It is, I think, proven that the periodical administration of IVIG is very important to us and an important factor in preventing new attacks of our disease. Of course it does not give us the  guarantee of not getting a new attack. I always look forward to go to the hospital to receive the IVIG. It takes about 5 hours and it makes me feel more comfortable.

Next Tuesday I will receive my new portion (1g/kg every 3 weeks). My haematologist (not a SCLS speciailist, ) always comminicates me my IgG level (from the blood taken just before the new IVIG administration and that is always at a high  level between 15-17.  After my attack 3.5 year ago that parameter was low. I consider this parameter as important and feel rather comfortable with that IgG level being high.

Is that high level of IgG a good sign or do we have to take also other parameters (from the blood results) in consideration?

Thanks in advance for your comments.

Hans

 

 

Hello Hansdewit,
The residual IgG level just before the prophylactic infusion of polyclonal immunoglobulins is an important parameter in the follow-up of SCLS. If this level remains stable, the level of other Ig produced by the bone marrow (IgA, IgM) should be monitored. Indeed, the clone that produces the MGUS paraprotein found in 80% of SCLS patients could evolve and suppress the production of other "normal" Ig. It is also known that an Ig molecule is composed of 2 chains, one called "heavy" and the other called "light".  These "light" chains are of 2 different types: "Kappa" and "Lambda". The level of these 2 light chains and their ratio (Kappa/Lambda) must be monitored. Best regards. Claude  

 

To provide some background, on average, about 1% of people with an MGUS go on to develop multiple myeloma each year, a cancer of the plasma cells, see https://www.mayoclinic.org/diseases-conditions/multiple-myeloma/symptoms-causes/syc-20353378#:~:text=Multiple%20myeloma%20is%20a%20cancer,crowd%20out%20healthy%20blood%20cells

In years past, only people who had a high- or intermediate-risk MGUS were recommended to receive annual follow-up tests to check for signs of progression toward multiple myeloma.

However, in recent years more physicians have been recommending that all individuals with an MGUS have annual blood tests regardless of their initial risk assessment, a recommendation that certainly applies also to all SCLS patients with an MGUS, see https://www.cancer.gov/news-events/cancer-currents-blog/2019/mgus-multiple-myeloma-progression-risk#:~:text=On%20average%2C%20about%201%25%20of,certain%20markers%20in%20the%20blood 

There is an intriguing and related new article co-authored by Dr. Kirk Druey about two SCLS patients with an MGUS who went on to develop multiple myeloma, and after successful treatment one stopped her IVIG therapy and the other cut his pre-myeloma dose in half (from 2 to 1 g/kg) -- and yet neither has had an episode of SCLS in the subsequent 2-3 years, see article at https://www.jaci-inpractice.org/article/S2213-2198(22)01066-2/fulltext

However, in my view, it's way too premature to infer that SCLS-MGUS patients who develop multiple myeloma, are treated for it, and survive can do without IVIG afterwards.  How can one possibly draw trustworthy conclusions from just two reported patients, especially since only one of them has abstained from IVIG therapy, even if both remain episode-free for several years?  We have a number of patients in our community who receive 1 g/kg and don't suffer from renewed episodes of SCLS, so that's not news.

“Thank you very much for your reply, but I was misunderstood: I was not asking about the monitoring of the MGUS but about whether the measurement of our IgG levels is a reliable indicator of how much protection we have against an episode of SCLS.

 

Is it a good thing that my IgG levels even before my infusion of IVIG are relatively high?  I ask because I receive 1 g/kg of IVIG every 3 weeks, rather than 2 g/kg, so I wonder if that is enough.”

Hans, sorry for the misunderstanding.  My information is that IgG levels are not a reliable indicator of how much protection we have against an episode of SCLS.  (IgG is the most common type of antibody found in our blood.)  Experience shows that the protection provided by IVIG varies among SCLS patients, because they seem to need different amounts of IVIG in order to prevent episodes of SCLS.  There has not been a great deal of experimentation, but some appear to need 2 g/kg/4 weeks -- or in rare cases, even a little more -- while others remain stable with 1.5, 1.0, and in rare cases even 0.5 g/kg/4 weeks.  (By getting 1 g/kg/3 weeks, you are getting the equivalent of at least 1.3 g/kg/4 weeks.)

As you can read in https://rareshare.org/topics/2191, a group of European physicians has recently published the results of a first systematic survey of the experience with reducing the dosage of IVIG, and of ending the administration of IVIG, in patients with SCLS.  To make a long story short, IVIG tapering was not statistically associated with increased person-year incidence of attacks, while IVIG withdrawal, on the other hand, was associated with increased mortality and a higher rate of recurrence in SCLS patients.  A summary of and link to the article is included in our Disorder Resources section, see https://rareshare.org/communities/systemic-capillary-leak-syndrome#disorder_resources

What has also become clear in recent years is that we have stronger protection against episodes soon after our infusions -- no matter the amount we receive -- and that means we have weaker protection against episodes in the week before our next infusions.  Consequently, we must be more cautious in terms of wearing a mask and keeping our distance from crowds, especially indoors, towards the end of our infusion cycles, because we are more vulnerable to viral infections.  This is one of the lessons that we should draw from the recent article "Management of Acute Episodes of Clarkson Disease (Monoclonal Gammopathy-Associated Systemic Capillary Leak Syndrome) With Intravenous Immunoglobulins," see https://rareshare.org/topics/2192

Hy Hans, I totally agree with Arturo's opinion. In my case, I do not check the residual IgG level before my monthly infusion (2 g / kg). On the other hand, I check the immunological parameters (IgA, IgG, IgM, Kappa and Lambda light chains and their ratio) once a year, just before the prophylactic perfusion, to anticipate the occurrence of a myeloma... because I have been carrying a MGUS (IgG Kappa) for 20 years. Best regards. Claude