| Liver and Spleen Involvement |
| This page was written by Shelley Ensz, and has not yet been medically edited. Systemic Scleroderma (SD) affects everyone differently. Just because something is listed here does not mean that it is caused by systemic scleroderma, nor that an individual scleroderma patient will ever experience it. See Disclaimer. |
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| Overview |
| Liver "The liver is the largest gland in the vertebrate body, composed of a spongy mass of wedge-shaped lobes that has many metabolic and secretory functions. The liver secretes bile, a digestive fluid; metabolizes proteins, carbohydrates, and fats; stores glycogen, vitamins, and other substances; synthesizes blood-clotting factors; removes wastes and toxic matter from the blood; regulates blood volume; and destroys old red blood cells. It is essential to life." ENCYCLOPEDIA BRITANNICA |
| What are the diseases that affect the liver? Simple descriptions of dozens of liver diseases from Alagille Syndrome to Wilson's Disease. American Liver Foundation. |
| Liver function tests. These tests help in the diagnosis of any liver conditions, whether there are symptoms and signs like jaundice, itchiness, enlarged liver as well as the monitoring of the progress and severity of existing liver conditions. The Star Online: Lifestyle. 11/01/06. |
| D-penicillamine for primary sclerosing cholangitis. This research analysis found no sufficient evidence to support or refute the use of D-penicillamine for patients with primary sclerosing cholangitis and does not recommend the use of D-penicillamine for primary sclerosing cholangitis outside randomised trials. The Cochrane Database of Systematic Reviews 2006 Issue 1. |
| Hepatitis: Autoimmune (AIH) |
| Autoimmune liver disease. Autoimmune hepatitis can present as acute or chronic hepatitis and as allograft dysfunction after liver transplantation. PubMed.Curr Opin Gastroenterol. 2006 May;22(3):234-40. |
| Characteristics and Long-term Prognosis of the Autoimmune Hepatitis (AIH) /Primary Sclerosing Cholangitis (PSC) Overlap Syndrome. Overlap of AIH and PSC was detected most reliably on grounds of serologic markers and histology; early bile duct changes were often missed by endoscopic retrograde cholangiography. S. Luth, MD. Journal of Clinical Gastroenterology. January 2009. (Also see: Hepatitis, Autoimmune) |
| Type I Autoimmune Hepatitis Presenting With Acute Liver Failure in the Setting of Wild Mushroom Ingestion. Here, we report the unusual case of a woman in her seventh decade who presented with acute liver failure as her initial manifestation of autoimmune hepatitis, and who had been a long-time ingestor of hand-picked, wild mushrooms. Rogart, J. N. MD. Journal of Clinical Gastroenterology. July 2008. |
| A comparison of autoimmune liver disease in juvenile and adult populations with systemic lupus erythematosus—a retrospective review of cases. Our study confirms that autoimmune liver disease occurs infrequently in adult lupus patients but should be considered in a patient with persistent liver enzyme abnormalities. However, in our study there is a significantly higher prevalence in juvenile lupus patients. Rheumatology. Volume 46, Number 7 Pp. 1171-1173. (Also see: Lupus) |
| The development of connective tissue diseases (CTD) in patients with autoimmune hepatitis (AIH): a case series. Patients with AIH may be at increased risk for developing systemic CTD and patients with systemic CTD may be at increased risk of developing AIH. PubMed. Semin Arthritis Rheum. 2006 Jun;35(6):344-8. (Also see: Overlap: MCTD) |
| Thiopurine Methyltransferase Deficiency and Azathioprine Intolerance in Autoimmune Hepatitis. Routine screening of blood thiopurine methyltransferase levels has a low yield for identifying individual patients at risk for azathioprine toxicity during conventional low dose therapy for autoimmune hepatitis. PubMed. Digestive Diseases and Sciences. 06/14/06. |
| Pregnancy in Autoimmune Hepatitis: Outcome and Risk Factors. The presence of autoantibodies may be a risk factor for adverse pregnancy outcome in AIH patients. Close monitoring of both mother and fetus seems advisable due to a significant rate of maternal and fetal complications. PubMed: Am J Gastroenterol. 2006 Feb 8. |
| Scleroderma-like lesions in the course of autoimmune hepatitis. Case report. The presence of scleroedematous lesions of the skin accompanying chronic hepatic inflammation enabled us to establish the diagnosis of scleroderma-like lesions in the course of long lasting mild autoimmune hepatitis. PubMed. Przegl Lek. 2005;62(5):314-6. |
