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Primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is an inflammatory disease of the bile duct, which leads to cholestasis (blockage of bile transport to the gut). Bile is necessary for the absorption of dietary fat. more...

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Blockage of the bile duct leads to accumulation, damages the liver (leading to jaundice) and eventually causes liver failure. PSC is considered an autoimmune disease.

Signs and symptoms

  • Severe jaundice with itching
  • Malabsorption (especially of fat) and steatorrhea, leading to decreased levels of the fat-soluble vitamins, A, D and K.
  • Signs of cirrhosis
  • Infection of the bile duct (as ascending bacteria are not cleared)


The diagnosis is by imaging of the bile duct, usually in the setting of endoscopic retrograde cholangiopancreatography (ERCP, endoscopy of the bile duct and pancreas). Another option is magnetic resonance cholangiopacreaticography (MRCP), where magnetic resonance imaging is used to visualise the biliary tract.

Other tests often done are a full blood count, liver enzymes, bilirubin levels (usually grossly elevated), renal function, electrolytes. Fecal fat determination is occasionally ordered when the symptoms of malabsorption are prominent.

Differential diagnostics: Primary biliary cirrhosis.


The cause(s) for PSC are unknown, but it is considered an autoimmune disorder.

Bile ducts, both intra- and extrahepatically (inside the liver and outside), are inflamed and develop scarring, obstructing the flow of bile. As bile assists in the enteric breakdown and absorption of fat, the absence of bile leads to fat malabsorption. The bile accumulates in the duct, leading to liver cell damage and liver failure.

PSC is associated with ulcerative colitis. It is assumed that these diseases share a common cause.


It happens more in men than in women. The disease normally starts from age 30 to 60. It can however also start with children. PSC progresses slowly, so the disease can be active for a long time before it is noticed or diagnosed.


Standard treatment includes ursodiol, a bile acid naturally produced by the liver, which has been shown to lower elevated liver enzyme numbers in people with PSC, but has not yet been proven effective at prolonging the life of the liver. Treatment also includes medication to relieve itching (antipruritics), antibiotics to treat infections, and vitamin supplements, as people with PSC are often deficient in vitamin A, vitamin D, and vitamin K.

In some cases, surgery to open major blockages in the common bile duct is also necessary. Liver transplantation (including live transplants whereby a portion of a living donor is given to the recipient) is an option if the liver begins to fail.


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Severe autoimmune hemolytic anemia treated by paralysis, induced hypothermia, and splenic embolization
From CHEST, 2/1/05 by Ronna Campbell

Autoimmune hemolytic anemia (AIHA) is the clinical condition in which IgG antibodies bind to RBC surface antigens and initiate RBC destruction via the complement and reticuloendothelial system. AIHA is commonly treated with transfusions, corticosteroids, and splenectomy. We present a ease of an adult with life-threatening AIHA secondary to ulcerative colitis emergently managed with neuromuscular paralysis, induced hypothermia, and splenic embolization.

Key words: autoimmune hemolytic anemia; hypothermia; liver transplantation; splenic embolization; ulcerative colitis

Abbreviations: AIHA = autoimmune hemolytic anemia; LDH = lactate dehydrogenase; OPSI = overwhelming postsplenectomy infection; PRBC = packed RBC; V[O.sub.2] = oxygen uptake


Autoimmune hemolytic anemia (AIHA) is the clinical condition in which IgG antibodies bind to BBC surface antigens and initiate RBC destruction via the complement and reticuloendothelial system. (1,2) Since the autoantibodies are usually directed against high-incidence antigens, they often exhibit reactivity against allogenic RBCs as well. AIHA is an uncommon disease, with an incidence of approximately 10 cases per million population. (1,2) AIHA occurs more commonly in women than in men and usually occurs in midlife. Approximately 50% of cases are primary AIHA, the remaining 50% being associated with an underlying disease, most commonly lymphoproliferative and connective tissue disease. AIHA can also be induced by drags. AIHA is rarely associated with ulcerative colitis, occurring in approximately 0.6 to 1.7% of cases. (1,3,4) Because the severity of AIHA may range from indolent to acutely life threatening, the impetus to initiate treatment must begin with a thorough appraisal of symptoms and the severity of hemolysis. Rapidly developing anemia with a hematocrit of < 20 requires urgent management. AIHA is commonly treated with transfusion, corticosteroids, and splenectomy. Treatments for refractory anemia include immunosuppressive agents, IV Ig, plasma exchange, and danazol, which may have limited efficacy and delayed onset of action. (1,2,5) We present a case of an adult with life-threatening AIHA secondary to ulcerative colitis emergently managed with neuromuscular paralysis, induced hypothermia, and splenic embolization.


