Scleroderma Liver and Spleen Involvement

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Hepatitis, Autoimmune
Hepatitis, Viral
Cancer of Liver
Cirrhosis of Liver
Biliary Cirrhosis Symptoms
Biliary Cirrhosis Diagnosis
Primary Biliary Cirrhosis
Primary Sclerosing Cholangitis
Personal Stories
Support Groups


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 donation for transplant misunderstood, underutilized. Lack of knowledge among community physicians may be keeping them from raising the possibility of living donor transplants with their patients with liver disease, new survey data suggest. Reuters Health, 06/19/2019.

Children lose out on liver transplants, study finds. A system used to determine who is most in need of a transplant significantly underestimates the risk of death for younger children with liver disease, according to a new study. NBC Health News, 09/17/2018.

Thyroid disorders and gastrointestinal and liver dysfunction: A state of the art review. In this review, we discuss disorders of the thyroid-gut axis and identify the evidence base behind the management of such disorders. PubMed, Eur J Intern Med. (Also see Gastrointestinal Involvement and Thyroid Diseases)

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.

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. 24 Medica.

Case Report: Autoimmune Hepatitis and Systemic Sclerosis: a Rare Association. Autoimmune hepatitis and systemic sclerosis may be associated, therefore patients with these diseases should be monitored closely. PubMed, Eur J Case Rep Intern Med.

A System to Determine Risk of Osteoporosis in Patients With Autoimmune Hepatitis. Almost 20% of patients with AIH older than 50 years have osteoporosis. PubMed, Clin Gastroenterol Hepatol, 06/01/2019. (Also see Osteoporosis)

Hepatitis C (Viral Hepatitis)

Cryoglobulinemic Vasculitis (CV) in primary Sjögren's Syndrome pSS): Clinical presentation, association with lymphoma and comparison with Hepatitis C-related disease. Though infrequent, pSS-CV constitutes a distinct severe clinical phenotype of pSS. Seminars in Arthritis and Rheumatism, 07/27/2020. (Also see Symptoms and Complications of Sjögren's Syndrome)

Incidence and risk factors for reactivation from resolved hepatitis B virus (HBV) in rheumatoid arthritis patients treated with biological disease-modifying antirheumatic drugs (bDMARDs). HBV reactivation occurred in 4.6% of RA patients with resolved HBV during the treatment with bDMARDs and the absence of anti-HBs may be a risk factor for the reactivation of resolved HBV. PubMed, Int J Rheum Dis, 2019 Apr;22(4):574-582. (Also see Rheumatoid Arthritis in Overlap and Clinical Trials)

(Case Report) 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. (Also see Causes of Scleroderma: Drugs and Medications)

Liver Cancer

Autoimmunity and cancer: a lesson from systemic sclerosis. Allied Academies, 2018. (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.

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.

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.

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. (Also see Liver and Spleen Involvement)

Comorbidity of localized scleroderma and primary biliary cholangitis (PBC). The coexistence of morphea, anti–mitochondrial antibodies positivity and PBC is a rare but possible association. PubMed, J Dtsch Dermatol Ges, 2018 Nov;16(11):1323-1327. (Also see Conditions Associated with Morphea)

Case Report: Autoimmune Hepatitis and Celiac Disease 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. PubMed, Tovoli F. Case Rep Gastroenterol. (Also see Celiac Disease and Autoimmune Hepatitis)

Primary Sclerosing Cholangitis

Case Report: Primary sclerosing cholangitis associated with CREST in an elderly woman. This case highlights the need to consider primary sclerosing cholangitis in patients with CREST who present with abdominal symptoms and deranged liver enzymes when other causes have been excluded. PubMed, J Med Case Rep.

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..

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

Adverse Events Related to Partial Splenic Embolization for the Treatment of Hypersplenism: A Systematic Review. Interventional radiologists should be aware of the complication profile of this procedure and further advance research in techniques dealing with hypersplenism. PubMed, J Vasc Interv Radiol, 01/31/2020.

Case Report: Extra pulmonary tuberculosis presenting as fever with massive splenomegaly and pancytopenia. We report the case of a 13-year old male who presented with fever, weight loss, pallor and massive splenomegaly with pancytopenia. PubMed, IDCases. (Also see Extrapulmonary Tuberculosis)

Go to Lung (Pulmonary) Involvement

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