| Autoimmune hepatitis in India: profile of an uncommon disease. In India, autoimmune hepatitis is uncommon and usually presents with chronic hepatitis or cirrhosis, acute hepatitis being less common. PubMed. BMC Gastroenterol. 2005 Aug 15;5(1):27. |
| Hepatitis C (Viral Hepatitis) |
| Systemic sclerosis after interferon alphacon-1 therapy for hepatitis C. IFN has multiple effects on the immune system and is known to trigger the development of autoantibodies, as well as the onset or exacerbation of autoimmune disease. We suspect that the immunomodulatory effects of IFNalphacon-1 triggered the clinical manifestations of SSc in this patient. PubMed. Intern Med. 2007;46(8):473-6. (Also see: Causes of Scleroderma: Drugs and Medications) |
| Autoimmunity and hepatitis C. It is well recognized that not only are autoantibodies present in autoimmune hepatitis frequently detected in hepatitis C, but also that an array of immune-mediated symptoms and diseases occur in patients with chronic hepatitis C. This has prompted research aimed at identifying a link between hepatitis C and autoimmunity, and autoimmune hepatitis in particular. PubMed. Autoimmun Rev. 2003 Oct;2(6):322-31. |
| Systemic sclerosis developing in association with the use of interferon alpha therapy for chronic viral hepatitis. We speculate that the immunomodulatory effects of IFNalpha triggered the clinical manifestations of SSc in this patient. To our knowledge, this is the second case of SSc developing after therapy with IFNalpha and the first in a patient treated for chronic viral hepatitis C. PubMed. Clin Exp Rheumatol. 2004 Sep-Oct;22(5):625-8. (Also see: Causes of Scleroderma: Medications) |
| Triple association between hepatitis C virus infection, systemic autoimmune diseases, and B cell lymphoma. We describe 6 patients with a triple association of HCV infection, systemic autoimmune disease, and non-Hodgkin's lymphoma (NHL). This triple association reinforces the suspected links between autoimmunity, infection, and cancer. PubMed. J Rheumatol. 2004 Mar;31(3):495-9. (Also see: Associated Conditions: Cancer) |
| Hepatitis C virus, Sjogren's syndrome and B-cell lymphoma: linking infection, autoimmunity and cancer. Viruses have been proposed as possible etiologic or triggering agents of systemic autoimmune diseases, with hepatitis C virus (HCV) being one of the viruses most frequently associated with autoimmune features and with systemic autoimmune diseases such as mixed cryoglobulinemia or Sjogren's Syndrome. PubMed. Autoimmun Rev. 2005 Jan;4(1):8-15. (Also see: Cancer and Scleroderma, Sjogren's Syndrome, and Causes of Scleroderma: Infection) |
| A case of Behcet's disease with esophageal ulcers complicated with systemic sclerosis, chronic hepatitis C, and pancytopenia. This is a rare case of Behcet's disease complicated with esophageal ulcers, systemic sclerosis, chronic hepatitis C, and pancytopenia. Of interest is the mechanism of coincidence of these diseases from the pathological point of view. PubMed. Nihon Rinsho Meneki Gakkai Kaishi. 2004 Jun;27(3):164-70. (Also see: Behcet's and Pancytopenia) |
| Liver Cancer |
| Risk of malignancy in scleroderma: A population-based cohort study. Contrary to previous studies, this study did not find statistical evidence of an increased incidence of cancer in scleroderma patients, except for liver cancer. It may be necessary to consider local cancer rates when comparing different scleroderma cohorts. PubMed. Arthritis Rheum. 2005 Jul 28;52(8):2415-2424. (Also see: Scleroderma and Cancer) |
| Cirrhosis of the Liver |
| Cirrhosis "The end result of many forms of chronic liver injury is cirrhosis, or scarring of liver tissue in reponse to previous acinar necrosis and irregular regeneration of liver nodules and bile ducts." ENCYCLOPEDIA BRITANNICA |
| Systemic scleroderma can involve the liver, causing biliary cirrhosis, and idiopathic portal hypertension. |
| Scientists say liver damage can be reversed. This raises hopes of a potential cure for cirrhosis (tissue scarring) caused by factors such as alcohol poisoning, burn injuries and scleroderma. The Herald. 12/28/07. |
| Idiopathic portal hypertension associated with systemic sclerosis and Sjogren's syndrome. The patient was diagnosed as having IPH associated with SSc and Sjogren's syndrome and cirrhosis of the liver. These observations suggest an immunological role in the pathogenesis of IPH. PubMed. Clin Rheumatol. 2005 May 5. (Also see: Sjogren's Syndrome) |
