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 therapeutic 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 therapeutic agents used for secondary type are as follows:
- Conservative management
- Immunosuppressive agents
- Plasmapheresis
1.Immunosuppressive agents
The main aim of immunosuppressive therapy in cryoglobulinemia is its employment for the patients having rapidly progressive, organ-threatening or life-threatening course of the disease regardless of the underlying cause.
Indications for immunosuppressive therapy:
The indications for using immunosuppressive therapy are as follows:[1][2][3][4]
- 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.[5][6]
Rituximab 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 rituximab 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 PO followed by 20 mg/day for another 2-4 weeks. The dose is finally tapered by 5 mg/week.
2.Plasmapheresis
Plasma exchange is indicated in the following situations:[7]
- 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.
Plasmapheresis regimen:
- Preferred regimen: Plasma exchange 3 litres daily for 10 to 14 sessions or three exchanges per week for two to three weeks.
Treatment of the underying etiology
The group of patients having mixed cryoglobulinemia secondary to an underlying disease should have a targeted therapy for the particular disease. Such as heptitis C patients having cryoglobulinemia should receive antiviral therapy. Similarly, patients suffering from lymphoproliferative disorders should have specific disease therapy.[8][9]
References
- ↑ De Vita S, Quartuccio L, Isola M, Mazzaro C, Scaini P, Lenzi M; et al. (2012). "A randomized controlled trial of rituximab for the treatment of severe cryoglobulinemic vasculitis". Arthritis Rheum. 64 (3): 843–53. doi:10.1002/art.34331. PMID 22147661.
- ↑ Sneller MC, Hu Z, Langford CA (2012). "A randomized controlled trial of rituximab following failure of antiviral therapy for hepatitis C virus-associated cryoglobulinemic vasculitis". Arthritis Rheum. 64 (3): 835–42. doi:10.1002/art.34322. PMC 3243106. PMID 22147444.
- ↑ Benstead TJ, Chalk CH, Parks NE (2014). "Treatment for cryoglobulinemic and non-cryoglobulinemic peripheral neuropathy associated with hepatitis C virus infection". Cochrane Database Syst Rev (12): CD010404. doi:10.1002/14651858.CD010404.pub2. PMID 25525951.
- ↑ Ignatova TM, Chernova OA, Gaĭdasheva EV (2012). "[Successful rituximab therapy of HCV-cryoglobulinemic vasculitis with severe ulcerative and necrotic lesions of the skin]". Klin Med (Mosk). 90 (5): 64–6. PMID 22993956.
- ↑ Zaja F, De Vita S, Mazzaro C, Sacco S, Damiani D, De Marchi G; et al. (2003). "Efficacy and safety of rituximab in type II mixed cryoglobulinemia". Blood. 101 (10): 3827–34. doi:10.1182/blood-2002-09-2856. PMID 12560225.
- ↑ Quartuccio L, Soardo G, Romano G, Zaja F, Scott CA, De Marchi G; et al. (2006). "Rituximab treatment for glomerulonephritis in HCV-associated mixed cryoglobulinaemia: efficacy and safety in the absence of steroids". Rheumatology (Oxford). 45 (7): 842–6. doi:10.1093/rheumatology/kel004. PMID 16418196.
- ↑ Sandri AM, Elewa U, Poterucha JJ, Fervenza FC (2011). "Treatment of hepatitis C-mediated glomerular disease". Nephron Clin Pract. 119 (2): c121–9, discussion c129-30. doi:10.1159/000325220. PMID 21757949.
- ↑ Dammacco F, Sansonno D (2013). "Therapy for hepatitis C virus-related cryoglobulinemic vasculitis". N Engl J Med. 369 (11): 1035–45. doi:10.1056/NEJMra1208642. PMID 24024840.
- ↑ Pietrogrande M, De Vita S, Zignego AL, Pioltelli P, Sansonno D, Sollima S; et al. (2011). "Recommendations for the management of mixed cryoglobulinemia syndrome in hepatitis C virus-infected patients". Autoimmun Rev. 10 (8): 444–54. doi:10.1016/j.autrev.2011.01.008. PMID 21303705.