Guest guest Posted March 28, 2009 Report Share Posted March 28, 2009 I have been looking at previous posts trying to come up with an answer and was not able to find what I needed; I am sure this has already been discussed sometime in the history of this group so I apologize ahead of time for asking the question again. Suzanne is getting an ERCP on Monday because of significant blockage of her main bile duct. Dr. Shneider from Childrens of Pgh showed me her MRCP from last year and it was the first time I realized that the bile duct that was affected was the main bile duct, not the smaller " branches " (using Dr. Shneider's description). I have read that small duct PSC is a slower progressing type. What about what I am describing in Suzanne? Thank you, LINDA (Mom of Suzanne, Indeterminant Colitis, 1/04; PSC 3/04; j-pouch 2/07) Quote Link to comment Share on other sites More sharing options...
Guest guest Posted March 28, 2009 Report Share Posted March 28, 2009 Dear ; I can recommend this article as a source for some of the information that you are looking for: LaRusso NF, Shneider BL, Black D, Gores GJ, SP, Doo E, Hoofnagle JH 2006 Primary sclerosing cholangitis: summary of a workshop. Hepatology 44: 746-764 http://www.niddk.nih.gov/fund/other/PSCSept2006.pdf " PSC typically affects both intra- and extra-hepatic bile ducts. " Small duct PSC " refers to disease that affects bile ducts that are too small to be identified by endoscopic retrograde cholangiopancreatography (ERCP).7,8 Among adults with PSC, 75% have involvement of both small and large ducts, 15% small ducts only, and 10% large ducts only. Small duct PSC typically presents with insidious onset of symptoms or with end-stage liver disease. Episodes of cholangitis are rare. Small duct PSC can be associated with IBD, has a more favorable prognosis, and is less likely than large duct PSC to lead to cholangiocarcinoma.9 Many patients with PSC have IBD as well, typically ulcerative colitis and less commonly Crohn's disease with colonic involvement.10 IBD is diagnosed before PSC in 75% of cases and afterward in the remainder. Indeed, the diagnosis of IBD may not become evident until after liver transplantation for end-stage PSC; and conversely, PSC may not become apparent in patients with ulcerative colitis until after colectomy. There is little or no correlation between the severity of PSC and that of the associated IBD. Furthermore, therapy of IBD has little effect on the course of PSC, and vice versa. Other less common disease associations are thyroiditis, ankylosing spondylitis, and celiac disease. PSC with autoimmune hepatitis-like features has been referred to as autoimmune cholangitis.11,12 These patients usually present with high serum alanine aminotransferase (ALT) levels, modest or no elevations in serum alkaline phosphatase, high titers of antinuclear (ANA) and antismooth muscle antibodies (SMA) and liver histology typical of autoimmune hepatitis. Corticosteroid therapy may lead to improvements in symptoms and liver enzyme abnormalities. Eventually, however, patients become resistant to therapy and serum enzymes rise again, with prominent elevations in alkaline phosphatase and gamma glutamyl transpeptidase (GGT). Thus, PSC is a heterogeneous condition that can present with distinct clinical patterns that may have important implications for pathogenesis, prognosis and therapy. " So, I think they are possibly distinguishing here between small-duct PSC, large-duct PSC, small-duct + large-duct PSC, and PSC/AIH (autoimmune cholangitis). Perhaps these should also be sub-divided further in