Cryoglobulinemia: Difference between revisions

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== Introduction ==
== Introduction ==
Cryoglobulinemia is an autoimune disorder that is characterised by the persistent presence of a cryoglobulin (Igs) in the serum.<ref name=":0">Naciri Bennani H, Banza AT, Terrec F, Noble J, Jouve T, Motte L, Malvezzi P, Rostaing L. [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10084379/ Cryoglobulinemia and double‐filtration plasmapheresis: Personal experience and literature review.] Therapeutic Apheresis and Dialysis. 2023 Feb;27(1):159-69.</ref> <ref name=":1">Bhandari J, Awais M, Aeddula NR. [https://www.ncbi.nlm.nih.gov/books/NBK557606/ Cryoglobulinemia].reasure Island (FL): StatPearls Publishing; 2023 Jan-.</ref> Cryoglobulins are proteins that precipitate from an individual’s serum or plasma at temperatures lower than 37 °C, leading to cryoprecipitate. They can be a mixture of immunoglobulin (Ig) and complement components or immunoglobulins alone. Cryoglobulins, which accumulate in blood vessels, can cause endothelial injury and end-organ damage, resulting in cryoglobulinemia.<ref name=":1" />The disease mainly affects small to medium-sized blood vessels<ref>Muchtar E, Magen H, Gertz MA. [https://www.sciencedirect.com/science/article/pii/S000649712033771X?via%3Dihub#cesec50 How I treat cryoglobulinemia. Blood,] The Journal of the American Society of Hematology. 2017 Jan 19;129(3):289-98.</ref> and causes vasculitis, which leads to a systemic inflammatory syndrome characterised by fatigue, arthralgia, purpura, neuropathy, and glomerulonephritis. <ref name=":0" />According to Brouet’s classification, cryoglobulins are classified into three types: Type I, Type II, and Type III, on the basis of their immunoglobin (Ig) composition.<ref name=":1" /> <ref name=":2">Roccatello D, Saadoun D, Ramos-Casals M, Tzioufas AG, Fervenza FC, Cacoub P, Zignego AL, Ferri C. [https://www.nature.com/articles/s41572-018-0009-4#Sec7 Cryoglobulinaemia]. Nature Reviews Disease primers. 2018 Aug 2;4(1):11.</ref><ref name=":3">Takada S, Shimizu T, Hadano Y, Matsumoto K, Kataoka Y, Arima Y, Inoue T, Sorano S. [https://www.spandidos-publications.com/mmr/6/1/3/ Cryoglobulinemia.] Molecular medicine reports. 2012 Jul 1;6(1):3-8.</ref>
Cryoglobulinemia is an autoimune disorder that is characterised by the persistent presence of a cryoglobulin (Igs) in the serum.<ref name=":0">Naciri Bennani H, Banza AT, Terrec F, Noble J, Jouve T, Motte L, Malvezzi P, Rostaing L. [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10084379/ Cryoglobulinemia and double‐filtration plasmapheresis: Personal experience and literature review.] Therapeutic Apheresis and Dialysis. 2023 Feb;27(1):159-69.</ref> <ref name=":1">Bhandari J, Awais M, Aeddula NR. [https://www.ncbi.nlm.nih.gov/books/NBK557606/ Cryoglobulinemia].reasure Island (FL): StatPearls Publishing; 2023 Jan-.</ref> Cryoglobulins are proteins that precipitate from an individual’s serum or plasma at temperatures lower than 37 °C, leading to cryoprecipitate. They can be a mixture of immunoglobulin (Ig) and complement components or immunoglobulins alone. Cryoglobulins, which accumulate in blood vessels, can cause endothelial injury and end-organ damage, resulting in cryoglobulinemia.<ref name=":0" /><ref name=":1" />The disease mainly affects small to medium-sized blood vessels<ref>Muchtar E, Magen H, Gertz MA. [https://www.sciencedirect.com/science/article/pii/S000649712033771X?via%3Dihub#cesec50 How I treat cryoglobulinemia. Blood,] The Journal of the American Society of Hematology. 2017 Jan 19;129(3):289-98.</ref> and causes vasculitis, which leads to a systemic inflammatory syndrome characterised by fatigue, arthralgia, purpura, neuropathy, and glomerulonephritis. It should be suspected in patients with skin ulcers, arthralgia, glomerulonephritis, neuropathy, and purpura.<ref name=":0" /><ref name=":1" />According to Brouet’s classification, cryoglobulins are classified into three types: Type I, Type II, and Type III, on the basis of their immunoglobin (Ig) composition.<ref name=":1" /><ref name=":2">Roccatello D, Saadoun D, Ramos-Casals M, Tzioufas AG, Fervenza FC, Cacoub P, Zignego AL, Ferri C. [https://www.nature.com/articles/s41572-018-0009-4#Sec7 Cryoglobulinaemia]. Nature Reviews Disease primers. 2018 Aug 2;4(1):11.</ref><ref name=":3">Takada S, Shimizu T, Hadano Y, Matsumoto K, Kataoka Y, Arima Y, Inoue T, Sorano S. [https://www.spandidos-publications.com/mmr/6/1/3/ Cryoglobulinemia.] Molecular medicine reports. 2012 Jul 1;6(1):3-8.</ref> Cryoglobulinemia is a rare condition affecting 1 in 100,000 people, with about 15%-20% of HIV-infected individuals, 40-65% of [[Hepatitis A, B, C|Hepatitis]] C-infected patients, and 64% of HIV/HepC coinfected individuals. Type 1 cryoglobulin patients make up 5%-25% of cases, according to the available studies.<ref name=":1" />


