Abstract
We report a severe case of chronic non-bacterial osteomyelitis (CNO) associated with total myeloperoxidase (MPO) deficiency. Chronic non-bacterial osteomyelitis (CNO) is often considered an autoinflammatory disease. In pediatric literature CNO is often referred to as chronic recurrent multifocal ostemyelitis (CRMO). CNO is occasionally associated with extremely rare monogenic disease but in most cases the etiology is unknown. MPO deficiency is characterized by low levels of leukocyte MPO, and in some cases even total lack of the enzyme. The condition is seldom associated with pathology; severe Candida infection occurs in about 5% of cases and there is a slightly increased frequency of minor infections.
Highlights
We report a severe case of chronic non-bacterial osteomyelitis (CNO) associated with total myeloperoxidase (MPO) deficiency
Cytokines and phagocyte function was performed both during the flare and after reintroduction of adalimumab. To our knowledge, this is the first time a case of Chronic non-bacterial osteomyelitis (CNO) associated to MPO deficiency is described
Many autoinflammatory diseases are IL-1 driven, which is probably not the case in our patient as she did not respond to IL-1 blockade
Summary
We report a severe case of chronic non-bacterial osteomyelitis (CNO) associated with total myeloperoxidase (MPO) deficiency. Chronic non-bacterial osteomyelitis (CNO) is often considered an autoinflammatory disease. In pediatric literature CNO is often referred to as chronic recurrent multifocal ostemyelitis (CRMO). CNO is occasionally associated with extremely rare monogenic disease but in most cases the etiology is unknown. MPO deficiency is characterized by low levels of leukocyte MPO, and in some cases even total lack of the enzyme. The condition is seldom associated with pathology; severe Candida infection occurs in about 5% of cases and there is a slightly increased frequency of minor infections
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