A 43-year-old woman, status-post total colectomy in 1991 for ulcerative colitis and orthotopic liver transplant in 1999 for sclerosing cholangitis, was transferred from an outside hospital with a 2-day history of nausea and vomiting and 1-day history of jaundice. Physical examination revealed intact mental status, jaundice, and pallor. CBC count revealed a hemoglobin of 4.6 g/dL with a reticulocyte count of 42.5%, a WBC count of 17.2 x [10.sup.9]/L, with 68% neutrophils and 12% bands, and a platelet count of 368 x [10.sup.9]/L. Spherocytes were present on peripheral smear. Blood chemistry revealed increased total bilirubin of 5.5 mg/dL (<0.1 mg/dL conjugated bilirubin) and lactate dehydrogenase (LDH) of 922 IU/L. Serum haptoglobin was <5.0 mg/dL. The direct antiglobulin test (direct Coombs test) was positive for C3 and IgG. A diagnosis of severe AIHA was made. The patient was started on oral corticosteroids (prednisolone, 60 mg/d), and cross-matched blood was ordered. Due to the presence of alloantibodies and antoantibodies, it was extremely difficult to find blood for the patient.

On the second clay of hospitalization, the patient became symptomatic secondary to tissue hypoxia. Physical examination revealed tachycardia (120 to 130 beats/min), tachypnea (20 to 26 breaths/min), mild hypotension (100/53 mm Hg), temperature of 37.4[degrees]C, and a markedly depressed level of consciousness. CBC count showed hemoglobin of 3.3 g/dL, and blood chemistry revealed a lactate level of 6.2 mg/dL. At this time, the patient was urgently admitted to the liver transplant ICU. She was intubated and placed on mechanical ventilation (fraction of inspired oxygen, 100%.) In order to decrease oxygen uptake (V[O.sub.2]), the patient was sedated and paralyzed (with propofol and vecuronium) and actively cooled with a cooling blanket and cooled IV crystalloids. In addition, the patient received 1 g of methylprednisolone IV. Secondary to these interventions, the patient's pulse rate decreased to the upper 80s, temperature decreased to 33.8[degrees]C, BP decreased slightly to 90/53 mm Hg, and lactate level decreased to 1.0 mg/dL. The patient was then slowly transfused with 1 U of blood, crossed-matched as closely as possible, when it became available later in the day. The hemoglobin level subsequently increased to 3.9 g/dL, and LDH further increased to 1,058 IU/L. Given the severity of the AIHA an d the paucity of blood available for transfusion, it was decided that the patient would likely benefit from splenic embolization.

Embolization of the splenic artery, with polyvinyl alcohol particles, was performed the following day in the interventional radiology suite. The patient tolerated the procedure well. On the day of the splenic embolization, the patient subsequently received three more units of packed RBCs (PRBCs). The hemoglobin level increased to 9.4 g/dL (hematocrit 26.5), and LDH increased to 2,333 IU/L. Following the blood transfusion, the neuromuscular blocking agent was discontinued and the propofol gradually weaned off The patient was extubated the following day. The patient's temperature, hemoglobin concentration, LDH, and lactate levels over this time period are illustrated in Figure 1. After extubation, the patient complained of abdominal pain, which was managed with oral narcotics. The pain subsided over the course of 3 days. An abdominal CT performed 2 days after the embolization demonstrated marked splenic heterogeneity with multiple central and peripheral hypodense regions consistent with multifocal infarctions (Fig 2). Two accessory spleens within the splenic hilum also demonstrated heterogeneity, indicating multiple infarcts. The LDH level remained stable for 3 to 4 days and began to steadily decrease approximately 4 days after splenic embolization until it fell into the normal range. Although there was no evidence of further hemolysis, the hematocrit continued to decrease secondary to GI bleeding from an antral ulcer, which subsequently necessitated antrectomy. While in surgery, the patient also underwent a prophylactic splenectomy. There has been no recurrence of the AIHA.



