, Edgbaston, Birmingham R D Laitt S G Hubscher J A Buckels S Darby E Elias Correspondence to: D E Elias, Liver Unit, Queen Elizabeth Hospital, Edgbaston, Birmingham B15 2TH. Accepted for publication 2 March 1992 Abnormal fibrous tissue overgrowth has long been known to affect a number of widely separate organ systems. The terms Riedel's thyroiditis, sclerosing cholangitis, retroperitoneal fibrosis, mediastinal fibrosis, and pseudotumour of the orbit reflect the multiple areas affected. These fibrotic processes are rare and tend to occur in isolation, although a number of overlap syndromes has been reported with multiple organ involvement. This overlap was first described by Bowlby3 in 1885, in a necropsy report that commented on a hard infiltrative mass affecting the thyroid and other structures in the neck contiguous with similar tissue throughout the mediastinum. Barrett4 was the first to suggest that these fibrotic conditions were interrelated and probably different manifestations of the same disorder. Patients in whom fibrosis occurred in a combination of sites, particularly the biliary tree and retroperitoneal area, are well documented in a review by Lillemoe et al.' However, the association of fibrous thyroiditis with other sites of fibrosis was not widely noted until 1962 when Hache6 reported an association with retroperitoneal fibrosis and Woolner7 an association with sclerosing cholangitis. A year later Bartholemew' noted an association of Riedel's thyroiditis, sclerosing cholangitis, and retroperitoneal fibrosis in the same patient. Gleeson2 is the only other author to have documented this pattern of organ involvement. Both authors used steroids in an attempt to control the disease process, with some success. We report a further patient with Reidel's thyroiditis, sclerosing cholangitis, and retroperitoneal fibrosis whose condition improved coincident with steroid therapy. Case report A 51 year old woman was admitted to another hospital in February 1988 with a two month history of malaise and swelling in the neck. There was no relevant drug history. On physical examination she was afebrile but looked hypothyroid with a large, firm, generalised thyroid swelling. Her blood pressure was normal. Hypothyroidism was confirmed by a serum thyroid stimulating hormone concentration of more than 50 mU/l (normal range <6 mU/l) and a thyroxine concentration of 29 nmol/l (70-140 nmol/l). Thyroid autoantibodies were weakly positive. Thyroxine replacement treatment was begun with some improvement but by May the goitre had increased in size and began to cause local pressure effects. Thyroidectomy was performed without problem or need for blood transfusion. Notes taken at the time of operation commented on the appearances of 'an anaplastic thyroid carcinoma infiltrating strap muscles anteriorly and carotid sheath posteriorly. Formal resection was not possible.' Histology (Fig 1) showed complete effacement of the normal thyroid architecture and its replacement by dense collagenous and inflamed fibrous tissue. The inflammatory process was seen to extend into adjacent skeletal muscle and was consistent with a diagnosis of Riedel's thyroiditis. She recovered well and was allowed home on thyroxine replacement treat-