Cryoglobulinemia medical therapy

Jump to navigation Jump to search

Cryoglobulinemia Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Cryoglobulinemia from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X Ray

CT

MRI

Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Cryoglobulinemia medical therapy On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Cryoglobulinemia medical therapy

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Cryoglobulinemia medical therapy

CDC on Cryoglobulinemia medical therapy

Cryoglobulinemia medical therapy in the news

Blogs on Cryoglobulinemia medical therapy

Directions to Hospitals Treating Cryoglobulinemia

Risk calculators and risk factors for Cryoglobulinemia medical therapy

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.

Cryoglobulinemia associated with Hepatitis C virus

References


Template:WikiDoc Sources