The treatment of life-threatening AIHA must be based on the severity of the patient's presentation. In the setting of an altered mental status and rising lactate level, the patient must be managed very aggressively in order to optimize oxygen delivery and minimize V[O.sub.2]. For this reason, our patient was sedated, paralyzed, intubated, and cooled. With these initial interventions, the patient's lactate decreased significantly and rapidly, demonstrating almost immediate efficacy. Although controlled mechanical ventilation reduces V[O.sub.2], neuromuscular paralysis has been demonstrated to further reduce V[O.sub.2] and improve tissue oxygenation in patients with evidence of tissue hypoxia. (6) Furthermore, neuromuscular paralysis is required to facilitate induced hypothermia. Moderate hypothermia decreases V[O.sub.2] by decreasing the basal metabolic rate. In patients with acute ischemic stroke, Bardutzky and colleagues (7) demonstrated that the ratio of total energy expenditure to predicted basal energy expenditure declined from 1.01 before induction of hypothermia (to 33[degrees]C) to an average of 0.74 during steady state of hypothermia.

AIHA is commonly treated with transfusion, corticosteroids, and splenectomy. Management of AIHA in the setting of ulcerative colitis is generally the same as in other diseases with the exception of the role of colectomy. Colectomy results in a short-term remission in most patients; treatment that does not include colectomy results in a remission rate of approximately 50%. (3,4.8,9) However, as in our case, hemolysis can occur years after total colectomy, indicating that colectomy is not necessarily protective.

In our patient, transfusion with PRBCs was made difficult due to the presence of alloantibodies and autoantibodies, and was of only limited efficacy due to the ongoing hemolysis. The patient received high-dose methylprednisolone to blunt the ongoing immune response. (1,2) Although the onset of the response to corticosteroids is usually rapid and significant hematologic improvement can become evident within a few days, (5) a more aggressive course was desirable in our patient due to the life-threatening nature of the anemia.

Splenectomy is usually considered the second-line treatment in surgical candidates in whom glucocorticoid therapy is unsuccessful. (1,2,5) Removal of the spleen theoretically has a twofold effect. First, because IgG antibodies predominantly mediate AIHA, it removes the primary site of extravascular hemolysis. Less importantly, the spleen is a site of antibody production. Splenectomy in patients with AIHA and underlying systemic diseases has decreased efficacy and increased surgical morbidity compared to patients with idiopathic AIHA, with only 19% of patients having a complete response and 37% a partial response. This compares to an 82% complete response and 18% partial responses in patients with idiopathic AIHA. (10) Furthermore, patients with underlying disease have an increased incidence of postoperative complications mostly in the form of bacterial infections. (10) The most serious adverse effect of splenectomy is overwhelming postsplenectomy infection (OPSI). Although published estimates of the incidence of OPSI vary, (11-13) and most of the published data antedate the widespread availability of the pneumococcal and Haemophilus influenzae vaccines, OPSI remains an important consideration when contemplating splenectomy. Laproscopic splenectomy, though associated with decreased incidence of postoperative complications, (14) may not be a safe option in critically ill patients.

Surgical morbidity was expected to be high in our patient, preventing urgent splenectomy. An alternative approach to decrease the function of the spleen in a critically ill patient is splenic embolization. Partial splenic embolization has been shown to be a safe and effective alternative to splenectomy or partial splenectomy for alleviating hypersplenism in the treatment of hereditary spherocytosis, (15) and in children with thalassemia. (16.17) Partial splenic embolization has also been successfully used in an infant with autoimmune hemolytic anemia. (18) Described complications of splenic embolization include postembolization syndrome (ie, pain, fever, vomiting), spontaneous rupture, and splenic abscess. (19,20) In two studies (21,22) of patients undergoing postembolization CT scans to identify complications secondary to embolization, only 2 of 96 patients and 1 of 53 patients, respectively, were found to have a splenic abscess. Furthermore, while partial splenic embolization will decrease the hemolytic rate, it does not destroy the phagocytic function of the spleen, thereby avoiding the risk of OPSI. In our patient, the LDH level rapidly stabilized following splenic embolization, reflecting decreased hemolysis.

Other therapies available for the treatment of AIHA include immunosuppressive drugs such as cyclophosphamide and azathioprine, danazol, and high-dose IV [gamma]-globulin. (1,2,5,9) However, these therapies may require days to weeks to become effective. (1,5) Plasma exchange may be indicated for acute reversal of severe hemolysis while other therapies are taking effect. However, due to the continuous antibody production and large extravascular distribution of IgG, it is of limited efficacy. (23) In conclusion, this case report highlights the potential for induced hypothermia and splenic embolization as useful rescue therapy in the management of life-threatening AIHA, especially in the setting of secondary AIHA, and a safe alternative to splenectomy in an unstable patient.

* From the Department of Critical Care Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA.


