Hello everyone!

Since 2019, I have experienced SCLS (few acute attack when triggers were present, such as influenza, infection etc). In 2025, the formal diagnosis was confirmed. SCLS manifests in a more chronic way in my case, and I lack the M-protein. Also, often the IgG is not below reference in my case. I have been put on the TKI Imatinib in June '25, starting at a dose of 100mg. This was increased to 200mg in September '25 and 300mg in June '26. I do experience some improvements but also still the leaking, manifesting as malaise, persistent edema etc. next to quite debilitating side effects from the Imatinib (neuropathy, anemia, neutropenia, leukopenia, nausea, vertigo, etc.). My question is, are any of you familiar with the treatment of TKI for Clarkson? If so, what did you experience?  

I personally feel it seems to do something sometimes, but not much and I still feel like my quality of life has not really increased....  I am unsure whether this treatment suffices in my specific case. I would love to hear from your experiences and if you maybe have some recommendations for me as to what other treatments can be explored. My specialist seems pretty conservative when it comes to IVIG....

Thank you in advance!!

Welcome to our community, Marielle!

As to your question, No, to my knowledge, besides one Dutch patient (see below), nobody in our community has been treated with Imatinib, a medication used to treat certain types of leukemia, bone marrow conditions, and stomach tumors, and it is definitely NOT a recommended therapy for patients with typical SCLS.

In 2012, an article by Dutch physicians affiliated with the University Medical Center in Amsterdam was published recommending the use of Imatinib to reduce capillary leakage in the lungs, see https://www.ahajournals.org/doi/10.1161/circulationaha.112.134304 

However, more recent and robust medical research has found that Imatinib does NOT reduce pulmonary edema or improve clinical outcomes, see https://pubmed.ncbi.nlm.nih.gov/37291677/  Besides, typical SCLS does NOT cause leakages in the lungs or other organs.

Therefore, I suggest that you seek a second and even third opinion on your diagnosis and treatment from a senior internal-medicine physician affiliated with some other major medical center and university school of medicine in the Netherlands, and preferably also elsewhere in Europe.

In particular, I recommend that you make an appointment to be examined by Prof. Dr. Zahir Amoura in Paris, who is Europe's top expert on SCLS, see https://www.aphp.fr/pr-amoura-zahir

For that purpose, you will need to obtain and put together your medical and hospitalization records, preferably electronically, so you or your main physician or your specialist can send your medical history to whoever you choose to see in Holland or elsewhere -- before they actually meet you.

You should also contact one of our members who is both a patient and a retired physician in the Netherlands to seek his advice: see https://rareshare.org/profiles/hiltjo  You can do so via the Message button and also direrctly via email, at hiltjo@xs4all.nl

Hiltjo has been treated with several medications including Imatinib, so he can tell you about his experience. I do not know whether he has ever received, or is receiving, IVIG -- the standard of care for patients with typical SCLS.

Good luck and keep us informed about your journey!

Arturo