Abstract

THE SCHNITZLER SYNDROME: AN ACQUIRED AUTOINFLAMMATORY SYNDROME BY MONOCLONAL IMMUNOGLOBULIN

Kushal Nandi*, Sandip Sarkar, Pritam Bakshi, Saroni Saha, Dhrubo Jyoti Sen, Kishor Dholwani and Dhananjoy Saha

Abstract

Schnitzler syndrome is a rare disorder characterized by a chronic reddish rash that resembles hives (urticaria) and elevated levels of a specific protein in the blood (monoclonal IgM gammopathy). Symptoms associated with Schnitzler syndrome may include repeated bouts of fever, joint inflammation (arthritis), joint pain (arthralgia), bone pain, and other findings such as enlarged lymph nodes (lymphadenopathy). A monoclonal IgM gammopathy refers to the uncontrolled growth of a single clone (monoclonal) of plasma cells, which results in the abnormal accumulation of M-proteins (also known as immunoglobulin M or IgM) in the blood. However, the specific role these proteins play and the exact cause of Schnitzler syndrome is unknown. Schnitzler syndrome is difficult to classify and some researchers have suggested that it is an acquired autoinflammatory syndrome. Autoinflammatory syndromes are a group of disorders characterized by recurrent episodes of inflammation due to an abnormality of the innate immune system. They are not the same as autoimmune disorders, in which the adaptive immune system malfunctions and mistakenly attacks healthy tissue. This review will provide a comprehensive overview of the clinical and biological features of this syndrome, emphasizing its particular rash, its complications and its treatment.

Keywords: Fever, Joint Pain, Schnitzler Syndrome, Organomegaly.