Cryoglobulinemia medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Feham Tariq, MD [2]
Overview
The medical treatment of cryoglobulinemia depends on the underlying etiology, nature and progression of the disease. The main indication for therapy is to halt the progressive end organ damage affecting the kidneys, skin, gastrointestinal system, central nervous system and the extremities. The therpeutic regimens mainly used are immunosuppressive agents and plasmapheresis.
Medical Therapy
The main pharmacotherapy for each type of cryoglobulinemia is discussed below:
Assymptomatic cryoglobulinemia
This type of cryoglobulinemia requires no medical treatment.
Secondary cryoglobulinemia
This category includes type 1 and mixed cyroglobulinemia. The goal of treatment for secondary type is as follows:
- Conservative management
- Immunosuppressive agents
- Plasmapheresis
Immunosuppressive agents
The main aim of immunosuppressive therapy in cryoglobulinemia is its employment for the patients having rapidly progressive, organ-threatening or like-threatening course of the disease regardless of the underlying cause.
Indications for immunosuppressive therapy:
The indications for using immunosuppressive therapy are as follows:
- Digital ischemia leading to amputation
- Gastrointestinal vasculitis resulting in abdominal pain
- Heart failure
- Pulmonary vasculitis associated with alveolar hemorrhage
- Central nervous system vasculitis that presents as stroke
- Glomerulonephritis associated with nephrotic range proteinuria
Choice of immunosuppressive therapy:
The patients selected for immunosuppressive therapy are preferably initiated with rituximab along with pulsed doses of glucocorticoids.
Ritximab Regimen:
- Preferred regimen (1): Four infusions of rituximab 375 mg/m2 at weekly intervals (day 0, day 7, day 14, day 21).
- Preferred regimen (2): Two infusions of ritximab 1000 mg IV seperated by two weeks interval (day 0 and day 14).
- Preferred regimen (3): Four infusions of rituximab 375 mg/m2 at weekly intervals (day 0, day 7, day 14, day 21) followed by additional doses at day 49 and day 77.
Glucocorticoid Regimen:
- Preferred regimen (1): Methylprednisone 7.5 to 15 mg/kg per day IV once daily for 3 days based on the severity of symptoms
- Preferred regimen (2): First regimen followed by prednisone 1 mg/kg per day PO once daily for 14-28 days.
- Tapering regimen: 40 mg/day for 2 weeks followed by 20 mg/day for another 2-4 weeks. The dose is finally tapered by 5 mg/week
Plasmapheresis
Plasma exchange is indicated in the following situations:
- Patients having hyperviscosity syndrome secondary to mixed cryoglobulinemia.
- Life-threatening cryglobulinemia to reduce productions of cryoglobulins.
- Used along with rituximab or other immunosuppressive agents in case of refractory skin ulcers.
- Dialysis requiring rapidly progressive crescentic glomerulonephritis.