terms of whether the patient has celiac disease, ulcerative colitis, Crohn's disease or no IBD at all? Other types of sclerosing cholangitis (SC) (not discussed to any extent in this article, but possibly relevant to understanding pathogenesis (?)) include: Segmental PSC associated with Sjogren's syndrome: Guerrero P, M, Conde JM, Castro M, MC, Castilla L, Guillen P 1991 Primary segmental sclerosing cholangitis associated with Sjogren's syndrome. Rev. Esp. Enferm. Dig. 79: 363-366. http://www.ncbi.nlm.nih.gov/pubmed/1867927 Neonatal sclerosing cholangitis: Hadj-Rabia S, Baala L, Vabres P, Hamel-Teillac D, Jacquemin E, Fabre M, Lyonnet S, De Prost Y, Munnich A, Hadchouel M, Smahi A 2004 Claudin-1 gene mutations in neonatal sclerosing cholangitis associated with ichthyosis: a tight junction disease. Gastroenterology 127: 1386-1390. http://www.ncbi.nlm.nih.gov/pubmed/15521008 (P)SC associated with hyperimmunoglobulin M immunodeficiency: DiPalma JA, Strobel CT, Farrow JG 1986 Primary sclerosing cholangitis associated with hyperimmunoglobulin M immunodeficiency (dysgammaglobulinemia). Gastroenterology 91: 464-468. http://www.ncbi.nlm.nih.gov/pubmed/3721129 (P)SC associated with isolated IgA deficiency: Wagner A, Eichmann D 1989 Primary sclerosing cholangitis in isolated IgA deficiency. Schweiz. Med. Wochenschr. 119: 835-838. (P)SC associated with Wiskott-Aldrich syndrome: Kahn K, Sharp H, Hunter D, Kerzner B, Jessurun J, Blaese M 2001 Primary sclerosing cholangitis in Wiskott-Aldrich syndrome. J. Pediatr. Gastroenterol. Nutr. 32: 95-99. http://www.ncbi.nlm.nih.gov/pubmed/11176335 (P)SC associated with Riedel's thyroiditis and/or retroperitoneal fibrosis: De Boer WA 1993 Riedel's thyroiditis, retroperitoneal fibrosis, and sclerosing cholangitis: diseases with one pathogenesis? Gut 34: 714. http://www.ncbi.nlm.nih.gov/pubmed/8504980 Hatanaka H, Suzuki M, Onodera H, Ukai K, Kayaba Y, Sasaki A, Takahashi K, Kagaya H, Kikuchi T, Oriuchi T, Asano N, Kinouchi Y, Ueno Y 2002 A case of primary sclerosing cholangitis associated with retroperitoneal fibrosis. Nippon Shokakibyo Gakkai Zasshi 99: 1360-1365. http://www.ncbi.nlm.nih.gov/pubmed/12462057 SC associated with systemic lymphadenopathy: Kazumori H, Hashimoto T, Akagi S, Adachi K, Watanabe M, Kinoshita Y 2001 Sclerosing cholangitis and systemic lymphadenopathy. J. Gastroenterol. 36: 429-432. http://www.ncbi.nlm.nih.gov/pubmed/11428591 Hypereosinophilic sclerosing cholangitis: al-Abdulla NA, Schulick RD, Regan F 2000 Hypereosinophilic sclerosing cholangitis: findings using half-Fourier magnetic resonance imaging. Hepatogastroenterology 47: 359-361. http://www.ncbi.nlm.nih.gov/pubmed/10791189 Grauer L, Padilla VM 3rd, Bouza L, Barkin JS 1993 Eosinophilic sclerosing cholangitis associated with hypereosinophilic syndrome. Am. J. Gastroenterol. 88: 1764-1769. http://www.ncbi.nlm.nih.gov/pubmed/8213721 Ichikawa N, Taniguchi A, Akama H, Ishiguro H, Kurihara T, Terai C, Hara M, Kashiwazaki S 1997 Sclerosing cholangitis associated with hypereosinophilic syndrome. Intern. Med. 36: 561-564. http://www.ncbi.nlm.nih.gov/pubmed/9260773 (P)SC associated with sarcoidosis: Alam I, Levenson SD, Ferrell LD, Bass NM 1997 Diffuse intrahepatic biliary strictures in sarcoidosis resembling sclerosing cholangitis. Case report and review of the literature. Dig. Dis. Sci. 42: 1295-1301. http://www.ncbi.nlm.nih.gov/pubmed/9201098 Ilan Y, Rappaport I, Feigin R, Ben-Chetrit E 1993 Primary sclerosing cholangitis in sarcoidosis. J. Clin. Gastroenterol. 