# '''Type I cryoglobulinemia:''' This is characterised by a monoclonal immunoglobulin (usually IgM) or a single monoclonal immunoglobulin (most commonly immunoglobulin M (IgM), rarely IgG or IgA). It is often associated with lymphoproliferative disorders like Waldenström macroglobulinemia and multiple myeloma.<ref name=":2" /> <ref name=":3" />
# '''Type I cryoglobulinemia:''' This is characterised by a monoclonal immunoglobulin (usually IgM) or a single monoclonal immunoglobulin (most commonly immunoglobulin M (IgM), rarely IgG or IgA). It is often associated with lymphoproliferative disorders like Waldenström macroglobulinemia, multiple myeloma, chronic lymphocytic leukemia, or protein-secreting monoclonal gammopathies like monoclonal gammopathy of undetermined significance (MGUS).<ref name=":1" /> <ref name=":2" /><ref name=":3" />
# '''Type II cryoglobulinemia:''' In this type, a combination of monoclonal antibodies (usually IgM with rheumatoid factor activity) forms immune complexes with polyclonal IgG. It is commonly associated with hepatitis C virus (HCV) infection.<ref name=":2" /><ref name=":3" />
# '''Type II cryoglobulinemia:''' In this type, a combination of monoclonal antibodies (usually IgM with rheumatoid factor activity) forms immune complexes with polyclonal IgG. It is commonly associated with [[Hepatitis A, B, C|Hepatitis]] C virus (HCV) infection, Mixed cryoglobulinemia syndrome, [[Hepatitis A, B, C|Hepatitis]] B virus (HBV), HIV,  Sjögren's syndrome, Lymphoproliferative disorders.<ref name=":1" /><ref name=":2" /><ref name=":3" />
# '''Type III cryoglobulinemia:''' This type involves polyclonal IgM and IgG-forming immune complexes with no monoclonal immunoglobulin. Type III cryoglobulinemia is frequently seen in patients with autoimmune diseases like systemic lupus erythematosus (SLE) and rheumatoid arthritis.<ref name=":3" />
# '''Type III cryoglobulinemia:''' This type involves polyclonal IgM and IgG-forming immune complexes with no monoclonal immunoglobulin. Type III cryoglobulinemia is frequently seen in patients with autoimmune diseases like systemic lupus erythematosus (SLE) and rheumatoid arthritis.<ref name=":3" />


== Sub Heading 2 ==
== Etiology ==


== Sub Heading 3 ==
== Pathophysiology ==
Cryoglobulinemia is a multifactorial disease characterized by the production of abnormal immunoglobulins by B lymphocytes or plasma cells, which can form immune complexes with antigens, particularly at lower temperatures. Mono-, oligo-, or polyclonal immunoglobulin synthesis is increased by lymphoproliferation and chronic immunological activation, which results in the development of cryoglobulins and antigen-antibody complexes. Accumulation of these complexes occurs from insufficient clearance which deposit in small blood vessels, particularly in the skin, kidneys, and peripheral nerves, causing inflammation and tissue damage.


