Case Report on the Illness of Paul Klee (1879-1940)
29th June is designated World Scleroderma Day, marking the death of the artist Paul Klee in 1940, postulated to be from complications of Systemic Sclerosis. Hans Suter, a dermatologist, started researching the cause of death some 40 years later, publishing his conclusion in 2014.
Without a doubt, Scleroderma (or Systemic Sclerosis SSc) is the toughest autoimmune disease to treat.
To date, no effective treatment has been found to work well for even one aspect of SSc, let alone its myriad systemic manifestations (skin, lungs, heart, gut, kidneys). This is in stark contrast to other rheumatic diseases like RA, AS, PsA or even SLE, where we are talking about treat-to-target strategies, remission maintenance and even tapering of therapies.
One important reason for this dismal state of SSc therapeutics is that we still don’t know how this disease is triggered and driven. Our best clues are that blood flow to end organs is compromised, and that excessive scarring ensues. So research is focused on the endothelial cells and the fibroblasts.
The key to better treatment lies in better understanding of the disease, which in turn lies in basic science.
Innate Immunity in Systemic Sclerosis – Role of Toll-Like Receptors, Interferon, and the Potential Impact of Vitamin D
I find this paper most intriguing and thought-provoking. If proven true, this may well be the Holy Grail in the pathogenesis of Scleroderma, paving the way to the discovery of effective therapeutics for the early phases of the disease.