Postgrad Med J: first published as 10.1136/pgmj.47.552.666 on 1 October 1971. Downloaded from Postgraduate Medical Journal (October 1971) 47, 666-670. CLINICAL REVIEW Primary sclerosing cholangitis LAURENCE TINCKLER M.D., Ch.M., F.R.C.S., F.A.C S. Formerly Professor of Surgery, University of Singapore Summary however, had had previous biliary surgery. Miller The literature on the subject ofsclerosing cholangitis is reported a case in 1927. reviewed. Carter & Gilletie in 1951 described fifteen patients The value of conserving the gall bladder, of opera- with strictures involving the intrahepatic biliary tive cholangiography and of the administration of system. In 1963, Bartholomew et al. reported two steroids in the management of these patients is patients and suggested an association with retro- emphasized. peritoneal fibrosis and Riedel's thyroiditis. They con- sidered the possibility that a hypersensitivity reaction PRIMARY sclerosing cholangitis, stenosing cholangitis, might be the underlying aetiological factor. In one of chronic obliterative their patients, as well as in the experience of Schwartz Protected by copyright. fibrosing cholangitis, cholangitis & Dale (1958) corticosteroids appeared to be of are all terms referring to a rare diffuse type ofchronic definite therapeutic value. inflammation of the entire common bile duct and at In 1958, Schwartz & Dale found only thirteen times of the hepatic ducts and their radicles. In most authentic cases of the primary type in the literature instances, sclerosing cholangitis is idiopathic or and added six cases of their own. The majority of primary. cases reported were examples of extensive involve- Bartholomew et al., in 1963 found only twenty ment of the entire common bile duct with, at times, unequivocal cases reported in the previous 40 years cystic duct involvement. They suggested that the and considered that the rarity of the syndrome and disease is commoner than previously considered and the poor results of treatment warranted reporting of therefore its recognition and therapy assume greater individual cases, particularly if they provided new significance; they noted that matters of aetiology, aetiological or therapeutic data. Although Schwartz clinical course and final prognosis remain speculative & Dale (1958) described a diffuse obliterative ductal at this stage. Involvement of the sclerosis in with intrahepatic ducts patients gall-stone disease, stenosing as in the case described by Klemperer (1937) and by http://pmj.bmj.com/ cholangitis involving only a short segment of the Shumarer (1958) is evidently exceedingly rare. common duct may occur and Baddeley & Stammers The disease is one of adult life, commonly in (1964) infer that such lesions may diffusely spread patients of middle-age, and men are affected more throughout the bile duct system. often than women. Glen & Witsell (1966) stipulate the following Manesie & Sullivan (1965) reviewed the literature criteria for making the diagnosis: progressive biliary between 1924 and 1964 and found only twenty cases obstruction, exclusion of surgical injury as a possible fulfilling the criteria. Wolubitsky & Mackenzie (1964) causative agent, operative demonstration of a diffuse found on review of the literature between 1924 and on September 29, 2021 by guest. sclerosing process involving the common duct, 1964, twenty-four acceptable cases out of 100 frequently the hepatic ducts and sometimes the gall reported and added four of their own. bladder, a fibrotic process and absence of malignant Warren, Athanassiades & Monge (1966) found the neoplasm requiring histological confirmation. records of forty-two patients at the Lahey Clinic and noted that twelve of these patients had associated Incidence ulcerative colitis; eleven patients had no history of The first case was reported by Delbet in 1924; gall stones or previous surgery, and there were no and the second 1 year later by Lafucade (1925). other associated diseases; a fourth group included Judd reported three patients in 1926 all of whom, four patients in whom severe periportal inflamma- tion preceded the sclerosing obliterative process. In Address for reprints: 7 Mossgiel Avenue, Ainsdale, South- one of these four patients suppuration also had port, Lancs. occurred. Clinical review 667 Postgrad Med J: first published as 10.1136/pgmj.47.552.666 on 1 October 1971. Downloaded from Glen & Witsell (1966) reported seven patients sclerosing cholangitis clinically and in gross appear. under their care at the New York Hospital. Operative ance. findings were similar in all patients. The gall bladder in each patient exhibited some degree of fibrosis but Pathology calculi were present in only three. Enlargement of Great thickening of the wall of the involved duct lymph