Schnitzler-Syndrom

Abstract

Schnitzler syndrome is a very rare acquired systemic disease with many similarities to hereditary autoinflammatory syndromes. The main characteristics are generalized exanthema and a monoclonal gammopathy with IgM. Other clinical features include fever, muscle, bone and/or joint pain, and lymphadenopathy. About 15-20% of patients with Schnitzler syndrome develop lymphoproliferative diseases and, in rare cases, amyloid A (AA) amyloidosis can occur if the disease is not treated. Activation of the innate immune system, especially interleukin(IL)-1β, is central in the pathogenesis of the disease. Consequently, complete control of disease symptoms can be achieved in 80% of patients by treatment with the IL-1 receptor antagonist anakinra.

Case report: Successful treatment with tofacitinib and colchicine in a patient with Schnitzler syndrome

“…The median onset age is 59 years. It was first reported by French dermatologist Dr. Liliane Schnitzler in 1972 1,2 and was primarily reported in Caucasian populations 3 . A study by Mayo Clinic reported about 74% of the patients were misdiagnosed 4 .…”

“…Schnitzler syndrome is a rare acquire systemic inflammatory disease, characterized by chronic urticarial-like rash and monoclonal 1,2 and was primarily reported in Caucasian populations. 3 A study by Mayo Clinic reported about 74% of the patients were misdiagnosed.…”

“…Chronic urticaria rash, monoclonal IgG gammopathy, neutrophilic dermal infiltration on skin biopsy, elevated CRP level, and recurrent fever fulfilled 2 obligate criteria and 3 minor criteria of the Strasbourg criteria. 1 The patient was thus diagnosed with Schnitzler syndrome.…”

Autoinflammatory diseases and the kidney

“…The risk of amyloidosis for uncontrolled active disease must always be taken into consideration [ 60 , 61 ].…”

Informed consent and biological agents in rheumatology and internal medicine

“…40 Infliximab Pityriasis rubra pilaris, 30 idiopathic hypereosinophilic syndrome, 41 cachexia in advanced pancreatic cancer patients, 42 refractory retinal vasculitis due to sarcoidosis, 43 cardiac sarcoidosis, 44 Takayasu arteritis, 45 refractory dermatomyositis, 46 steroid-resistant graft-versus-host disease, 47 complex regional pain syndrome, 48 hidradenitis suppurativa, 49 pyoderma gangrenosum, 50 Behçet's disease, 51 childhood refractory chronic uveitis, 26 refractory neuro-Behçet. 52 Anakinra Colchicine-resistant familial Mediterranean fever, 53 Schnitzler's syndrome, 54 Muckle-Wells syndrome, 55 acute gout, 56 hidradenitis suppurativa. 57 Abatacept Systemic lupus erythematosus.…”