16: 326-328. http://www.ncbi.nlm.nih.gov/pubmed/8331269 Ishak KG 1998 Sarcoidosis of the liver and bile ducts. Mayo Clin. Proc. 73: 467-472. http://www.ncbi.nlm.nih.gov/pubmed/9581591 SC associated with cystic fibrosis: Benett I, Salh B, Haboubi NY, Braganza JM 1989 Sclerosing cholangitis with hepatic microvesicular steatosis in cystic fibrosis and chronic pancreatitis. J. Clin. Pathol. 42: 466-469. http://www.ncbi.nlm.nih.gov/pubmed/2732340 Durieu I, Pellet O, Simonot L, Durupt S, Bellon G, Durand DV, Minh VA 1999 Sclerosing cholangitis in adults with cystic fibrosis: a magnetic resonance cholangiographic prospective study. J. Hepatol. 30: 1052-1056. http://www.ncbi.nlm.nih.gov/pubmed/10406183 IgG4-associated (P)SC: Bjornsson E 2008 Immunoglobulin G4-associated cholangitis. Curr. Opin. Gastroenterol. 24: 389-394. http://www.ncbi.nlm.nih.gov/pubmed/18408470 Erdogan D, Kloek JJ, Ten Kate FJ, Rauws EA, Busch OR, Gouma DJ, van Gulik TM 2008 Immunoglobulin G4-related sclerosing cholangitis in patients resected for presumed malignant bile duct strictures. Br. J. Surg. 95: 727-734. http://www.ncbi.nlm.nih.gov/pubmed/18418862 Frossard JL, Spahr L, Rubbia-Brandt L, Hadengue A 2008 Biliary, pancreatic and systemic diseases associated with IgG4. Rev. Med. Suisse. 4: 1856-1858. http://www.ncbi.nlm.nih.gov/pubmed/18831404 Iida Y, Onitsuka A, Katagiri Y 2009 Immunoglobulin G4-related sclerosing cholangitis without pancreatic involvement. Dig. Surg. 26: 117-118. http://www.ncbi.nlm.nih.gov/pubmed/19262061 (P)SC associated with autoimmune pancreatitis: Deshpande V, Mino-Kenudson M, Brugge W, Lauwers GY 2005 Autoimmune pancreatitis: more than just a pancreatic disease? A contemporary review of its pathology. Arch. Pathol. Lab. Med. 129: 1148-1154. http://www.ncbi.nlm.nih.gov/pubmed/16119989 Eerens I, Vanbeckevoort D, Vansteenbergen W, Van Hoe L 2001 Autoimmune pancreatitis associated with primary sclerosing cholangitis: MR imaging findings. Eur. Radiol. 11: 1401-1404. http://www.ncbi.nlm.nih.gov/pubmed/11519549 Hamano H, Kawa S, Uehara T, Ochi Y, Takayama M, Komatsu K, Muraki T, Umino J, Kiyosawa K, Miyagawa S 2005 Immunoglobulin G4-related lymphoplasmacytic sclerosing cholangitis that mimics infiltrating hilar cholangiocarcinoma: part of a spectrum of autoimmune pancreatitis? Gastrointest. Endosc. 62: 152-157. http://www.ncbi.nlm.nih.gov/pubmed/15990840 SC associated with Langerhans cell histiocytosis: Braier J, Ciocca M, Latella A, de Davila MG, Drajer M, Imventarza O 2002 Cholestasis, sclerosing cholangitis, and liver transplantation in Langerhans cell histiocytosis. Med. Pediatr. Oncol. 38: 178-182. http://www.ncbi.nlm.nih.gov/pubmed/11836717 Gey T, Bergoin C, Just N, Paupard T, Cazals-Hatem D, Xuan KH, Tavernier JY, Wallaert B 2004 Langerhans cell histiocytosis and sclerosing cholangitis in adults. Rev. Mal. Respir. 21: 997-1000. http://www.ncbi.nlm.nih.gov/pubmed/15622348 and this is by no means a complete list! Best regards, Dave (father of (23); PSC 07/03; UC 08/03) Quote Link to comment Share on other sites More sharing options...
Guest guest Posted March 28, 2009 Report Share Posted March 28, 2009 Dear ; I can recommend this article as a source for some of the information that you are looking for: LaRusso NF, Shneider BL, Black D, Gores GJ, SP, Doo E, Hoofnagle JH 2006 Primary sclerosing cholangitis: summary of a workshop. Hepatology 44: 746-764 http://www.niddk.nih.gov/fund/other/PSCSept2006.pdf " PSC typically affects both intra- and extra-hepatic bile ducts. " Small duct PSC " refers to disease that affects bile ducts that are too small to be identified by endoscopic retrograde cholangiopancreatography (ERCP).7,8 Among adults with PSC, 