Just wanted to alert the Community about one of the several new articles I have listed and summarized in the Disorder Resources section of this website.

While there is still a great deal that we do not know about SCLS, the article titled "Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated SCLS" -- it is listed in 2nd place right now -- is important because it summarizes the experience of 69 mostly European patients with SCLS who have been followed for up to 20 years by an international study group comprising 49 medical centers in eight countries (France, Italy, Israel, Switzerland, Lebanon, Canada, Spain, and Turkey).  This is by far the largest group of SCLS patients ever reported on in the medical literature.

The authors, who are the physicians who have been taking care of these patients -- and some of them are members of this Community -- report comprehensively on the clinical characteristics, natural history, and outcome of their patients with SCLS and the MGUS (monoclonal gammopathy) marker. (Typically, about 80% of patients with SCLS have the MGUS, but for this study only those who had it were included.)

Patients were followed starting in January 1, 1997 and were monitored through end-March, 2016. The mean age at diagnosis was slightly over 50 years – children were excluded – and the patients were, by chance, half women and half men.

Four preventive treatments were considered in this study: IVIG, theophylline, terbutaline, and thalidomide. Before the year 2000, the physicians write, the combination of theophylline and terbutaline (T&T) used to be administered as the first therapy, and then IVIG began to be administered as a second-line therapy in cases of T&T failure, but in the past decade IVIG has become the treatment of first choice.

Fifty-seven (86%) of the patients received at least one preventive treatment during follow-up, including: IVIG (48 patients), theophylline (22), terbutaline (22), and thalidomide (5). Twenty-three (35%) of the patients received more than one preventive treatment, which is why the total exceeds 69.

Multiple myeloma occurred in 5 (or 7%) of patients. Twenty-four patients (35%) died after a median of 3.3 years, with the range going from less than one year to as many as 8 years: 20 patients (83%) died during a severe attack and four (17%) from multiple myeloma. Four patients died during their first episode. Patients with a high level of the MGUS at diagnosis were more susceptible to severe relapses.

One, two, five and ten-year survival rates were 97%, 95%, 78%, and 69%, respectively -- meaning that, for example, 69% of total patients has lived for at least a decade after diagnosis. The survivors received significantly more frequent preventive treatment with IVIG than did non-survivors. Five- and 10-year survival rates in patients treated with IVIG were 91% and 77%, respectively, compared to 47% and 37% in patients not treated with IVIG. Patients treated with IVIG also had significantly less recurrence of attacks and fewer severe attacks when compared to patients not treated with IVIG.

In sum, while we are still not sure why IVIG works as well as it does, the wide-ranging evidence strongly suggests that IVIG is the best treatment for SCLS.  Any SCLS patient who doubts the efficacy of IVIG, or any physician who doubts it, or any medical insurance company or medical provider who doubts it, should be given a copy of this article to read.  I have the approved pre-publication draft of the article, and anybody who wants it should email me at aporzeca@american.edu