Cryoglobulinemia
The cryoglobulinemia belongs to the group of vasculitis (vasculitides) which do not ANCA are -assoziiert.
First describer
Wintrobe and Buell 1933 56-year-old woman with multiple myeloma and "kältepräzipitierenden serum globulins".
In 1947, Lerner and Watson described a patient with unusual purpura and high concentrations of "cold-precipitating serum globulins", which they called cryoglobulins and thus coined the term.
Pathogenesis
Cryoglobulins are antibodies ( immunoglobulins ) that become insoluble when cold and redissolve when heated. In addition, they can have the properties of rheumatoid factors or cold agglutinins . A distinction is made between monoclonal cryoglobulins and mixed cryoglobulins . The latter are immune complexes .
There are three different types of cryoglobulins:
- Type 1 cryoglobulins : monoclonal immunoglobulin G (IgG) or immunoglobulin M (IgM), rarely other immunoglobulins. Monoclonal cryoglobulins.
- Type 2 cryoglobulins : mostly monoclonal immunoglobulin M, which, like rheumatoid factors, binds to the Fc piece of IgG. Mixed cryoglobulins.
- Type 3 cryoglobulins : polyclonal immunoglobulin M that forms immune complexes with another polyclonal immunoglobulin. Mixed cryoglobulins.
Chronic hepatitis C causes cryoglobulinemia in about 80 percent of cases . Other causes can be:
- Waldenström's disease
- multiple myeloma
- systemic lupus erythematosus
- Sjogren's syndrome
- Polyarteritis nodosa
- Rheumatoid arthritis
- Sarcoid
- Ulcerative colitis
- Infections with:
- Epstein Barr Virus (EBV)
- Toxoplasma gondii , the causative agent of toxoplasmosis
- Treponema pallidum , the causative agent of syphilis
- Borrelia
- Cytomegalovirus (CMV)
- Hepatitis C.
Symptoms
- Skin symptoms:
- Bleeding from the skin due to damage to the wall of the acres , these skin manifestations are known as "palpable purpura"
- Raynaud's disease
- Acrocyanosis
- Necrosis of the acres
- Arthralgia (joint pain)
- Myalgia (muscle pain)
- Focus neuropathy
- Liver / spleen enlargement in 60 percent of patients (especially in Waldenström's disease and plasmacytoma)
- Lymph node disease
- Glomerulonephritis with hematuria and proteinuria
- neuropathy in 50 percent of patients
- multiple organ attacks
diagnosis
The diagnosis is based on anamnesis , vascular biopsy and possible evidence of hepatitis C. The sedimentation rate is strongly accelerated at 37 ° C and normal at 4 ° C. The cryoglobulins can be detected by electrophoresis .
treatment
On the one hand, possible underlying diseases are treated, on the other hand, corticosteroids and various chemotherapeutic agents are used.