75% have involvement of both small and large ducts, 15% small ducts only, and 10% large ducts only. Small duct PSC typically presents with insidious onset of symptoms or with end-stage liver disease. Episodes of cholangitis are rare. Small duct PSC can be associated with IBD, has a more favorable prognosis, and is less likely than large duct PSC to lead to cholangiocarcinoma.9 Many patients with PSC have IBD as well, typically ulcerative colitis and less commonly Crohn's disease with colonic involvement.10 IBD is diagnosed before PSC in 75% of cases and afterward in the remainder. Indeed, the diagnosis of IBD may not become evident until after liver transplantation for end-stage PSC; and conversely, PSC may not become apparent in patients with ulcerative colitis until after colectomy. There is little or no correlation between the severity of PSC and that of the associated IBD. Furthermore, therapy of IBD has little effect on the course of PSC, and vice versa. Other less common disease associations are thyroiditis, ankylosing spondylitis, and celiac disease. PSC with autoimmune hepatitis-like features has been referred to as autoimmune cholangitis.11,12 These patients usually present with high serum alanine aminotransferase (ALT) levels, modest or no elevations in serum alkaline phosphatase, high titers of antinuclear (ANA) and antismooth muscle antibodies (SMA) and liver histology typical of autoimmune hepatitis. Corticosteroid therapy may lead to improvements in symptoms and liver enzyme abnormalities. Eventually, however, patients become resistant to therapy and serum enzymes rise again, with prominent elevations in alkaline phosphatase and gamma glutamyl transpeptidase (GGT). Thus, PSC is a heterogeneous condition that can present with distinct clinical patterns that may have important implications for pathogenesis, prognosis and therapy. " So, I think they are possibly distinguishing here between small-duct PSC, large-duct PSC, small-duct + large-duct PSC, and PSC/AIH (autoimmune cholangitis). Perhaps these should also be sub-divided further in terms of whether the patient has celiac disease, ulcerative colitis, Crohn's disease or no IBD at all? Other types of sclerosing cholangitis (SC) (not discussed to any extent in this article, but possibly relevant to understanding pathogenesis (?)) include: Segmental PSC associated with Sjogren's syndrome: Guerrero P, M, Conde JM, Castro M, MC, Castilla L, Guillen P 1991 Primary segmental sclerosing cholangitis associated with Sjogren's syndrome. Rev. Esp. Enferm. Dig. 79: 363-366. http://www.ncbi.nlm.nih.gov/pubmed/1867927 Neonatal sclerosing cholangitis: Hadj-Rabia S, Baala L, Vabres P, Hamel-Teillac D, Jacquemin E, Fabre M, Lyonnet S, De Prost Y, Munnich A, Hadchouel M, Smahi A 2004 Claudin-1 gene mutations in neonatal sclerosing cholangitis associated with ichthyosis: a tight junction disease. Gastroenterology 127: 1386-1390. http://www.ncbi.nlm.nih.gov/pubmed/15521008 (P)SC associated with hyperimmunoglobulin M immunodeficiency: DiPalma JA, Strobel CT, Farrow JG 1986 Primary sclerosing cholangitis associated with hyperimmunoglobulin M immunodeficiency (dysgammaglobulinemia). Gastroenterology 91: 464-468. http://www.ncbi.nlm.nih.gov/pubmed/3721129 (P)SC associated with isolated IgA deficiency: Wagner A, Eichmann D 1989 Primary sclerosing cholangitis in isolated IgA deficiency. Schweiz. Med. Wochenschr. 