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Cholangitis Lenta a Clinicopathologic Study of 28 Cases

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Cholangitis Lenta a Clinicopathologic Study of 28 Cases ORIGINAL ARTICLE Cholangitis Lenta A Clinicopathologic Study of 28 Cases Vanda F. Torous, MD,* A. Laura De La Cruz, MD,† Bita V. Naini, MD,‡ and Hanlin L. Wang, MD, PhD‡ angitis that has been described primarily in septic Abstract: Cholangitis lenta, also known as ductular cholestasis, patients.1–5 It has also been observed in a small subset of cholangiolar cholestasis, or subacute nonsuppurative cholangitis, is patients with obstructive jaundice.5,6 The characteristic an uncommon type of cholangitis characterized by ductular re- histopathologic features of cholangitis lenta are pro- action with inspissated bile in dilated ductules. The literature on liferation of bile ductules at the periphery of portal tracts, this unique entity has been limited to only a few studies based on a presence of inspissated bile within dilated ductules, and very limited number of cases, which importantly suggest an asso- relatively unremarkable interlobular bile ducts. ciation with sepsis and/or intra-abdominal infection. The clinical, The term cholangitis lenta was first coined by laboratory, and histologic features of 28 cases of cholangitis lenta Schottmuller7 in 1921, which was considered to be a are herein investigated. Twenty-five (89.3%) patients were liver hepatic analog of an indolent cardiac infection by viridans transplant recipients. Most notably, the majority of patients streptococci. The term subacute nonsuppurative chol- showed clinical signs and symptoms of sepsis, and positive mi- angitis was first used by Lin et al5 in their publication in crobiology cultures were demonstrated in 24 (85.7%) patients. 2007. Once thought to be largely because of mechanical Significantly, 15 (53.6%) patients died during their hospitalization, biliary obstruction, this entity is now believed to reflect a ranging from 2 days to 5 months after the initial liver biopsy that more aggressive clinical picture in septic patients. How- showed histologic features of cholangitis lenta. Among the 13 ever, the pathophysiologic mechanisms leading to this discharged patients, including 2 who received retransplantation, 4 form of liver injury are not entirely clear, with questions (14.3%) subsequently died of pneumonia, graft dysfunction, or remaining about its relationship to infection, sepsis, and fungal infection within 7 months to 9.3 years. Only 9 (32.1%) liver transplantation, as well as its clinical implications. patients were alive at the last follow-up, with the follow-up time In this study, we reviewed the largest cohort of patients ranging from 3.8 to 10.4 years. Our data show that the finding of with cholangitis lenta studied to date in order to further cholangitis lenta on liver biopsy is thus frequently associated with characterize the clinicopathologic features of this entity. We sepsis and with a high mortality rate. Therefore, accurate diagnosis attempted to elucidate the relationship between cholangitis of this condition on liver biopsy is imperative as it is an indication lenta to various clinical factors, including laboratory tests, that the patient may have a potentially life threatening condition signs and symptoms of sepsis, and liver transplantation sta- that requires immediate medical attention and management. tus. We also detailed the clinical outcomes of these patients. Key Words: cholangitis lenta, sepsis, ductular cholestasis, chol- angiolar cholestasis, cholangitis MATERIALS AND METHODS (Am J Surg Pathol 2017;41:1607–1617) Patients After approval by the Internal Review Board of our institution (the University of California, Los Angeles), we holangitis lenta, also known as ductular cholestasis, retrospectively searched our pathology information system Ccholangiolar cholestasis, or subacute nonsuppurative to identify liver biopsies that carried the potential diagnosis cholangitis, is a unique histopathologic pattern of chol- of cholangitis lenta. A variety of diagnostic phrases and combinations of histologic descriptions were used to max- “ ” From the *Department of Pathology and Laboratory Medicine, Beth imize the search results. These included cholangitis lenta, Israel Deaconess Medical Center, Harvard Medical School, Boston, “ductular cholestasis,”“cholangiolar cholestasis,”“subacute MA; †Department of Pathology and Laboratory Medicine, Rideout nonsuppurative cholangitis,”“ductular reaction and cho- Memorial Hospital, Marysville; and ‡Department of Pathology and lestasis,”“ductular proliferation and cholestasis,”“ductular Laboratory Medicine, David Geffen School of Medicine, University ” “ of California at Los Angeles, Los Angeles, CA. reaction and inspissated bile, and dilated ductules and Conflicts of Interest and Source of Funding: The authors have disclosed cholestasis.” Seventy-five potential cases spanning over a that they have no significant relationships with, or financial interest period of 10 years were initially identified based on the in, any commercial companies pertaining to this article. diagnosis or microscopic description in pathology reports. Correspondence: Hanlin L. Wang, MD, PhD, Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California at Los Angeles, 10833 Le Conte Avenue, Los Evaluation of Liver Biopsy Histopathology Angeles, CA 90095 (e-mail: [email protected]). Hematoxylin and eosin–stained slides were available Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved. for review for all 75 potential cases, along with special Am J Surg Pathol Volume 41, Number 12, December 2017 www.ajsp.com | 1607 Copyright r 2017 Wolters Kluwer Health, Inc. All rights reserved. Torous et al Am J Surg Pathol Volume 41, Number 12, December 2017 TABLE 1. Clinical and Laboratory Findings in Patients With Cholangitis Lenta Patient Age (y) Sex Race Temperature, Maximum (°C) AST (IU/L) ALT (IU/L) ALP (IU/L) T-Bili (mg/dL) WBC (×103/μL) 1 42 M AA 38.0 2575 1015 352 11.8 45.2 2 59 M W 36.9 2404 1744 421 39.0 15.4 3 15 M H 38.2 74 52 122 7.0 43.0 4 59 F NA 38.0 134 95 177 15.2 49.0 5 59 M W 36.2 394 451 357 4.1 5.6 6 56 M W 37.9 322 270 719 37.1 20.2 7 61 F W 37.7 119 423 111 17.8 6.3 8 29 F A 38.5 95 333 427 13.7 9.7 9 53 M W 37.4 154 43 204 5.2 18.1 10 65 F AA 37.7 2790 1360 195 40.4 19.8 11 62 M H 37.9 1088 467 2152 39.4 31.1 12 41 M H 39.0 78 170 161 17.2 19.1 13 56 F H 37.3 15,388 9272 2221 25.9 17.0 14 54 M H 38.0 48 75 68 3.0 6.8 15 58 F AA 38.4 301 269 1096 18.0 12.5 16 66 M W 37.5 99 84 413 38.3 42.0 17 61 M W 37.4 126 142 481 1.6 8.4 18 3 F W 39.1 236 91 247 11.2 47.2 19 14 M W 38.5 77 113 149 1.7 18.1 20 60 F W 37.8 87 203 260 13.4 23.2 21 52 M W 39.3 116 101 291 14.3 7.5 22 36 F H 37.7 47 49 981 2.1 11.2 23 85 F W 37.3 322 170 638 8.2 20.4 24 68 M A 38.4 492 132 122 42.5 10.1 25 59 M W 38.4 60 48 171 24.2 23.83 26 18 M H 39.4 96 237 123 6.8 33.04 27 62 M W 38.3 745 626 1010 43.0 21.5 28 65 M W 37.4 1162 513 267 46.0 16.21 A indicates Asian; AA, African American; ALP, alkaline phosphatase (normal range, 45 to 115 IU/L); ALT, alanine transaminase (normal range, 7 to 55 IU/L); AST, aspartate transaminase (normal range, 8 to 48 IU/L); F, female; H, Hispanic; M, male; NA, not available; T-Bili, total bilirubin (normal range, 0.1 to 1.0 mg/dL); W, white; WBC, white blood cell count (normal range, 4.5 to 11.0×103/μL). stains for trichrome, reticulin, periodic acid-Schiff (with maximum body temperature and white blood cell counts and without diastase), and iron. The biopsy specimens during the course of hospitalization. The infectious disease were reviewed by 2 pathologists, both specialized in liver work-up was also reviewed for each case, which included pathology (B.V.N. and H.L.W.). They were blinded to the results of blood, urine, respiratory tract, wound, and body clinical parameters and prior rendered diagnoses in order to fluid cultures. Pertinent radiologic studies were reviewed for confirm the diagnosis of cholangitis lenta. Diagnostic cri- the presence or absence of intra-abdominal abscess, biliary teria included the following: proliferation of bile ductules at obstruction, abnormalities of hepatic vasculature, or other the edges of portal tracts, inspissated bile within dilated bile signs of infection. The time frame between the onset of ductules, and no bile accumulation in interlobular bile clinical conditions and liver biopsy was recorded. ducts. A total of 28 cases fulfilled the histologic criteria for The clinical outcomes were then investigated for each cholangitis lenta and were included in the study. The re- case. This included either death or discharge home after hos- maining 47 cases did not meet the above diagnostic criteria pitalization, as well as the condition of survived patients after mainly because of lack of apparent ductular dilatation and discharge. When performed, follow-up liver biopsies, explants lack of characteristic inspissated bile in dilated ductules. of retransplantation, or autopsy results were reviewed. Cirrhotic livers with marked canalicular, cytoplasmic, and ductular cholestasis were also excluded from the study be- cause the findings were believed to represent decom- RESULTS pensated end-stage chronic liver disease. Clinical and Laboratory Findings Of the 28 confirmed cases of cholangitis lenta, 18 pa- Clinical Variables and Infectious Disease tients were male and 10 were female.
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