nodes along the common duct was fre- or ducts associated with marked luminal narrowing quently accompanied by oedema and fibrosis in the characterizes the lesion. In most cases the common region of Calot's triangle. The external diameter of duct lumen has been between 3 and 5 mm in diameter the common duct although not increased and even (Jackson & MacVey, 1962), it being possible to sometimes reduced was firm and thickened to palpa- insert a fine probe only into the lumen. The wall of tion in every patient. Narrowing of various portions the ducts may be up to eight times the normal of the biliary tree was demonstrated by operative thickness (Roberts, 1955) and on palpation the cholangiography in six of the seven patients. Radio- common duct is cord-like throughout. Lymph nodes logical visualization of the biliary system could not about the duct are usually enlarged and at times the be accomplished in the remaining patient because entire area is encased in dense adhesions (Schwartz almost complete obliteration of the lumen of the & Dale, 1958). The duct mucosa is normal, the duct common duct made introduction of the contrast thickening involving the sub-mucosa and sub-serosa agent impossible. Common duct exploration was in a diffuse fibrotic process with oedema inter- accomplished in five patients and in each was vening. Biopsy of the liver shows bile stasis and demonstrated either localized segmental or general periportal fibrosis. The diagnosis is made on the diffuse involvement of the ductal system. When the basis of findings at operation which may be of a lumen was narrowed there was a corresponding dense inflammatory reaction in the region of the increase in the thickness of the wall. The process was gall bladder and gastro-hepatic ligament. This may so marked in one patient that common duct explora- be so intense that demonstration of the common Protected by copyright. tion was not feasible. Biopsy of the common duct duct is difficult; its size is not diagnostic because this was performed on four patients: dense vascular represents the sum of the narrowed lumen and fibrous tissue was demonstrated in three of the thickened wall (Schwartz & Dale, 1958). Palpation biopsies, while the fourth showed only common duct of the common duct imparts the sensation of a cord- mucosa. like structure which may feel like a thrombosed blood vessel. When the wall of the common duct is Clinical picture incised the diagnosis should be clear; the wall is The usual clinical picture is one of slowly progres- obviously thickened, cuts with difficulty and the sive jaundice often associated with discomfort in the edges of the incision pout outwards. It is possible to abdomen or upper lumbar region. True biliary colic insert a fine probe only into the lumen. is unusual. The age range has been from 21-67 years, It is of interest that in all cases in which operative patients in the fourth and fifth decade being more cholangiograms were obtained, radio-opaque ma- common. The ratio of males to females has been terial was seen to enter the duodenum thus indicating about 3:1. Laboratory findings have invariably been that obstruction is not always complete. http://pmj.bmj.com/ those of obstructive jaundice. Symptoms of scleros- k Bartholomew et al (1963) say that essentially there ing cholangitis are indistinguishable from those is inflammatory fibrosis quite comparable to, if not associated with chronic gall-bladder disease, the identical with that seen in Riedel's struma. The most frequent sign being a slowly deepening jaundice histological similarity of Riedel's struma and idio- over a period of weeks or months. Hepatomegaly is pathic fibrous mediastinitis has been emphasized, not usual. A definitive diagnosis is established by and it seems logical to infer that Riedel's struma, abdominal exploration, by X-ray and by histological idiopathic fibrous mediastinitis, sclerosing cho- on September 29, 2021 by guest. study of the biliary tree. langitis and retroperitoneal fibrosis represent a The disease results in chronic biliary obstruction basically similar pathological process occurring in followed by secondary biliary sclerosis, eventual different sites. liver failure and terminal coma. Less often portal According to Glen & Witsell (1966) the operative hypertension and bleeding oesophageal varices occur findings seem to vary with stages of the disease but as terminal events. It is associated in a significant the characteristic changes are located in the gall number of cases with chronic, moderately severe bladder and especially in the common duct. ulcerative colitis, often with stones, occasionally with Although the gall bladder is frequently calculus free, Riedel's struma and peri-ureteric fibrosis. It should stones are occasionally found.