119: 835-838. (P)SC associated with Wiskott-Aldrich syndrome: Kahn K, Sharp H, Hunter D, Kerzner B, Jessurun J, Blaese M 2001 Primary sclerosing cholangitis in Wiskott-Aldrich syndrome. J. Pediatr. Gastroenterol. Nutr. 32: 95-99. http://www.ncbi.nlm.nih.gov/pubmed/11176335 (P)SC associated with Riedel's thyroiditis and/or retroperitoneal fibrosis: De Boer WA 1993 Riedel's thyroiditis, retroperitoneal fibrosis, and sclerosing cholangitis: diseases with one pathogenesis? Gut 34: 714. http://www.ncbi.nlm.nih.gov/pubmed/8504980 Hatanaka H, Suzuki M, Onodera H, Ukai K, Kayaba Y, Sasaki A, Takahashi K, Kagaya H, Kikuchi T, Oriuchi T, Asano N, Kinouchi Y, Ueno Y 2002 A case of primary sclerosing cholangitis associated with retroperitoneal fibrosis. Nippon Shokakibyo Gakkai Zasshi 99: 1360-1365. http://www.ncbi.nlm.nih.gov/pubmed/12462057 SC associated with systemic lymphadenopathy: Kazumori H, Hashimoto T, Akagi S, Adachi K, Watanabe M, Kinoshita Y 2001 Sclerosing cholangitis and systemic lymphadenopathy. J. Gastroenterol. 36: 429-432. http://www.ncbi.nlm.nih.gov/pubmed/11428591 Hypereosinophilic sclerosing cholangitis: al-Abdulla NA, Schulick RD, Regan F 2000 Hypereosinophilic sclerosing cholangitis: findings using half-Fourier magnetic resonance imaging. Hepatogastroenterology 47: 359-361. http://www.ncbi.nlm.nih.gov/pubmed/10791189 Grauer L, Padilla VM 3rd, Bouza L, Barkin JS 1993 Eosinophilic sclerosing cholangitis associated with hypereosinophilic syndrome. Am. J. Gastroenterol. 88: 1764-1769. http://www.ncbi.nlm.nih.gov/pubmed/8213721 Ichikawa N, Taniguchi A, Akama H, Ishiguro H, Kurihara T, Terai C, Hara M, Kashiwazaki S 1997 Sclerosing cholangitis associated with hypereosinophilic syndrome. Intern. Med. 36: 561-564. http://www.ncbi.nlm.nih.gov/pubmed/9260773 (P)SC associated with sarcoidosis: Alam I, Levenson SD, Ferrell LD, Bass NM 1997 Diffuse intrahepatic biliary strictures in sarcoidosis resembling sclerosing cholangitis. Case report and review of the literature. Dig. Dis. Sci. 42: 1295-1301. http://www.ncbi.nlm.nih.gov/pubmed/9201098 Ilan Y, Rappaport I, Feigin R, Ben-Chetrit E 1993 Primary sclerosing cholangitis in sarcoidosis. J. Clin. Gastroenterol. 16: 326-328. http://www.ncbi.nlm.nih.gov/pubmed/8331269 Ishak KG 1998 Sarcoidosis of the liver and bile ducts. Mayo Clin. Proc. 73: 467-472. http://www.ncbi.nlm.nih.gov/pubmed/9581591 SC associated with cystic fibrosis: Benett I, Salh B, Haboubi NY, Braganza JM 1989 Sclerosing cholangitis with hepatic microvesicular steatosis in cystic fibrosis and chronic pancreatitis. J. Clin. Pathol. 42: 466-469. http://www.ncbi.nlm.nih.gov/pubmed/2732340 Durieu I, Pellet O, Simonot L, Durupt S, Bellon G, Durand DV, Minh VA 1999 Sclerosing cholangitis in adults with cystic fibrosis: a magnetic resonance cholangiographic prospective study. J. Hepatol. 30: 1052-1056. http://www.ncbi.nlm.nih.gov/pubmed/10406183 IgG4-associated (P)SC: Bjornsson E 2008 Immunoglobulin G4-associated cholangitis. Curr. Opin. Gastroenterol. 24: 389-394. http://www.ncbi.nlm.nih.gov/pubmed/18408470 Erdogan D, Kloek JJ, Ten Kate FJ, Rauws EA, Busch OR, Gouma DJ, van Gulik TM 2008 Immunoglobulin G4-related sclerosing cholangitis in patients resected for presumed malignant bile duct strictures. Br. J. Surg. 95: 727-734. http://www.ncbi.nlm.nih.gov/pubmed/18418862 Frossard JL, Spahr L, Rubbia-Brandt L, Hadengue A 2008 Biliary, pancreatic and systemic diseases associated with IgG4. Rev. Med. Suisse. 