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Proteasome inhibitors for malignancy‐related lambert‐eaton myasthenic syndrome
Author(s) -
Wang Chen,
Chen Shaobo,
Feng Bing,
Guan Yuzhou
Publication year - 2014
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.24122
Subject(s) - malignancy , lambert eaton myasthenic syndrome , medicine , myasthenia gravis , immunology , autoantibody , cancer , lung cancer , proteasome , antibody , biology , microbiology and biotechnology
Lambert‐Eaton myasthenic syndrome (LEMS) is an autoimmune disorder characterized by autoantibodies against presynaptic voltage‐gated calcium channels that impair neuromuscular transmission. Malignancies, especially small cell lung cancer (SCLC), have been associated with LEMS and account for approximately 60% of cases, making malignancy management a central step in LEMS therapy. In addition, immunosuppressive therapy is also recommended for symptomatic control. Interestingly, both pathological and epidemiological data suggest that the autoimmune response can inhibit progression of tumors in malignancy‐associated LEMS. Thus, conventional broad‐spectrum immunosuppressants may not be effective agents for treatment of LEMS, especially in those with malignancy‐associated LEMS. Recent preclinical and clinical studies have indicated that proteasome inhibitors can eliminate antibody‐producing cells efficiently, block dendritic cell maturation, and have anti‐tumor activity. We hypothesize that proteasome inhibitors may be promising agents for treatment of malignancy‐related LEMS. Muscle Nerve 49 :325–328, 2014