| Liver Failure Often Due to Supplements Diet aids, herbal supplements may play A role in organ failure. In 11 of the 20 patients, the use of over-the-counter weight loss supplements, a bodybuilding supplement, or herbs used for energy or relaxation were the only possible explanations they could find for the liver damage. The supplements contained either unlabeled ingredients, or herbs that previously have been linked to liver problems. WebMD Health 11/4/02. (Also see Scleroderma and Alternative Therapies) |
| Biliary Cirrhosis Symptoms |
| Symptoms include chronic fatigue, intense and unrelenting skin itching, gradual darkening of the skin, small yellow or white bumps under the skin (usually around the eyes), dry eye syndrome, thyroid problems, and arthritic aches and pains. |
| Nausea and Vomiting Many illnesses can cause stomach pain, nausea and vomiting, including hepatitis. Some are mild sicknesses that will pass by themselves, but others are serious and need medical attention. familydoctor.org |
| Diagnosis of Biliary Cirrhosis |
| "Patients are diagnosed with routine liver blood tests, but a liver biopsy is recommended to confirm the diagnosis and to determine the severity and extent of liver damage. Primary biliary cirrhosis is slow to evolve. Patients can lead active, normal, and symptom-free lifestyles for more than 10 years after diagnosis, but most patients eventually develop liver failure with its complications." Expired Article, University of Chicago Division of Gastroenterology. |
| Clinical and pathological characteristics of the autoimmune hepatitis and primary biliary cirrhosis overlap syndrome. Although AIH/PBC overlap syndrome does exist, it is infrequent and needs to be diagnosed carefully using close clinical and histological follow up to enable timely and effective treatment. PubMed. J Gastroenterol Hepatol. 2004 Jun;19(6):699-706. |
| Primary Biliary Cirrhosis (PBC) |
| "Primary biliary cirrhosis (PBC) is a chronic inflammatory disease of the liver which damages the interlobular bile ducts causing their gradual destruction, leading to progressive cholestasis and eventual cirrhosis. This disease predominantly affects middle-aged women (ratio women to men 9:1) of all races. There is a familial incidence of primary biliary cirrhosis and it is not unusual for the disease in the younger member of the family to be more severe than in the older." Medscape. |
| Primary biliary cirrhosis. Primary biliary cirrhosis is a disease in which the bile ducts in your liver are slowly destroyed. Your body has an intricate system of ducts designed specifically to transport bile, a fluid produced in your liver. MayoClinic.com. (Also see: Liver and Spleen Involvement) |
| Mitochondrial Autoantibodies. Concentrations of mitochondrial autoantibodies (MA), which are found in ~95% of patients with primary biliary cirrhosis (PBC), do not correlate with disease stage and prognosis, but are now known to play an important role in the immunopathogenesis of PBC. Approximately 3% of patients with PBC have scleroderma, usually of the CREST syndrome variety. Specialty Laboratories. (Also see: Types of Scleroderma and Causes of Scleroderma: Molecular Defect) |
| Patterns of autoimmunity in primary biliary cirrhosis patients and their families: a population-based cohort study. Primary biliary cirrhosis (PBC) is a chronic liver disease characterized by damage to, and destruction of, the biliary epithelial cells lining small intra-hepatic bile ducts.1 PBC exhibits a number of autoimmune features, including the almost universal presence of auto-antibodies reactive with highly conserved mitochondrial antigens (anti-mitochondrial antibodies, AMA). Q J Med 2004; 97: 397-406. (Also see: Causes of Scleroderma: Molecular Defect) |
| Primary biliary cirrhosis(PBC): a thirteen years experience. An early diagnosis and treatment of a disease such as PBC will reduce the incidence of complications and the use of costly treatments. PubMed. Rev Med Chil. 2006 Apr;134(4):469-74. |
| Clinical features and prognosis of primary biliary cirrhosis associated with systemic sclerosis. Liver disease has a slower progression in PBC-SSc, compared to matched patients with PBC alone. PubMed. Gut. 2005 Sep 8. |
| Treatment For Rare Liver Disease Increases Mortality Risk. Patients with primary biliary cirrhosis who are treated with methotrexate have an increased risk of death, according to a new systematic review of studies. ScienceDaily. 08/24/05. |