4: 1856-1858. http://www.ncbi.nlm.nih.gov/pubmed/18831404 Iida Y, Onitsuka A, Katagiri Y 2009 Immunoglobulin G4-related sclerosing cholangitis without pancreatic involvement. Dig. Surg. 26: 117-118. http://www.ncbi.nlm.nih.gov/pubmed/19262061 (P)SC associated with autoimmune pancreatitis: Deshpande V, Mino-Kenudson M, Brugge W, Lauwers GY 2005 Autoimmune pancreatitis: more than just a pancreatic disease? A contemporary review of its pathology. Arch. Pathol. Lab. Med. 129: 1148-1154. http://www.ncbi.nlm.nih.gov/pubmed/16119989 Eerens I, Vanbeckevoort D, Vansteenbergen W, Van Hoe L 2001 Autoimmune pancreatitis associated with primary sclerosing cholangitis: MR imaging findings. Eur. Radiol. 11: 1401-1404. http://www.ncbi.nlm.nih.gov/pubmed/11519549 Hamano H, Kawa S, Uehara T, Ochi Y, Takayama M, Komatsu K, Muraki T, Umino J, Kiyosawa K, Miyagawa S 2005 Immunoglobulin G4-related lymphoplasmacytic sclerosing cholangitis that mimics infiltrating hilar cholangiocarcinoma: part of a spectrum of autoimmune pancreatitis? Gastrointest. Endosc. 62: 152-157. http://www.ncbi.nlm.nih.gov/pubmed/15990840 SC associated with Langerhans cell histiocytosis: Braier J, Ciocca M, Latella A, de Davila MG, Drajer M, Imventarza O 2002 Cholestasis, sclerosing cholangitis, and liver transplantation in Langerhans cell histiocytosis. Med. Pediatr. Oncol. 38: 178-182. http://www.ncbi.nlm.nih.gov/pubmed/11836717 Gey T, Bergoin C, Just N, Paupard T, Cazals-Hatem D, Xuan KH, Tavernier JY, Wallaert B 2004 Langerhans cell histiocytosis and sclerosing cholangitis in adults. Rev. Mal. Respir. 21: 997-1000. http://www.ncbi.nlm.nih.gov/pubmed/15622348 and this is by no means a complete list! Best regards, Dave (father of (23); PSC 07/03; UC 08/03) Quote Link to comment Share on other sites More sharing options...
Guest guest Posted March 28, 2009 Report Share Posted March 28, 2009 Dear ; I can recommend this article as a source for some of the information that you are looking for: LaRusso NF, Shneider BL, Black D, Gores GJ, SP, Doo E, Hoofnagle JH 2006 Primary sclerosing cholangitis: summary of a workshop. Hepatology 44: 746-764 http://www.niddk.nih.gov/fund/other/PSCSept2006.pdf " PSC typically affects both intra- and extra-hepatic bile ducts. " Small duct PSC " refers to disease that affects bile ducts that are too small to be identified by endoscopic retrograde cholangiopancreatography (ERCP).7,8 Among adults with PSC, 75% have involvement of both small and large ducts, 15% small ducts only, and 10% large ducts only. Small duct PSC typically presents with insidious onset of symptoms or with end-stage liver disease. Episodes of cholangitis are rare. Small duct PSC can be associated with IBD, has a more favorable prognosis, and is less likely than large duct PSC to lead to cholangiocarcinoma.9 Many patients with PSC have IBD as well, typically ulcerative colitis and less commonly Crohn's disease with colonic involvement.10 IBD is diagnosed before PSC in 75% of cases and afterward in the remainder. Indeed, the diagnosis of IBD may not become evident until after liver transplantation for end-stage PSC; and conversely, PSC may not become apparent in patients with ulcerative colitis until after colectomy. There is little or no correlation between the severity of PSC and that of the associated IBD. Furthermore, therapy of IBD has little effect on the course of PSC, and vice versa. Other less common disease associations are thyroiditis, ankylosing spondylitis, and celiac disease. PSC with autoimmune hepatitis-like features has been referred to as autoimmune cholangitis.11,12 These patients usually present with high serum alanine aminotransferase (ALT) levels, modest or no elevations in serum alkaline phosphatase, high titers of antinuclear (ANA) and antismooth muscle antibodies (SMA) and liver histology typical of autoimmune hepatitis. Corticosteroid therapy may lead to improvements in symptoms and liver enzyme