Cryoglobulins and the Kidney
Cryoglobulins are Igs that reversibilly precipitate at
temperatures <37C. This results in
symptoms that can lead to rashes, vasculitis, ulcers, ischemia, joint pains,
and eventually in 25% cases – renal injury.
Two mechanisms: immune complex GN vs high viscosity
related. All have nephritic/nephrotic
syndrome with various levels of kidney function.
1/3 with purpura and arthralgia
¾ with low C4 and ½ with low C3
¾ with low C4 and ½ with low C3
All 3 types of cryoglobulins reported with renal disease
Most commonly it occurs in settings of type II (“mixed”) cryo
secondary to hepatitis C virus. Most
patients with type II or III cryoglobulins have a positive rheumatoid factor. The pathology pattern of injury is either
membranoproliferative or diffuse proliferative GN.
•
Type 1 Cryo( monoclonal IgM or IgG) – MM,
MGUS, WM, lymphomas and CLL
•
Type 2 Cryo(monoclonal IgM + polyclonal IgG)-
CLL, lymphoma, SLE, Hep C
•
Type 3 Cryo(polyclonal)- lymphoproliferative
diseases, SLE
Treatment
Treatment
of underlying cause- Hep, SLE, Myeloma
Steroids
Alkylating
Agents
Anti
CD-20 agents( good response in some cases)
Plasmapheresis
( adjunct) if skin involved and early renal
involvement
involvement