CRISIS TREATMENTS

• Last update 11 February 2010
• Plasmapheresis
  • Plasmapheresis essentially cleans antibodies from the patient's blood.
    • The patient's blood plasma can be either replaced or treated to filter out the antibodies.
    • It is a subset of various kinds of apheresis.
  • WARNING - A 1995 study warned that treatments (such as plasmapheresis) that decrease plasma alpha-1 antitrypsin  serum level may be insufficient in patients with alpha-1 anti-trypsin deficiency (AATD) unless the alpha-1 antitrypsin is replaced.
  • One study (Aug 2006)  found that plasmapheresis (plasma exchange) was a positive predictor of dialysis independence after 12 months for the entire patient group studied.
    • Plasma exchange remained a positive predictor when patients who were dialysis dependent at presentation were analyzed separately.
    • Normal glomeruli were a positive predictor of dialysis independence and improved renal function after 12 mo, indicating that the unaffected part of the kidney is vital in determining renal outcome.
  • An abstract of a 2006 article in European Journal of Pediatrics noted that plasmapheresis, "appears to be of benefit during the acute phase of illness, especially in children with organ-specific disease".
  • Various immuno-absorption devices such as the Prosorba column or Excorim or similar may be effective in removing harmful antibodies.
    • In February of 2001, some success was reported using immunoadsorption in early stages of the vasculitis.
  • Click here to view an Adobe Acrobat file with guidelines on using plasmapheresis (Hemapheresis).
• Aggressive dosing
  • High dosage Prednisone or similar is the first line of attack.  As much as 400 to 1000 mg per day of prednisone might be given intravenously to an adult in crisis.
  • Dosages of immunosuppressives may be doubled in crisis cases.
    • Kidney condition may not allow increased dosing, and may require reduction of ISs from the normally prescribed amount..
• Biologicals
  • Some of the current biologicals and possibly some in development may prove to be useful in crisis.
    • See the section on biological treatments above.
• Hyperbaric oxygen
  • Hyperbaric oxygen may have been used to treat claudication (poor blood flow, particularly in extremities) leading to peripheral neuropathy.  It has been successfully used to assist in healing skin lesions in some AV cases.
• Immunoglobulin (IVIg)
  • Apparently, effectiveness over a large population of AV patients hasn't been studied, however a 2005 study showed that IVIg (6 mo) were able to induce complete remission of relapsed ANCA-associated systemic vasculitides in 12/20 (60%) patients.
  • Because of their safety and good tolerance, IVIg should perhaps be included in a therapeutic strategy comprising other drugs to treat relapse or maintain remission of these vasculitides.
    • Some adverse reactions have occurred using IVIg for other diseases.
    • IV treatments may expose the patient to a slight risk of viral infection from the plasma bank.
    • Click here to download an Adobe Acrobat file on off-label use of IVIg.  Downloading of pdf file may take longer than most downloads, and requires Adobe Acrobat Reader.
  • 2007 research showed that the effective part of immunoglobulin is in 0.5 % and in the form of immune complexes that may be possible to produce without extraction from human immunoglobulin G.
• Stem cell transplant
  • Stem cell transplant (SCT) has been used in a few cases of AV and may be a good choice of treatment for a few selected refractory cases, although it has significant risks and costs.
    • A recent study showed that some of the risk can be removed by having not only an HLA match, but a match between donor and recipient of the Interleukin-10 genotype.
    • For the present, SCT probably should be used only in cases refractory to other treatments.
    • The cost is estimated as roughly $100,000, plus pre-admittance costs, etc
    • Both allogenic and autologous methods are in use, as are conditioning regimens that can be either myeloablative or non-myeloablative.
    • For information on types of SCT and on effects on patients, see  http://en.wikipedia.org/wiki/Bone_marrow_transplant#Conditioning_regimens
    • For a detailed study on SCT for autoimmune disease, see http://www.nature.com/nature/journal/v435/n7042/full/nature03728.html
    • The article must be purchased if one is not a subscriber.
    • An Oct. 2007 rather limited study suggested that, "... non-myeloablative autologous HSCT is safe and effective treatment for select patients with SV refractory to conventional immunosuppressive therapies."�
  • Northwestern University in Chicago, Illinois is enrolling selected AV patients who have failed all conventional therapies into a "hematopoeitic stem cell transplant" clinical trial.
    • Information is available at the office of Kathleen Quigley. Div. of Immunology, Dep't. of Medicine, Northwestern Medical Center, Chicago, Illinois, Ph 312-908- 0059.
    • Generally, most medical costs are paid for by the clinical trial if one is accepted.
    • The Northwestern web page for the Feinburg School of Medicine Immunology Department is http://cms.medicine.northwestern.edu/divisions/immunotherapy_autoimmune/contact.html
  • Insurance companies may be cajoled into paying for the expensive process to avoid future hospitalization and years of treatment and physicians appointments and testing.
  • Another treatment which has had very limited use in treating WG is immune system ablation without stem cell transplant.
    • This method depends on surviving bone marrow stem cells to rebuild a normal immune system.
      • For information, contact Dr. Brodsky at Johns Hopkins Vasculitis Center.
    • The first WG patient is starting this treatment in Dec. 2006.
    • Progress for one WG patient treated with ablation therapy has not been satisfactory.
  • B cell depletion using Rituximab (Rituxan) may be a useful and safer alternative than immune system ablation [in the compiler's opinion].  Rituximab used for patients with Hepatitis C-caused cryoglobulinemia vasculitis can cause dangerous precipitations.