abnormalities. Eventually, however, patients become resistant to therapy and serum enzymes rise again, with prominent elevations in alkaline phosphatase and gamma glutamyl transpeptidase (GGT). Thus, PSC is a heterogeneous condition that can present with distinct clinical patterns that may have important implications for pathogenesis, prognosis and therapy. " So, I think they are possibly distinguishing here between small-duct PSC, large-duct PSC, small-duct + large-duct PSC, and PSC/AIH (autoimmune cholangitis). Perhaps these should also be sub-divided further in terms of whether the patient has celiac disease, ulcerative colitis, Crohn's disease or no IBD at all? Other types of sclerosing cholangitis (SC) (not discussed to any extent in this article, but possibly relevant to understanding pathogenesis (?)) include: Segmental PSC associated with Sjogren's syndrome: Guerrero P, M, Conde JM, Castro M, MC, Castilla L, Guillen P 1991 Primary segmental sclerosing cholangitis associated with Sjogren's syndrome. Rev. Esp. Enferm. Dig. 79: 363-366. http://www.ncbi.nlm.nih.gov/pubmed/1867927 Neonatal sclerosing cholangitis: Hadj-Rabia S, Baala L, Vabres P, Hamel-Teillac D, Jacquemin E, Fabre M, Lyonnet S, De Prost Y, Munnich A, Hadchouel M, Smahi A 2004 Claudin-1 gene mutations in neonatal sclerosing cholangitis associated with ichthyosis: a tight junction disease. Gastroenterology 127: 1386-1390. http://www.ncbi.nlm.nih.gov/pubmed/15521008 (P)SC associated with hyperimmunoglobulin M immunodeficiency: DiPalma JA, Strobel CT, Farrow JG 1986 Primary sclerosing cholangitis associated with hyperimmunoglobulin M immunodeficiency (dysgammaglobulinemia). Gastroenterology 91: 464-468. http://www.ncbi.nlm.nih.gov/pubmed/3721129 (P)SC associated with isolated IgA deficiency: Wagner A, Eichmann D 1989 Primary sclerosing cholangitis in isolated IgA deficiency. Schweiz. Med. Wochenschr. 119: 835-838. (P)SC associated with Wiskott-Aldrich syndrome: Kahn K, Sharp H, Hunter D, Kerzner B, Jessurun J, Blaese M 2001 Primary sclerosing cholangitis in Wiskott-Aldrich syndrome. J. Pediatr. Gastroenterol. Nutr. 32: 95-99. http://www.ncbi.nlm.nih.gov/pubmed/11176335 (P)SC associated with Riedel's thyroiditis and/or retroperitoneal fibrosis: De Boer WA 1993 Riedel's thyroiditis, retroperitoneal fibrosis, and sclerosing cholangitis: diseases with one pathogenesis? Gut 34: 714. http://www.ncbi.nlm.nih.gov/pubmed/8504980 Hatanaka H, Suzuki M, Onodera H, Ukai K, Kayaba Y, Sasaki A, Takahashi K, Kagaya H, Kikuchi T, Oriuchi T, Asano N, Kinouchi Y, Ueno Y 2002 A case of primary sclerosing cholangitis associated with retroperitoneal fibrosis. Nippon Shokakibyo Gakkai Zasshi 99: 1360-1365. http://www.ncbi.nlm.nih.gov/pubmed/12462057 SC associated with systemic lymphadenopathy: Kazumori H, Hashimoto T, Akagi S, Adachi K, Watanabe M, Kinoshita Y 2001 Sclerosing cholangitis and systemic lymphadenopathy. J. Gastroenterol. 36: 429-432. http://www.ncbi.nlm.nih.gov/pubmed/11428591 Hypereosinophilic sclerosing cholangitis: al-Abdulla NA, Schulick RD, Regan F 2000 Hypereosinophilic sclerosing cholangitis: findings using half-Fourier magnetic resonance imaging. Hepatogastroenterology 47: 359-361. http://www.ncbi.nlm.nih.gov/pubmed/10791189 Grauer L, Padilla VM 3rd, Bouza L, Barkin JS 1993 Eosinophilic sclerosing cholangitis associated with hypereosinophilic syndrome. Am. J. Gastroenterol. 