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Publication of Results from Survey of IVIG Therapy

aporzeca Message
14 Sep 2014, 10:22 PM

I’m extremely pleased to report that Dr. Kirk Druey and his team from the U.S. National Institutes of Health (NIH) have had their paper based on the results of a patient survey on the outcome of IVIG as therapy for SCLS accepted for publication later this year in _The American Journal of Medicine_. The peer-reviewed and accepted, but not yet edited, typeset or proofed, version is available online for journal subscribers, or others with access (e.g., through a university or public library subscription), to the Journal's restricted website. Those Community members who lack access and wish to receive this pre-publication version of the article may request it from me at _aporzeca@american.edu_ Please include your RareShare username in the email to me. As many of you will recall from a post earlier this year (see Discussion Forum topic "Survey of All Users of IVIG"), the article reflects the results of a questionnaire sent by Dr. Druey to all SCLS patients who have been in touch with him over the years, to find out about their experience with any IVIG therapy. Most of the questionnaire respondents, of course, are members of this Community. To quote from the article's Conclusion: "Our case series, a European registry, and numerous individual case reports suggest that monthly administration of IVIG is associated with a marked reduction in the number of episodes of hypovolemic shock and edema in patients with classic acute Systemic Capillary Leak Syndrome. These studies indicate that IVIG may be considered as frontline therapy for subjects with a clear-cut diagnosis of the Systemic Capillary Leak Syndrome and recurrent attacks." *Therefore, this article is likely to promote the use of, and facilitate access to, IVIG as the medication of choice for all confirmed SCLS patients.* Following are the results as summarized in the abstract appearing on the Internet page _http://www.ncbi.nlm.nih.gov/pubmed/25193271_ *High dose intravenous immunoglobulin therapy of the Systemic Capillary Leak Syndrome (Clarkson disease).* Xie Z, Chan E, Long LM, Nelson C, Druey KM. Laboratory of Allergic Diseases, NIAID/NIH, Bethesda, MD 20892 BACKGROUND: The Systemic Capillary Leak Syndrome is a highly rare disorder of unknown etiology. The disease is characterized by episodes of transient vascular collapse, which leads to hypotensive shock and anasarca. Previous treatment of this potentially devastating condition has been largely ineffective. We evaluated IVIG prophylactic therapy in a cohort of 29 patients with Systemic Capillary Leak Syndrome in a longitudinal follow-up study. METHODS: All patients received treatments at the discretion of their primary providers and retrospectively via questionnaire-recorded symptoms beginning with their first documented episode of the Systemic Capillary Leak Syndrome until May 31, 2014. RESULTS: Twenty-two out of 29 patients responded to the questionnaire, and 18 out of the 22 respondents received monthly prophylaxis with IVIG during the study period for a median interval of 32 months. The median annual attack frequency was 2.6/patient prior to IVIG therapy and 0/patient following initiation of IVIG prophylaxis (P = 0.001). 15 out of 18 subjects with a history of one or more acute Systemic Capillary Leak Syndrome episodes experienced no further symptoms while on IVIG therapy. CONCLUSIONS: *IVIG prophylaxis is associated with a dramatic reduction in the occurrence of Systemic Capillary Leak Syndrome attacks in most patients, with minimal side effects.* A prospective, randomized trial may be necessary to fully assess the benefits of IVIG for the Systemic Capillary Leak Syndrome and to determine optimal dosage and duration of therapy.
stedrick Message
15 Sep 2014, 04:17 AM

Arturo, Bless you for this information. May I have permission to print it for my primary care team and regional emergency departments? It just may break the logjam. Susan
aporzeca Message
15 Sep 2014, 10:17 AM

Susan, Of course! And I've just emailed you the actual article, as well.
WazzaACT Message
16 Sep 2014, 12:54 AM

This is great news. I have sent a copy of your post to the Canberra Hospital. Can you send me a copy of the full article too please. Warren
custom Message
16 Sep 2014, 12:33 PM

Arturo Hope you are doing well. Please send me a copy of the results . I should be one of the 29 patients that responded.
susanfv Message
16 Sep 2014, 12:42 PM

I sent a copy of your post to Dr. Lauter . He recently presented on SCLS at an Allergy/Immunology conference in California . I would appreciate it if you could send me the full article as I know he and Dr. Steigerwalt would both like a copy . I continue to receive IVIG every 2 weeks. It has been over 2 years now . Other then some swelling in between treatments as a result of missing a week , I have had no attacks. It takes 7 -7.5 hours to infuse . I then receive 45 minutes of a mixture of fluids which has helped my migraines and nausea tremendously . I am very fatigued for 48 hours following the infusion . Thank you to all who contribute to this site - the information is invaluable . A special thank you to Arturo.
Barney Message
16 Sep 2014, 01:45 PM

Arturo - This is absolutely terrific. I continue to receive monthly treatments - I go in again this Thursday and Friday for 7 - 8 hours each day to receive Octagam. Since I have started this in July of 2011, I have been completely symptom free. I am back to exercising both for physical and mental health and can honestly say I am feeling like myself again. The only thing that worries me now from time to time is the potential risk that they stop using IVIG due to insurance or cost reasons. An article like this is invaluable to validating the success many of us are having with IVIG. The backing of scientific evidence could support the adoption of using IVIG for SCLS is another step forward for all of us. Thank you for this article and for your support in the past as well. Marc
elganzory Message
17 Sep 2014, 08:14 AM

Hi Arturo Thank you so much for this information its importante to us if you mail it for me to inform My team also to send a copy to Dr Amoura as you know for the problem to reduce the ivig DOS ,but hear in Italy the y refused to reduce it. yaserelganzory@hotmail.it Thank you Yaser
rnuara Message
17 Sep 2014, 10:39 AM

Arturo, A special thank you for your assistance in addressing this rare disorder with NIH and Dr. Druey. After many years, SCLS is getting awareness that it needs. We are all grateful that you been persistent in this cause. At your convenience can you send me a copy. Hope to see for the Holidays. Robert
aporzeca Message
17 Sep 2014, 12:59 PM

Robert, Thank you so much for your kind words. The address I have for you bounced, so please send me an email.
Maccy80 Message
25 Sep 2014, 01:27 AM

Hi Arturo, Thank you for being a great advocate for all of us, and keeping us all informed. Is it possible to send me a copy of this study to my email address mel.mcf@bigpond.net.au. Thanks Melanie
tiggrrr027 Message
27 Sep 2014, 07:53 PM

Arturo, This is awesome news!! You are an amazing man and have helped so many. Thank you for all you do and congratulations to Dr druey and his team as well! Linda
elganzory Message
16 Oct 2014, 08:53 AM

Thank you Arturo I hope to recive a copy to My kip to be upgrade about this ivig , You alwyes very kind Thanks agin Yaser yaserelganzory@hotmail.it
starfrance Message
17 Oct 2014, 06:58 PM

Thank you Arturo, Can you please send me the whole copy too ? Fortunatly, dr Druey is here for us !! annelaureviv@gmail.com
josee Message
28 Oct 2014, 02:27 AM

Bonjour Arturo, j'aimerais aussi recevoir une copie de l'étude. il me fera plaisir d'en remettre une copie a mes Médecins à Amos. Merci a vous pour tout ce que vous faites !!! mon adresse courriel: foresteriesdj@tlb.sympatico.ca