Scleroderma Liver and Spleen Involvement |
| Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer |
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Overview |
| Liver disease can occur alone, from alcohol or medication side-effects, as part of other diseases, or as secondary to systemic sclerosis (scleroderma). (Also see: What is Scleroderma?, Types of Scleroderma, and Systemic Sclerosis) |
| 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 |
| Liver autoantibodies in patients with scleroderma. There is an increased prevalence of liver autoantibodies in patients with scleroderma than in control population. These patients should be carefully followed for liver dysfunction. (Springerlink) Thelma L. Skare, Clinical Rheumatology, 7 October 2010. |
| 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. |
| Should We Look for Celiac Disease among all Patients with Liver Function Test Abnormalities? Serological screening with IgA anti-tTG antibody test should be routinely performed in patients presenting with abnormal liver function tests (LFT) and especially those with chronic liver diseases including hypertransaminasemia, autoimmune hepatitis, and cryptogenic cirrhosis. Int J Prev Med. 2012 Mar;3(3):167-72. (Also see: Celiac Disease) |
Hepatitis: Autoimmune (AIH) |
| What is Autoimmune Hepatitis? Autoimmune hepatitis is an inflammation of the liver caused by an autoimmune disorder. An autoimmune disorder is one in which a person's immune system attacks his or her own body for unknown reasons. Glenn Rosenberg. 24 Medica. 10/05/09. |
| Autoimmune hepatitis. Autoimmune hepatitis (AIH) is a chronic, progressive disease of the liver characterized by hypergammaglobulinemia, circulating autoantibodies. Sulz MC. Ther Umsch. 2011 Apr;68(4):189-194. |
| Management of autoimmune hepatitis (AIH). These new findings are pertinent to the everyday clinical management of patients with AIH. Mayo MJ. Curr Opin Gastroenterol. 2011 Mar 30. |
| Autoimmune Hepatitis and Celiac Disease: Case Report Showing an Entero-Hepatic Link. Celiac disease is an autoimmune disorder primarily targeting the small bowel, although extraintestinal extensions have been reported. The autoimmune processes can affect the liver with manifestations such as primary biliary cirrhosis and autoimmune hepatitis. Tovoli F. (PubMed) Case Rep Gastroenterol. 2010 Oct 26. (Also see: Celiac Disease and Primary Biliary Cirrhosis ) |
| Autoimmune hepatitis, HLA and extended haplotypes. Characteristic liver-infiltrating immune cells in portal and periportal areas, hypergammaglobulinemia and typical autoantibodies indicate an ongoing autoimmune reaction against liver self antigens, which lead to irreversible cellular damage and ultimately to severe hepatic failure. Oliveira LC, (PubMed) Autoimmun Rev. 2010 Oct 5. |
| Turmeric Delays Liver Disease Progression. Scientists have found that the Indian spice turmeric delayed the progression of liver disease associated with autoimmune disease known as primary sclerosing cholangitis (PSC) and primary biliary cirrhosis. Kathleen Blanchard RN. EmaxHealth (British Medical Journal). 03/24/10. (Also see: Foods and Health) |
| Diffuse systemic sclerosis and autoimmune hepatitis (AIH): a unique association. The authors describe herein the first report of a patient with diffuse systemic sclerosis who was diagnosed with AIH with positive antimitochondrial antibody and had an excellent response to immunosuppressive drugs. (SpringerLink) C. Ewerton Maia Rodriguez. Clinical Rheumatology. February 7 2010. |
| Liver Lines: February 19, 2010. Autoimmune hepatitis is a common form of chronic liver disease that is quite commonly encountered in our area. As there are no specific markers for this condition and it is seldom considered by physicians, its diagnosis is often delayed or not made at all. David Bernstein, MD, FACP, FACG. Massapequan Observer. 02/19/10. |
| Regression of fibrosis in paediatric autoimmune hepatitis (AIH): morphometric assessment of fibrosis versus semiquantiatative methods. Regression of hepatic fibrosis in patients with AIH has been described in response to immunosuppressive therapy. A. Abdalla. Fibrogenesis & Tissue Repair. April 2 2009. |
| 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) |
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) |
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. 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. (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) |
| Autoimmune Hepatitis and Celiac Disease: Case Report Showing an Entero-Hepatic Link. Celiac disease is an autoimmune disorder primarily targeting the small bowel, although extraintestinal extensions have been reported. The autoimmune processes can affect the liver with manifestations such as primary biliary cirrhosis and autoimmune hepatitis. Tovoli F. (PubMed) Case Rep Gastroenterol. 2010 Oct 26. (Also see: Celiac Disease and Autoimmune Hepatitis) |
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... |
| Debs B: Diffuse Systemic Sclerosis (England) This seems to be a long list of ailments that I have been coping with for years, so the addition of diffuse systemic sclerosis did not really panic me... |
| 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. |