THIS SITE

• Intent
  • To assist vasculitis patients in getting early diagnoses, effective treatments, and to advise of patient, organization, and scientific resources concerning vasculitis.
• Sources Used
  • The following information is derived from a variety of sources over some ten+ years and is not to be considered as medical advice, but merely the opinions or experiences or findings of the writer who is not a physician and has no medical training.
  • Much comes from Medline abstracts and medical journal articles on vasculitis.  Some is from autoimmune vasculitis patients and carers, some from newsgroups,  internet web pages, etc. that also deal with vasculitis.
  • The compiler has attempted to use only recent valid medical information regarding vasculitis, but cannot guarantee the validity nor the currency in every case
• Limitations
  • No medical decisions should be made on the basis of information on this web page or on associated linked documents and web pages unless those are from a recognized medical professional or professional medical publication.
  • Limits to this web page concerning vasculitis:
    • The author/compiler/editor of this web page and related pages has had NO medical training.
    • Only autoimmune vasculitides will be considered, not hypersensitivity vasculitis nor vasculitis as a result of an allergic reaction to medication or vaccine..
    • Most sections apply to most autoimmune vasculitides.
      • One refers specifically to Wegener’s granulomatosis.
• Terminology
  • Some abbreviations and equivalencies are:
    • Hereafter, “autoimmune vasculitis” may be abbreviated “AV” or “AVs” for plural.
    • The term “Prednisone” is sometimes used where it or a similar glucocorticoid might be prescribed to treat vasculitis.
    • “Immunosuppressives” used to  treat vasculitis are sometimes abbreviated “ISs”.
• Updates
  • This update was on June 1, 2009 and is a complete rewrite of the former web page at http://www.wegenersgranulomatosis.net that also dealt with vasculitis.
• Files & links – Vasculitis related.
  • Click here to view a web page listing all the links and files from this page.
• Tables & Figures – Vasculitis related
  • Click here to view a listing of the tables and figures in this web page.
• Disclaimer
  • ALL MEDICAL QUESTIONS, SYMPTOMS, CONCERNS AND PROBLEMS SHOULD BE DIRECTED TO APROPRIATE LICENSED MEDICAL PROFESSIONALS.
  • The writer/editor/compiler does not vouch for the accuracy, completeness, nor applicability of the information included on this site to any person, whether a vasculitis patient or otherwise. 

This website and all content - © Copyright 2009, Bruce Macdonald. All Rights Reserved.
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