The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 28

36. Is MCAS less serious than SM?

No.

There is a lot of literature presenting data on SM. There is a lot less on MCAS. This is largely because of how recently it has described and the fact that different sets of criteria make it impossible to do large scale studies as have been done with SM. So it’s hard to objectively compare the data because the same volume just doesn’t exist yet.

Many providers and researchers think of MCAS as a form of “preclinical SM”. This term was tossed around in the early 2000s by SM researchers who found patients that seemed to have SM but didn’t meet the criteria for it. There were a few presentations in which an image was shown of a line with the different types of SM shown.

From left to right, the line read:
Preclinical SM/Indolent SM/Smoldering SM/Aggressive SM/Mast cell leukemia

Based upon this figure, and the fact that we are trained to look at lines like this as continuum that either increases or decreases in order, many people latched onto “preclinical SM” (like MCAS) as being the least dangerous. Importantly, the figure refers to the increasing danger of permanent organ damage by mast cells ending up in organ tissues. It does NOT refer to the danger of anaphylaxis.

Indolent systemic mastocytosis (ISM) is the least dangerous form of SM and by far the most common. When people ask if MCAS is less dangerous than SM, they usually mean is MCAS less dangerous than ISM. A couple of small study groups have found that prevalence of anaphylaxis in MCAS is less frequent than in ISM. However, this comparison is flawed. Many people have known they have SM for 20+ years. MCAS hasn’t even been a viable diagnosis for 10 years. MCAS is also less likely to be diagnosed due to decreased exposure on the part of many providers. Many MCAS patients are diagnosed with idiopathic anaphylaxis instead so you’re not really looking at a robust population of MCAS patients in these studies.

ISM has a normal lifespan. It is treated the same way as MCAS and the two conditions have remarkably few differences beyond very specific markers that show the body making too many sloppy mast cells.

Some MCAS patients have protracted anaphylaxis and a normal baseline of very serious daily symptoms. It is my personal opinion that the anaphylaxis episodes I have observed in many MCAS patients can be a lot worse than you see in ISM. MCAS patients also have a harder time finding treatment. While ISM patients certainly run into unknowledgeable providers, it is my experience that having an ISM diagnosis is more helpful for facilitating treatment than an MCAS diagnosis.

We need time in order for larger studies and more unifying MCAS criteria to emerge but I am certain that these will follow. MCAS is at least as dangerous as ISM, if not more. Both MCAS and ISM are less dangerous than SSM, ASM and MCL.

For more detailed reading, please visit these posts:
The Provider Primer Series: Mast cell activation syndrome (MCAS)
The Provider Primer Series: Diagnosis and natural history of systemic mastocytosis (ISM, SSM, ASM)