Vasculitis with essential cryoglobulinemia D89.1

Multi-organ disease caused by vasculitic inflammation in the context of essential cryoglobulinemia. Skin and joint infections are in the foreground of the symptoms. Less frequent are vasculitic phenomena on the larger vessels (aorta and branches of the aorta).

• Type I: Monoclonal immunoglobulin (mostly IgM).
• Type II: Mixed essential cryoglobulinemia with monoclonal IgM rheumatoid factor and polyclonal IgM. This type is the basis of essential cryoglobulinemic vasculitis (Chapel Hill Consensus Conference).
• Type III: Polyclonal IgG + polyclonal IgA or IgM.

The highest prevalence of cryoglobulinemia is expected in geographical regions with high hepatitis C and B prevalence and co-infection with HIV. While 40-60% of HCV-infected individuals have cryoglobulinemia, approximately 5-30% develop cryoglobulinemic vasculitis.

In type I cryoglobulinemia, the monoclonal cryoglobulins gel directly in the vessels under the influence of cold. This causes a hyperviscosity syndrome with vessel occlusion, live image and consecutive focal necroses.

In type II cryoglobulinemia, a monoclonal IgM forms a complex with IgG that has bound a protein of hepatitis C. This complex then gels when exposed to cold.

• Joints: Polyarthralgia.
• Kidney: Progressive glomerulonephritis, which can lead to acute renal failure within hours to days.
• Peripheral nervous system: Vasculitis of the vasa nervorum with mononeuritis multiplex or symmetrical axonal polyneuropathy.
• More rarely: manifestations in the GI tract, eyes (central artery occlusion), heart (myocardial infarction, pericarditis) and lungs (pleuritis).

Cryoglobulin detection: blood collection (10 ml of native and 10 ml of citrated blood) and collection of serum and plasma at 37 °C; cooling of samples in 0 °C water bath for 24, 48, 72 h).

HCV diagnostics should be performed in the laboratory.

Skin histology (fresh lesion only!) with evidence of leukocytoclastic vasculitis.

Treatment of the underlying disease. In case of mild clinic symptomatic therapy with NSAR. For chronic HCV infections: HCV therapy according to guidelines.

Patients without chronic viral infections with severe clinical symptoms and resistance to therapy can be treated immunosuppressively by leukocyte-adaptation, e.g. with prednisolone (Decortin H) 0.25-0.5 mg/kg bw/day in combination with cyclophosphamide (e.g. endoxane) 1-2 mg/kg bw/day (Fauci scheme). Alternatively: Melphalan (e.g. Alkeran) and prednisolone shock therapy as in plasmocytoma or long-term therapy with chlorambucil (e.g. Leukeran) 2-5 mg/day p.o.

In cases of rapid deterioration of the function of vital organs (kidney) and the occurrence of a hyperviscosity syndrome as well as progressive pareses, plasmapheresis in combination with immunosuppressive therapy is indicated.

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