I do not have a definite diagnosis for SCLS but my doctor suspects this may be my problem. I have not had a major life threatening episode but I have chronic episodes with all of the physical symptoms, including low BP, before and during. My swelling episodes are coming almost weekly and last from 3-4 days, but I have never been hospitalized. The one time I went to the ER for horrible swelling, shortness of breath, & weakness, with low BP, the doctor sent me home with a second diuretic and told me to lay off the salt! I have had alot of blood work and so far, most everything is normal. My doctor's nurse called me today to say my recent MGUS test was good. (I do show consistent low lymphocytes, iron saturation, & hgb) I do understand you can have SCLS without MGUS, however, without this marker, and other blood work showing normal, what is the next step for a diagnosis? I appreciate any advice and direction. Thank you!

I've had a bunch of blood tests that were normal. Arturo can answer better on the MGUS issue but the most important thing you can do is to have a Dr. you trust and that you can show the SCLS literature. I've had the same reaction that you have from emergency rooms, being released without understanding what the attack was about. Print out the Literature from the resources page here and show them to you Dr.

Dear Caseydan: As it says in the "Disorder Details" section, the symptoms for a diagnosis during an episode of SCLS are hypotension (low blood pressure), hemoconcentration (a high ratio of red cells to plasma), hypoalbuminemia (a low level of albumin), and the presence of the MGUS protein. What your doctor should do is have you go to a place where they will measure your blood pressure and draw and test your blood a couple of times a day on the days that you experience swelling -- to see if you have hypotension, hemoconcentration and hypoalbuminemia -- relative to days when you do not swell. If those markers don't show up, then you probably have some other disorder that causes swelling -- and there are several of those. Good luck on getting a proper diagnosis!

Thank you both for your comments!!

Like you I didn't have an attack, with severe lowering of Blood pressure that admission on IC was reguired. And experiencing also chronic persistent swellings it appears different from experiences of most others. As you can read on this forum and in the script of mr. Porzecanski patients with SCLS experiencing major attacks, report also smaller ones in between. In literature chronic SCLS is described in just à few cases, with and without presense of MGUS. It is considered to be à diagnostic challenge because of being so rare, and as was stated in 2010 ' all other diseases should be ruled out vigorously', which reguires à dedicated and experienced doctor to do so. I have been through that process which took about two years to find out and to come to à conclusion. If your diagnosis is still standing it might be interesting to keep in touch.

I had joined this forum to get information about SCLS for my brother-in-law who was in ICU since 23rd Sept in India.He was diagnosed with SCLS in 2008. This attack started with flu like symptoms and he had his body swollen and was have dialysis for last 6 days. He died today from Cardiac arrest. I was hoping that he would come out fine as he was showing positive signs in the last 2 days. Is there anything that i could do to help, please let me know. I wish someone finds cure for SCLS.

Dear Mathumx: Please accept my most sincere condolences on the untimely death of your brother-in-law. SCLS is an exceedingly rare episodic, life- and limb-threatening illness. While doctors have come a long way in recent years in terms of increasing their knowledge of how to manage and even prevent the episodes, and this knowledge has spread quite rapidly throughout the world via the Internet -- including via this site -- we are still suffering casualties from time to time. You can help by having his relatives in India obtain and forward a copy of his hospital records (and particularly his blood laboratory results) to Dr. Kirk Druey at the National Institutes of Health, see the tab "Disorder Details," then scroll to the bottom for contact information. He is collecting as much information as possible on patients and their treatments.