(1) Schwartz RS, Berkman EM, Silberstein LE. Autoimmune hemolytic anemia. In: Hoffman R, ed. Hematology: basic principles and practice, 3rd ed. Orlando, FL: Churchill Livingstone, 2000; 611-626

(2) Gehrs BC, Friedberg RC. Autoimmune hemolytic anemia. Am J Hematol 2002; 69:258-271

(3) Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises: Case 7-2000. A 23-year-old man with hemolytic anemia and bloody diarrhea. N Engl J Med 2000; 342:722-728

(4) Giannadaki E, Potamianos S, Roussomoustakaki M, et al. Autoimmune hemolytic anemia and positive Coombs test associated with ulcerative colitis. Am J Gastroenterol 1997; 92:1872-1874

(5) Petz LD. Treatment of autoimmune hemolytic anemias. Curr Opin Hematol 2001; 8:411-416

(6) Marik PE, Kaufman D. The effects of neuromuscular paralysis on systemic and splanchnic oxygen utilization in mechanically ventilated patients. Chest 1996; 109:1038-1042

(7) Bardutzky J, Georgiadis D, Kollmar R, et al. Energy expenditure in ischemic stroke patients treated with moderate hypothermia. Intensive Care Med 2004; 30:151-154

(8) Murphy PT, Cunney R, Nolan A, et al. Autoimmune haemolyric anaemia associated with ulcerative colitis. Irish Med J 1996; 89:172-173

(9) Petz LD, Garratty G. Immune hemolytic anemia, 2nd ed. Orlando, FL: Churchill Livingstone, 2003

(10) Akpek G, McAneny D, Weintraub L. Comparative response to splenectomy in Coombs-positive autoimmune hemolytic anemia with or without associated disease. Am J Hematol 1999; 61:98-102

(11) Brigden ML, Pattullo AL. Prevention and management of overwhelming postsplenectomy infection: an update. Crit Care Med 1999; 27:836-842

(12) Schilling RF. Estimating the risk for sepsis after splenectomy in hereditary spherocytosis. Ann Intern Med 1995; 122:187-188

(13) Schwartz PE, Sterioff S, Mucha P, et al. Postsplenectomy sepsis and mortality in adults. JAMA 1982; 248:2279-2283

(14) Flowers JL, Lefor AT, Steers J, et al. Laparoscopic splenectomy in patients with hematologic diseases. Ann Surg 1996; 224:19-28

(15) Kimura F, Ito H, Shimizu H, Togawa A, et al. Partial splenic embolization for the treatment of hereditary spherocytosis. AJR Am J Roentgenol 2003; 181:1021-1024

(16) Stanley P, Shen TC. Partial embolization of the spleen in patients with thalassemia. J Vase Intervent Radiol 1995; 6:137-142

(17) Pinca A, Di Palma A, Soriani S, et al. Effectiveness of partial splenic embolization as treatment for hypersplenism in thalassaemia major: a 7-year follow up. Eur J Haematol 1992; 49:49-52

(18) Taniuchi S, Fujita A, Iwamoto Y, et al. Successful use of partial splenic embolization in an infant with autoimmune hemolytic anemia. Pediatr Hematol Oncol 1993; 10:105-107

(19) Sangro B, Bilbao I, Herrero I, et al. Partial splenic embolization for the treatment of hypersplenism in cirrhosis. Hepatology 1993; 18:309-314

(20) Wholey MH, Chamorro HA, Rao G, et al. Splenic infarction and spontaneous rapture of the spleen after therapeutic embolization. Cardiovase Radiol 1978; 1:249-253

(21) Haan J, Bochicchio G, Kramer M, et al. Air following splenic embolization: infection or incidental finding? Am Surg 2003; 69:1036-1039

(22) Killeen KL, Shanmuganathan K, Boyd-Kranis R, et al. CT findings after embolization for blunt splenic trauma, J Vase Intervent Radiol 2001; 12:209-214

(23) Koo AP. Therapeutic apheresis in autoimmune and rheumatic diseases. J Clin Apheresis 2000; 15:18-27

Manuscript received June 1, 2004; revision accepted August 16, 2004.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (e-mail:

Correspondence to: Paul Marik, MD, FCCP, Professor of Critical Care and Medicine, Department of Critical Care, University of Pittsburgh, 640A Scaife Hall, 3550 Terrace St, Pittsburgh, PA 15261; e-mail:

COPYRIGHT 2005 American College of Chest Physicians
COPYRIGHT 2005 Gale Group

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