Just had another routine visit with my immunologist and everything is fine at the moment. However an interesting observation popped up. The MGUS (monoclonal antibody band) protein which was present in my blood before starting IVIG has disappeared. This is a real and repeatable result as I have had this measured 3 times post IVIG. This is interesting as IVIG had eliminated my leaks, as with most other SLCS patients on this treatment. Two interesting questions arise from this: 1. Is this how IVIG is working by down regulating the expression of the MGUS protein? 2. Has anyone else found this when they have started IVIG?

Krogers, I suggest that you write to Dr. Druey at NIH with these questions because he would know better than anybody else in the world, but as far as I know, the answers are: 1. Probably not, it's likely just a side effect, and 2. Yes, most everybody who goes on an IVIG therapy subsequently obtains lower MGUS readings. To recall, only a very small fraction of individuals with an MGUS actually go on to develop SCLS, and some of the latter don't even have it, so the connection between the two is tenuous and mysterious.

Hello Krogers and Arturo, To question 1 : please read the case report : BMJ Case Rep. 2011; Rare disease High-dose intravenous immunoglobulins: A promising therapeutic approach for idiopathic systemic capillary leak syndrome Manuel Zipponi, Roland Eugster, and Tanja Birrenbach Department of General Internal Medicine, Inselspital Bern, University Hospital Bern, Bern, Switzerland "Potential mechanisms of action (of IVIG) include an anti-idiotype effect against autoantibodies and induction of Fc-mediated blockade of Fc-receptors on leucocytes. By interacting with complement, IVIG prevent it from mediating tissue damage by scavenging its active components and diverting complement attack from cellular targets. The prophylactic efficacy might, under special circumstances, be related to the prevention of infections triggering an acute episode of SCLS, as proposed by Lassoued et al in a patient with SCLS and concomitant hypogammaglobulinaemia successfully treated with IVIG (0.4 g/kg monthly). Meanwhile, the hypogammaglobulinaemia in our patient was clinically insignificant and cannot have caused repeated attacks by predisposition to recurrent infections. Several reports demonstrate evidence of an immune-mediated pathogenesis of SCLS, with increased levels of cytokines, including IL-6, IL-10, interferon-γ and tumour necrosis factor-α. IVIG possess anticytokine properties and inhibit the synthesis of different ILs, including IL-2, and might operate in SCLS through modulation of the immune response". To question 2 : Arturo is right. My MGUS disappeared since the begining of treatment with IVIG. But I have no precise explanation why MGUS disappears. Is MGUS hidden by IVIG??? It is likely that the MGUS is not in itself the cause of SCLS. Claude Pfefferlé

I am being treated with IVIG. No more attacks so far but my MGUS has elevated.

Claude, Interesting article. Do you have a copy of the full article as I do not have access to it without paying for subscription and if so can you send me a copy please? Although not everybody has the MGUS it could still be important clue and moreover means that IVIG could have an additional benefit of lowering the chance of the development of myloema which 1% of MGUS patients develop each year.

Hell everybody, I had an appointment this last decembre with my hemathologist and surprise! My MUGS has increased and there are proteins in my urine. I am worry , prof Amoura said two years ago that I needn't to have IVIG. I go back to see my hematologist at the begining of april for anothers analys, and after that, we will see if I need to begin a treatment.......if you see what I mean? I didn't think that one day I will have this kind of problems ,he wants to see me now every four months. I will keep you in touch as soon as I have anothers informations about my health. Best regards, Anne Laure.