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Cryoglobulinemic vasculitis


Other Names:
Familial mixed cryoglobulinemia; Mixed cryoglobulinemia; Essential cryoglobulinemia; Familial mixed cryoglobulinemia; Mixed cryoglobulinemia; Essential cryoglobulinemia; Primary cryoglobulinemia; Essential mixed cryoglobulinemia; Meltzer syndrome See More
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Cryoglobulinemic vasculitis occurs when the body makes a mix of abnormal immune system proteins called cryoglobulins. At temperatures less than 98.6 degrees Fahrenheit (normal body temperature), cryoglobulins become solid or gel-like and can block blood vessels. This causes a variety of health problems. Many people with cryoglobulins will not experience any symptoms. When symptoms occur, they may include purplish discolored skin (purpura), weakness, joint pain, liver disease, and kidney problems. The underlying cause is unknown. Cryoglobulinemic vasculitis is typically associated with a chronic hepatitis C virus (HCV) infection. It is diagnosed based on the results of a clinical exam and the presence of cryoglobulins in the blood. Treatment varies based on the severity of symptoms and any underlying conditions.[1][2][3]
Last updated: 6/16/2020
The following list includes the most common signs and symptoms in people with cryoglobulinemic vasculitis. These features may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list does not include every symptom or feature that has been described in this condition.

Signs and symptoms may include:[1][3]
  • Purple spots and patches on the skin (purpura)
  • Nerve damage (peripheral neuropathy)
  • Joint pain and swelling (arthralgia)
  • Weakness
  • Kidney problems
  • Liver problems
Symptoms usually begin between ages 40 and 60. Although cryoglobulinemic vasculitis is a long-term disease, symptoms tend come and go. The severity of the symptoms can be difficult to predict, and may depend on underlying conditions. People who have organ involvement tend to have more severe disease.[2]
Last updated: 6/16/2020

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Circulating immune complexes 0012224
Cryoglobulinemia 0100778
Cutis marmorata 0000965
Fever 0001945
Mediastinal lymphadenopathy
Swollen lymph nodes in center of chest
0100721
Muscle weakness
Muscular weakness
0001324
Petechiae 0000967
Skin ulcer
Open skin sore
0200042
Vasculitis
Inflammation of blood vessel
0002633
30%-79% of people have these symptoms
Abdominal pain
Pain in stomach
Stomach pain
[ more ] 		0002027
Arthralgia
Joint pain
0002829
Arthritis
Joint inflammation
0001369
Gangrene
Death of body tissue due to lack of blood flow or infection
0100758
Gastrointestinal infarctions
Death of digestive organ tissue due to poor blood supply
0005244
Glomerulopathy 0100820
Hematuria
Blood in urine
0000790
Hepatomegaly
Enlarged liver
0002240
Mononeuropathy
Single damaged nerve
0009831
Myalgia
Muscle ache
Muscle pain
[ more ] 		0003326
Proteinuria
High urine protein levels
Protein in urine
[ more ] 		0000093
Renal insufficiency
Renal failure
Renal failure in adulthood
[ more ] 		0000083
Sensorimotor neuropathy
Nerve damage causing decreased feeling and movement
0007141
Splenomegaly
Increased spleen size
0001744
Viral hepatitis 0006562
5%-29% of people have these symptoms
Gastrointestinal hemorrhage
Gastrointestinal bleeding
0002239
Keratoconjunctivitis sicca
Dry eyes
0001097
Percent of people who have these symptoms is not available through HPO
Abnormality of blood and blood-forming tissues 0001871
Anasarca 0012050
Autosomal dominant inheritance 0000006
Chronic kidney disease 0012622
Elevated serum creatinine
Elevated creatinine
High blood creatinine level
Increased creatinine
Increased serum creatinine
[ more ] 		0003259
Hypertension 0000822
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Last updated: 7/1/2020
The cause of cryoglobulinemic vasculitis is unknown. It occurs mainly in people with a chronic hepatitis C infection. Other viral, bacterial, and fungal infections have also been associated with cryoglobulinemic vasculitis.[1][2]
Last updated: 6/16/2020
Cryoglobulinemic vasculitis can be diagnosed based on a clinical history and exam, the symptoms, and testing to look for cryoglobulins in the blood.[1][2]
Last updated: 6/16/2020
Treatment of cryoglobulinemic vasculitis is focused on the underlying disease and managing the symptoms. Treatment options include medications that suppress the immune system and antivirals.[1][2][3]

Specialists involved in the care of someone with cryoglobulinemic vasculitis may include:
  • Dermatologist
  • Neurologist
  • Kidney specialist
  • Liver specialist
  • Hematologist
Last updated: 6/16/2020
The number of people with cryoglobulinemic vasculitis is unknown. It has been estimated that about 1% of all people with hepatitis C viral infections develop cryoglobulinemic vasculitis.[2]
Last updated: 6/16/2020

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
Differential diagnoses include a wide range of systemic, infectious and neoplastic disorders, mainly autoimmune hepatitis, Sjogren's syndrome, B-cell lymphomas (see these terms), and polyarthritis.
Visit the Orphanet disease page for more information.

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • The Centers for Mendelian Genomics program is working to discover the causes of rare genetic disorders. For more information about applying to the research study, please visit their website.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Social Networking Websites

  • RareConnect has an online community for patients and families with this condition so they can connect with others and share their experiences living with a rare disease. The project is a joint collaboration between EURORDIS (European Rare Disease Organisation) and NORD (National Organization for Rare Disorders).

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
  • The Vasculitis Foundation provides information about Cryoglobulinemic vasculitis.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Cryoglobulinemic vasculitis. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

  1. Roccatello D, Saadoun D, Ramos-Casals M, et al. Cryoglobulinaemia. Nat Rev Dis Primers. 2018; 4(1):11. https://pubmed.ncbi.nlm.nih.gov/30072738.
  2. Ramos-Casals M, Stone JH, Cid MC, Bosch X. The cryoglobulinaemias. Lancet. 2012; 379(9813):348-360. https://pubmed.ncbi.nlm.nih.gov/21868085.
  3. Ghetie D, Mehraban N, Sibley CH. Cold hard facts of cryoglobulinemia: updates on clinical features and treatment advances. Rheum Dis Clin North Am. 2015; 41(1):93-108. https://pubmed.ncbi.nlm.nih.gov/25399942.