| Association of limited scleroderma and pulmonary hypertension in a patient with primary biliary cirrhosis. Sclerodactyly and telangiectasia of the fingers and chest wall were found. PubMed. J Nippon Med Sch. 2005 Aug;72(4):230-5. (Also see: Pulmonary Hypertension and Limited Scleroderma) |
| A human T-cell lymphotropic virus type-1 (HTLV-1) carrier complicated with various autoimmune diseases including primary biliary cirrhosis. A 47-year-old woman diagnosed as having overlap syndrome with scleroderma, systemic lupus erythematosus and possible polymyositis associated with primary biliary cirrhosis. (The tests) suggest the possibility of a relationship between HTLV-1 infection and various autoimmune disorders including primary biliary cirrhosis. PubMed. Hepatol Res. 2005 Feb 7. (Also see: Overlap Syndrome, Lupus, and Polymyositis) |
| Patterns of autoimmunity in primary biliary cirrhosis patients and their families: a population-based cohort study. PBC patients and their families have a wide susceptibility to autoimmunity. This observation supports an autoimmune aetiology and suggests that the genetic basis of PBC is likely to be expressed, at least in part, through factors controlling immune tolerance in general. PubMed. QJM. 2004 Jul;97(7):397-406. |
| Reynold's Syndrome: An Association to Remember. This syndrome constitutes the coexistence of two immunogenetically determined disorders: systemic scleroderma (CREST syndrome) and primary biliary cirrhosis (PBC), and it seems to be a favourable factor for liver disease progression. Association to Sjögren syndrome (SS) was reported. R. M. Morlà. AB0105 EULAR 2004. (Also see: CREST and Sjogren's) |
| Liver Personal Stories |
| (Romanian) Anca: Daughter of Polymyositis and Hepatitis C Patient Pentru inceput, ii salut pe toti cei ce acceseaza acest site, fie ca sufera de una din cumplitele boli de colagen... |
| Brenda M: Primary Biliary Cirrhosis (PBC), Fibromyalgia, Pulmonary Fibrosis, and Sjogren's Syndrome In 1965, when I was twenty-six, I went to work for a firm of electro-platers, which was mainly aircraft work... |
| Donna H: CREST, Autoimmune Hepatitis, Fibromyalgia I also have degenerative disc disease, osteoarthritis, anxiety/depression/panic disorder, irritable bowel... |
| Jaci: MCTD and Autoimmune Hepatitis Out of fear of going back to the doctors, I tried just to live with it, whatever it was. What I found strange was that nobody seemed to noticed how terrible I was feeling, not even the doctors... |
| Laira: MCTD, Scleroderma, SLE, Lupus, CREST, Lymphoma I was just seventeen when I was told I had rheumatoid arthritis... |
| Linda W: Surviving Daughter of Scleroderma Patient All she wanted was for others to learn of this disease and know her story. She requested this about two weeks before she died... |
| Mendyon: Mother-in-Law of CREST Patient Unfortunately, as I sit here writing this
letter
my husband and I are faced with some grim circumstances. She is currently losing
her battle to CREST syndrome... |
| Papa V: Lupus, Antiphospholipid Syndrome, Myasthenia Gravis, and Scleroderma About thirty years passed, and then—BAM !—I got a heart blockage, stroke, liver biopsy due to elevated enzymes, skin rash, high blood pressure, very high ANA.. |
| Ro M: Scleroderma After six months of suffering, I have just been diagnosed with scleroderma. I was constantly tired, run down and plagued with infections... |
| Sbirulino: Limited Systemic Sclerosis, First Stage Biliary Cirrhosis (Italy) They diagnosed Raynaud's Syndrome, until there was amputation of the first and second phalanges, which caused total invalidism... |
| (Italiano) Sbirulino: Sclerosi Sistemica variante Limitata, Cirrosi Biliare Primitiva (Italia) Mi avevano diagnosticato la sindrome di Reynaud, fino all'autoamputazione delle Ie e IIe falangi arrivando a portarmi ad una invalidità totale del 100%... |
| Suzy G: GAVE (Watermelon Stomach) and Primary Biliary Cirrhosis Just over six years ago I was diagnosed with primary biliary cirrhosis (PBC), a liver disease. Since then everything else seems to be happening to my body... |
| Liver Support Groups |
| American Liver Foundation |
| Canadian Liver Foundation |
| Children's Liver Disease Foundation |
| Spleen: Splenomegaly and Hypersplenism |
| Congenital absence of the portal vein with splenomegaly and hypersplenism in a young woman. Congenital absence of portal vein (CAPV) with a systemic shunt of enteric blood is a rare malformation. Beside suffering from circumscript scleroderma, splenomegaly and hypersplenism, the patient was otherwise healthy. PubMed. Digestion 2003;67(1-2):105-10. |