88: 1764-1769. http://www.ncbi.nlm.nih.gov/pubmed/8213721 Ichikawa N, Taniguchi A, Akama H, Ishiguro H, Kurihara T, Terai C, Hara M, Kashiwazaki S 1997 Sclerosing cholangitis associated with hypereosinophilic syndrome. Intern. Med. 36: 561-564. http://www.ncbi.nlm.nih.gov/pubmed/9260773 (P)SC associated with sarcoidosis: Alam I, Levenson SD, Ferrell LD, Bass NM 1997 Diffuse intrahepatic biliary strictures in sarcoidosis resembling sclerosing cholangitis. Case report and review of the literature. Dig. Dis. Sci. 42: 1295-1301. http://www.ncbi.nlm.nih.gov/pubmed/9201098 Ilan Y, Rappaport I, Feigin R, Ben-Chetrit E 1993 Primary sclerosing cholangitis in sarcoidosis. J. Clin. Gastroenterol. 16: 326-328. http://www.ncbi.nlm.nih.gov/pubmed/8331269 Ishak KG 1998 Sarcoidosis of the liver and bile ducts. Mayo Clin. Proc. 73: 467-472. http://www.ncbi.nlm.nih.gov/pubmed/9581591 SC associated with cystic fibrosis: Benett I, Salh B, Haboubi NY, Braganza JM 1989 Sclerosing cholangitis with hepatic microvesicular steatosis in cystic fibrosis and chronic pancreatitis. J. Clin. Pathol. 42: 466-469. http://www.ncbi.nlm.nih.gov/pubmed/2732340 Durieu I, Pellet O, Simonot L, Durupt S, Bellon G, Durand DV, Minh VA 1999 Sclerosing cholangitis in adults with cystic fibrosis: a magnetic resonance cholangiographic prospective study. J. Hepatol. 30: 1052-1056. http://www.ncbi.nlm.nih.gov/pubmed/10406183 IgG4-associated (P)SC: Bjornsson E 2008 Immunoglobulin G4-associated cholangitis. Curr. Opin. Gastroenterol. 24: 389-394. http://www.ncbi.nlm.nih.gov/pubmed/18408470 Erdogan D, Kloek JJ, Ten Kate FJ, Rauws EA, Busch OR, Gouma DJ, van Gulik TM 2008 Immunoglobulin G4-related sclerosing cholangitis in patients resected for presumed malignant bile duct strictures. Br. J. Surg. 95: 727-734. http://www.ncbi.nlm.nih.gov/pubmed/18418862 Frossard JL, Spahr L, Rubbia-Brandt L, Hadengue A 2008 Biliary, pancreatic and systemic diseases associated with IgG4. Rev. Med. Suisse. 4: 1856-1858. http://www.ncbi.nlm.nih.gov/pubmed/18831404 Iida Y, Onitsuka A, Katagiri Y 2009 Immunoglobulin G4-related sclerosing cholangitis without pancreatic involvement. Dig. Surg. 26: 117-118. http://www.ncbi.nlm.nih.gov/pubmed/19262061 (P)SC associated with autoimmune pancreatitis: Deshpande V, Mino-Kenudson M, Brugge W, Lauwers GY 2005 Autoimmune pancreatitis: more than just a pancreatic disease? A contemporary review of its pathology. Arch. Pathol. Lab. Med. 129: 1148-1154. http://www.ncbi.nlm.nih.gov/pubmed/16119989 Eerens I, Vanbeckevoort D, Vansteenbergen W, Van Hoe L 2001 Autoimmune pancreatitis associated with primary sclerosing cholangitis: MR imaging findings. Eur. Radiol. 11: 1401-1404. http://www.ncbi.nlm.nih.gov/pubmed/11519549 Hamano H, Kawa S, Uehara T, Ochi Y, Takayama M, Komatsu K, Muraki T, Umino J, Kiyosawa K, Miyagawa S 2005 Immunoglobulin G4-related lymphoplasmacytic sclerosing cholangitis that mimics infiltrating hilar cholangiocarcinoma: part of a spectrum of autoimmune pancreatitis? Gastrointest. Endosc. 62: 152-157. http://www.ncbi.nlm.nih.gov/pubmed/15990840 SC associated with Langerhans cell histiocytosis: Braier J, Ciocca M, Latella A, de Davila MG, Drajer M, Imventarza O 2002 Cholestasis, sclerosing cholangitis, and liver transplantation in Langerhans cell histiocytosis. Med. Pediatr. Oncol. 38: 178-182. http://www.ncbi.nlm.nih.gov/pubmed/11836717 Gey T, Bergoin C, Just N, Paupard T, Cazals-Hatem D, Xuan KH, Tavernier JY, Wallaert B 2004 Langerhans cell histiocytosis and sclerosing cholangitis in adults. Rev. Mal. Respir. 21: 997-1000. http://www.ncbi.nlm.nih.gov/pubmed/15622348 and this is by no means a complete list! Best regards, Dave (father of (23); PSC 07/03; UC 08/03) Quote Link to comment Share on other sites More sharing options...
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