Schnitzler’s syndrome, an underdiagnosed autoinflammatory disease: current and future perspective

Schnitzler’s syndrome is a rare but usually underdiagnosed autoinflammatory disease, characterized by monoclonal IgM gammopathy, persistent urticaria, intermittent fever, bone pain, and arthralgia or arthritis. Although first reported by Dr. Liliane Schnitzler in 1972, the syndrome continues to pose challenges in diagnosis, and it usually takes more than five years before it is properly identified. Disorders show up in a form of urticaria, arthritis, organomegaly, fever, lymphadenopathy, high ESR, leukocytosis, and bone pain. Central to its pathogenesis are immunologic perturbations and activation of the inflammasome. It is typically diagnosed through clinical exam with history and important feature identification such as monoclonal gammopathy and acute or relapsing urticarial rash. Therapeutically synthetic agents include anakinra, canakinumab, rilonacept, and anti-IL-6 have been used with variable success. Recent research also demonstrated how natural treatments such as Terminalia chebula, Emblica offcinalis, Schinus terebinthifolia, tulsi, asafoetida, and Wedelia plants have the potential for controlling the symptoms and changing inflammatory paths. Promising medicinal possibilities for these plants, according to in silico investigations, point to further research into their clinical uses. With new therapy paths that promise better patient outcomes, Schnitzler’s illness is overall a therapeutic challenge.