== Resources  ==
== Resources  ==

Revision as of 10:06, 28 September 2023

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Introduction[edit | edit source]

Cryoglobulinemia is an autoimune disorder that is characterised by the persistent presence of a cryoglobulin (Igs) in the serum.[1] [2] Cryoglobulins are proteins that precipitate from an individual’s serum or plasma at temperatures lower than 37 °C, leading to cryoprecipitate. They can be a mixture of immunoglobulin (Ig) and complement components or immunoglobulins alone. Cryoglobulins, which accumulate in blood vessels, can cause endothelial injury and end-organ damage, resulting in cryoglobulinemia.[1][2]The disease mainly affects small to medium-sized blood vessels[3] and causes vasculitis, which leads to a systemic inflammatory syndrome characterised by fatigue, arthralgia, purpura, neuropathy, and glomerulonephritis. It should be suspected in patients with skin ulcers, arthralgia, glomerulonephritis, neuropathy, and purpura.[1][2]According to Brouet’s classification, cryoglobulins are classified into three types: Type I, Type II, and Type III, on the basis of their immunoglobin (Ig) composition.[2][4][5] Cryoglobulinemia is a rare condition affecting 1 in 100,000 people, with about 15%-20% of HIV-infected individuals, 40-65% of Hepatitis C-infected patients, and 64% of HIV/HepC coinfected individuals. Type 1 cryoglobulin patients make up 5%-25% of cases, according to the available studies.[2]

  1. Type I cryoglobulinemia: This is characterised by a monoclonal immunoglobulin (usually IgM) or a single monoclonal immunoglobulin (most commonly immunoglobulin M (IgM), rarely IgG or IgA). It is often associated with lymphoproliferative disorders like Waldenström macroglobulinemia, multiple myeloma, chronic lymphocytic leukemia, or protein-secreting monoclonal gammopathies like monoclonal gammopathy of undetermined significance (MGUS).[2] [4][5]
  2. Type II cryoglobulinemia: In this type, a combination of monoclonal antibodies (usually IgM with rheumatoid factor activity) forms immune complexes with polyclonal IgG. It is commonly associated with Hepatitis C virus (HCV) infection, Mixed cryoglobulinemia syndrome, Hepatitis B virus (HBV), HIV, Sjögren's syndrome, Lymphoproliferative disorders.[2][4][5]
  3. Type III cryoglobulinemia: This type involves polyclonal IgM and IgG-forming immune complexes with no monoclonal immunoglobulin. Type III cryoglobulinemia is frequently seen in patients with autoimmune diseases like systemic lupus erythematosus (SLE) and rheumatoid arthritis.[5]

Etiology[edit | edit source]

Pathophysiology[edit | edit source]

Cryoglobulinemia is a multifactorial disease characterized by the production of abnormal immunoglobulins by B lymphocytes or plasma cells, which can form immune complexes with antigens, particularly at lower temperatures. Mono-, oligo-, or polyclonal immunoglobulin synthesis is increased by lymphoproliferation and chronic immunological activation, which results in the development of cryoglobulins and antigen-antibody complexes. Accumulation of these complexes occurs from insufficient clearance which deposit in small blood vessels, particularly in the skin, kidneys, and peripheral nerves, causing inflammation and tissue damage.

Resources[edit | edit source]

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References[edit | edit source]

  1. ↑ 1.0 1.1 1.2 Naciri Bennani H, Banza AT, Terrec F, Noble J, Jouve T, Motte L, Malvezzi P, Rostaing L. Cryoglobulinemia and double‐filtration plasmapheresis: Personal experience and literature review. Therapeutic Apheresis and Dialysis. 2023 Feb;27(1):159-69.
  2. ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 Bhandari J, Awais M, Aeddula NR. Cryoglobulinemia.reasure Island (FL): StatPearls Publishing; 2023 Jan-.
  3. ↑ Muchtar E, Magen H, Gertz MA. How I treat cryoglobulinemia. Blood, The Journal of the American Society of Hematology. 2017 Jan 19;129(3):289-98.
  4. ↑ 4.0 4.1 4.2 Roccatello D, Saadoun D, Ramos-Casals M, Tzioufas AG, Fervenza FC, Cacoub P, Zignego AL, Ferri C. Cryoglobulinaemia. Nature Reviews Disease primers. 2018 Aug 2;4(1):11.
  5. ↑ 5.0 5.1 5.2 5.3 Takada S, Shimizu T, Hadano Y, Matsumoto K, Kataoka Y, Arima Y, Inoue T, Sorano S. Cryoglobulinemia. Molecular medicine reports. 2012 Jul 1;6(1):3-8.
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